Articoli di riviste: "Acute exacerbation of interstitial lung diseases"

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Aritonang, Rachel S., Fanny Fachrucha, Mia Elhidsi e Ginanjar Arum Desianti. "Oxygen Therapy in Exacerbation of Interstitial Lung Disease". Respiratory Science 4, n. 3 (28 giugno 2024): 221–31. http://dx.doi.org/10.36497/respirsci.v4i3.129.

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Interstitial lung diseases (ILD) are a group of diseases that involve damage in the interstitial tissue, causing diffusion disorders which ultimately lead to hypoxemia. One of the conditions that aggravate hypoxemia in ILD patients is acute exacerbation. Acute exacerbation is a condition of deterioration of ILD that can occur in less than 1 month. During an acute exacerbation, there will be a worsening of the HRCT pattern with increased ground glass opacities and a worsening of the clinical picture including hypoxemia. Acute exacerbations are closely related to increased mortality rates. Oxygen administration is one of the supportive therapies that can be given to acute exacerbations. The provision of oxygen therapy is adjusted to the patient's needs using a high-flow nasal cannula, non-invasive ventilation, invasive mechanical ventilation, and extracorporeal membrane oxygenation.

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Kolb, Martin, Benjamin Bondue, Alberto Pesci, Yasunari Miyazaki, Jin Woo Song, Nitin Y. Bhatt, John T. Huggins et al. "Acute exacerbations of progressive-fibrosing interstitial lung diseases". European Respiratory Review 27, n. 150 (21 dicembre 2018): 180071. http://dx.doi.org/10.1183/16000617.0071-2018.

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Acute exacerbation of interstitial lung disease (ILD) is associated with a poor prognosis and high mortality. Numerous studies have documented acute exacerbation in idiopathic pulmonary fibrosis (IPF), but less is known about these events in other ILDs that may present a progressive-fibrosing phenotype. We propose defining acute exacerbation as an acute, clinically significant respiratory deterioration, typically less than 1 month in duration, together with computerised tomography imaging showing new bilateral glass opacity and/or consolidation superimposed on a background pattern consistent with fibrosing ILDs. Drawing on observations in IPF, it is suspected that epithelial injury or proliferation and autoimmunity are risk factors for acute exacerbation in ILDs that may present a progressive-fibrosing phenotype, but further studies are required. Current acute exacerbation management strategies are based on recommendations in IPF, but no randomised controlled trials of acute exacerbation management have been performed. Although there are no formal strategies to prevent the development of acute exacerbation, possible approaches include antifibrotic drugs (such as nintedanib and pirfenidone), and minimising exposure to infection, airborne irritants and pollutants. This review discusses the current knowledge of acute exacerbation of ILDs that may present a progressive-fibrosing phenotype and acknowledges limitations of the data available.

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Abo Elwafa, GihanS, NaglaaB Ahmed, AmanyA Abou Zeid e MaiA Abo Elhasab. "Noninvasive ventilation in acute exacerbation of interstitial lung diseases". Egyptian Journal of Chest Diseases and Tuberculosis 72, n. 1 (2023): 99. http://dx.doi.org/10.4103/ecdt.ecdt_40_22.

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Drakopanagiotakis, Fotios, Philipp Markart e Paschalis Steiropoulos. "Acute Exacerbations of Interstitial Lung Diseases: Focus on Biomarkers". International Journal of Molecular Sciences 24, n. 12 (15 giugno 2023): 10196. http://dx.doi.org/10.3390/ijms241210196.

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Interstitial lung diseases (ILDs) are a large group of pulmonary disorders characterized histologically by the cardinal involvement of the pulmonary interstitium. The prototype of ILDs is idiopathic pulmonary fibrosis (IPF), an incurable disease characterized by progressive distortion and loss of normal lung architecture through unchecked collagen deposition. Acute exacerbations are dramatic events during the clinical course of ILDs, associated with high morbidity and mortality. Infections, microaspiration, and advanced lung disease might be involved in the pathogenesis of acute exacerbations. Despite clinical scores, the prediction of the onset and outcome of acute exacerbations is still inaccurate. Biomarkers are necessary to characterize acute exacerbations better. We review the evidence for alveolar epithelial cell, fibropoliferation, and immunity molecules as potential biomarkers for acute exacerbations of interstitial lung disease.

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Drakopanagiotakis, Fotios, Ekaterina Krauss, Ira Michailidou, Vasileios Drosos, Stavros Anevlavis, Andreas Günther e Paschalis Steiropoulos. "Lung Cancer and Interstitial Lung Diseases". Cancers 16, n. 16 (13 agosto 2024): 2837. http://dx.doi.org/10.3390/cancers16162837.

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Lung cancer continues to be one of the leading causes of cancer-related death worldwide. There is evidence of a complex interplay between lung cancer and interstitial lung disease (ILD), affecting disease progression, management strategies, and patient outcomes. Both conditions develop as the result of common risk factors such as smoking, environmental exposures, and genetic predispositions. The presence of ILD poses diagnostic and therapeutic challenges in lung cancer management, including difficulties in interpreting radiological findings and increased susceptibility to treatment-related toxicities, such as acute exacerbation of ILD after surgery and pneumonitis after radiation therapy and immunotherapy. Moreover, due to the lack of large, phase III randomized controlled trials, the evidence-based therapeutic options for patients with ILDs and lung cancer remain limited. Antifibrotic treatment may help prevent pulmonary toxicity due to lung cancer treatment, but its effect is still unclear. Emerging diagnostic modalities and biomarkers and optimizing personalized treatment strategies are essential to improve outcomes in this patient population.

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Matsunashi, Atsushi, Kazuma Nagata, Takeshi Morimoto e Keisuke Tomii. "Mechanical ventilation for acute exacerbation of fibrosing interstitial lung diseases". Respiratory Investigation 61, n. 3 (maggio 2023): 306–13. http://dx.doi.org/10.1016/j.resinv.2023.01.008.

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Papanikolaou, Ilias C., Fotios Drakopanagiotakis e Vlasis S. Polychronopoulos. "Acute exacerbations of interstitial lung diseases". Current Opinion in Pulmonary Medicine 16, n. 5 (settembre 2010): 480–86. http://dx.doi.org/10.1097/mcp.0b013e32833ae49d.

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Akulkina, L. A., M. Yu Brovko, A. A. Schepalina, V. I. Sholomova, A. A. Kitbalian, A. S. Moiseev, P. P. Potapov e S. V. Moiseev. "A case of severe COVID-19 in a patient with progressive fibrosing interstitial lung disease". PULMONOLOGIYA 32, n. 5 (21 settembre 2022): 763–69. http://dx.doi.org/10.18093/0869-0189-2022-32-5-763-769.

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Interstitial lung diseases (ILDs) is a wide group of diffuse parenchymal lung diseases that can lead to interstitial pulmonary fibrosis. Clinical course of all ILDs, in particular with chronic fibrosing phenotype, can be complicated by an acute exacerbation caused by infection. Today, data about clinical course of COVID-19 in patients with progressive interstitial lung diseases is limited. In this case we described the course of COVID-19 infection in a patient with an interstitial pneumonia with autoimmune features (IPAF) and a progressive pulmonary fibrosis (PPF). We also discussed the typical features of COVID-19 in this population and the directions for further research.

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Magdy, Mohammed, Zainab Saaed, Emad Abd El-Aleem e Madyan Mahmoud. "Clinical and radiological signs of acute exacerbation of interstitial lung diseases". Minia Journal of Medical Research 30, n. 3 (1 luglio 2019): 116–19. http://dx.doi.org/10.21608/mjmr.2022.221906.

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Ito, Yoko, Gen Tazaki, Yusuke Kondo, Genki Takahashi e Fumio Sakamaki. "Therapeutic effect of nintedanib on acute exacerbation of interstitial lung diseases". Respiratory Medicine Case Reports 26 (2019): 317–20. http://dx.doi.org/10.1016/j.rmcr.2019.02.021.

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Suzuki, Atsushi, Yasuhiro Kondoh, Kevin K. Brown, Takeshi Johkoh, Kensuke Kataoka, Junya Fukuoka, Tomoki Kimura et al. "Acute exacerbations of fibrotic interstitial lung diseases". Respirology 25, n. 5 (19 agosto 2019): 525–34. http://dx.doi.org/10.1111/resp.13682.

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Amati, Francesco, Anna Stainer, Veronica Polelli, Marco Mantero, Andrea Gramegna, Francesco Blasi e Stefano Aliberti. "Efficacy of Pirfenidone and Nintedanib in Interstitial Lung Diseases Other than Idiopathic Pulmonary Fibrosis: A Systematic Review". International Journal of Molecular Sciences 24, n. 9 (25 aprile 2023): 7849. http://dx.doi.org/10.3390/ijms24097849.

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Pirfenidone and nintedanib are antifibrotic medications approved for idiopathic pulmonary fibrosis treatment by regulatory agencies and available for clinical use worldwide. These drugs have been shown to reduce the rate of decline in forced vital capacity and the risk of acute exacerbation among patients with idiopathic pulmonary fibrosis. Recent data suggest that different interstitial lung diseases with a progressive pulmonary fibrosis phenotype can share similar pathogenetic and biological pathways and could be amenable to antifibrotic therapies. Indeed, historical management strategies in interstitial lung disease have failed to identify potential treatments once progression has occurred despite available drugs. In this systematic review, we summarized data on the efficacy of pirfenidone and nintedanib in interstitial lung diseases other than idiopathic pulmonary fibrosis as well as ongoing and upcoming clinical trials. We identify two well-designed trials regarding nintedanib demonstrating the efficacy of this drug in slowing disease progression in patients with interstitial lung diseases other than idiopathic pulmonary fibrosis. On the other hand, results on the use of pirfenidone in interstitial lung diseases other than idiopathic pulmonary fibrosis should be interpreted with more caution on the basis of trial limitations. Several randomized control trials are underway to improve the quality of evidence in the interstitial lung disease field.

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On, Rintaro, Takemasa Matsumoto, Hisako Kushima, Ryosuke Hirano e Masaki Fujita. "Prevalence of viral infection in acute exacerbation of interstitial lung diseases in Japan". Respiratory Investigation 58, n. 6 (novembre 2020): 473–78. http://dx.doi.org/10.1016/j.resinv.2020.06.006.

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Salonen, Johanna, Minna Purokivi, Risto Bloigu e Riitta Kaarteenaho. "Prognosis and causes of death of patients with acute exacerbation of fibrosing interstitial lung diseases". BMJ Open Respiratory Research 7, n. 1 (aprile 2020): e000563. http://dx.doi.org/10.1136/bmjresp-2020-000563.

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BackgroundThe aim of this study was to compare the clinical characteristics, causes of death and factors impacting on the prognosis of patients with idiopathic pulmonary fibrosis (IPF) and other fibrosing interstitial lung disease (FILD) with a history of acute exacerbation (AE) of IPF or FILD.MethodsRetrospective data of hospital treatment periods caused by AE-IPF and AE-FILD were collected from medical records. Clinical features and survival data of IPF and non-IPF cases were evaluated and compared. The underlying and immediate causes of death were gathered from death certificates.ResultsA total of 128 patients fulfilled the criteria for inclusion. IPF (n=79/62%), rheumatoid arthritis-associated interstitial lung disease (RA-ILD; n=17/14%) and asbestosis (n=11/8.6%) were the most common FILD subgroups in the study. The median survival after hospitalisation in AE-IPF was 2.6 months compared with 21 months in other AE-FILDs (p<0.001). The survival difference was not explained by age, gender or pulmonary function test results at the time of hospitalisation. Patients with non-specific interstitial pneumonia and RA-ILD had the most favourable prognosis. ILD was the most common underlying cause of death in both patients with IPF and with other FILD accounting for 87% and 78% of deaths, respectively.ConclusionsWe detected a significantly longer survival in AE of patients with non-IPF compared with that of AE-IPFs. The prognosis of patients was affected by the underlying lung disease since pulmonary fibrosis was the underlying cause of death in the majority of all patients with FILD having experienced an AE.

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Enomoto, Noriyuki. "Pathological Roles of Pulmonary Cells in Acute Lung Injury: Lessons from Clinical Practice". International Journal of Molecular Sciences 23, n. 23 (30 novembre 2022): 15027. http://dx.doi.org/10.3390/ijms232315027.

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Interstitial lung diseases (ILD) are relatively rare and sometimes become life threatening. In particular, rapidly progressive ILD, which frequently presents as acute lung injury (ALI) on lung histopathology, shows poor prognosis if proper and immediate treatments are not initiated. These devastating conditions include acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF), clinically amyopathic dermatomyositis (CADM), epidermal growth factor receptor-tyrosine kinase inhibitor (EGFR-TKI)-induced lung injury, and severe acute respiratory syndrome coronavirus 2 (SARS-CoV2) infection named coronavirus disease 2019 (COVID-19). In this review, clinical information, physical findings, laboratory examinations, and findings on lung high-resolution computed tomography and lung histopathology are presented, focusing on majorly damaged cells in each disease. Furthermore, treatments that should be immediately initiated in clinical practice for each disease are illustrated to save patients with these diseases.

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Kaarteenaho, Riitta, e Vuokko L. Kinnula. "Diffuse Alveolar Damage: A Common Phenomenon in Progressive Interstitial Lung Disorders". Pulmonary Medicine 2011 (2011): 1–10. http://dx.doi.org/10.1155/2011/531302.

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It has become obvious that several interstitial lung diseases, and even viral lung infections, can progress rapidly, and exhibit similar features in their lung morphology. The final histopathological feature, common in these lung disorders, is diffuse alveolar damage (DAD). The histopathology of DAD is considered to represent end stage phenomenon in acutely behaving interstitial pneumonias, such as acute interstitial pneumonia (AIP) and acute exacerbations of idiopathic pulmonary fibrosis (IPF). Acute worsening and DAD may occur also in patients with nonspecific interstitial pneumonias (NSIPs), and even in severe viral lung infections where there is DAD histopathology in the lung. A better understanding of the mechanisms underlying the DAD reaction is needed to clarify the treatment for these serious lung diseases. There is an urgent need for international efforts for studying DAD-associated lung diseases, since the prognosis of these patients has been and is still dismal.

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Sato, Toshihiko, Satoshi Teramukai, Haruhiko Kondo, Atsushi Watanabe, Masahito Ebina, Kazuma Kishi, Yoshitaka Fujii et al. "Impact and predictors of acute exacerbation of interstitial lung diseases after pulmonary resection for lung cancer". Journal of Thoracic and Cardiovascular Surgery 147, n. 5 (maggio 2014): 1604–11. http://dx.doi.org/10.1016/j.jtcvs.2013.09.050.

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Austin, Adam, Johnny Jaber, Katherine Fu, Lauran Zeineddine, Mohamed Omballi, George Mckenney, Ramsy Abdelghani, Diana Espinoza, David Becnel e Hiren J. Mehta. "Acute exacerbation of interstitial lung diseases and mortality post-cryobiopsy: a multicenter cohort study". Journal of Thoracic Disease 16, n. 7 (luglio 2024): 4340–49. http://dx.doi.org/10.21037/jtd-24-270.

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Furukawa, Hiroshi, Shomi Oka, Kota Shimada, Atsushi Hashimoto, Akiko Komiya, Toshihiro Matsui e Shigeto Tohma. "Role of Deleterious Rare Alleles for Acute-Onset Diffuse Interstitial Lung Disease in Collagen Diseases". Clinical Medicine Insights: Circulatory, Respiratory and Pulmonary Medicine 13 (gennaio 2019): 117954841986644. http://dx.doi.org/10.1177/1179548419866443.

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Objective: Acute-onset diffuse interstitial lung disease (AoDILD) includes acute exacerbation of interstitial lung disease (ILD), drug-induced ILD, and Pneumocystis pneumonia in collagen diseases patients. As AoDILD causes a poor prognosis in collagen disease patients, the pathogenesis of AoDILD should be investigated. Exome sequencing studies revealed that rare variants were detected to be causative in some diseases. Recently reported upregulated genes in acute exacerbation of idiopathic pulmonary fibrosis could provide candidate genes for restricted exome analysis of AoDILD in collagen disease. Here, we investigated rare variants in the coding and boundary regions of these candidate genes in AoDILD. Methods: Deleterious rare variants in the coding and boundary regions of the candidate genes were analyzed by exome sequencing and the deleterious rare allele frequencies in AoDILD were compared with those of controls. Results: A significant association was detected for deleterious rare alleles in NPL ( P = .0044, Pc = .0399, odds ratio [OR] = 10.05, 95% confidence interval [CI] = 3.01-33.55). A deleterious rare allele frequency in the 9 candidate genes ( P = .0011, OR = 7.17, 95% CI = 2.80-18.33) was also increased in AoDILD in multigene panel analysis. The Krebs von den Lungen–6 (KL-6) levels in AoDILD patients with deleterious rare alleles were tended to be lower than those without ( P = .0168, Pc = .1509). Conclusions: The deleterious rare alleles in NPL were associated with AoDILD. In addition, the deleterious rare allele frequency in the 9 candidate genes was also increased in AoDILD. The deleterious rare alleles might contribute to the pathogenesis of AoDILD.

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Larsen, Brandon T., e Thomas V. Colby. "Update for Pathologists on Idiopathic Interstitial Pneumonias". Archives of Pathology & Laboratory Medicine 136, n. 10 (1 ottobre 2012): 1234–41. http://dx.doi.org/10.5858/arpa.2012-0225-ra.

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Context.—Idiopathic interstitial pneumonias are a subset of diffuse pulmonary interstitial diseases classified by international consensus in 2002 as idiopathic pulmonary fibrosis, nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, acute interstitial pneumonia, respiratory bronchiolitis interstitial lung disease, desquamative interstitial pneumonia, and lymphoid interstitial pneumonia. Each is associated with a characteristic histopathologic pattern. In 2011, updated consensus guidelines were released for diagnosis and management of idiopathic pulmonary fibrosis. The entire group of idiopathic interstitial pneumonias is currently undergoing refinement, with updates expected in a forthcoming consensus classification. Many of these recent and anticipated changes are relevant to pathologists. Objectives.—To review international consensus guidelines for diagnosis of idiopathic pulmonary fibrosis and other idiopathic interstitial pneumonias and to discuss recent and expected future classification updates. Data Sources.—Published peer-reviewed literature and personal experience of the authors. Conclusions.—Diagnosis of idiopathic interstitial pneumonias by multidisciplinary discussion among clinicians, radiologists, and pathologists is now strongly encouraged. Diagnosis of idiopathic pulmonary fibrosis no longer requires surgical lung biopsy; high-resolution computed tomography is an acceptable surrogate. In the context of clinical trials, pathologists are being asked to assign levels of confidence for histologic diagnosis of usual interstitial pneumonia in patients with idiopathic pulmonary fibrosis. Acute exacerbation of idiopathic pulmonary fibrosis is now accepted and should be considered when acute lung injury is superimposed on a background of usual interstitial pneumonia. The updated classification of idiopathic interstitial pneumonias will include a separate category for rare entities, including lymphoid interstitial pneumonia and idiopathic pleuroparenchymal fibroelastosis.

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Faverio, Paola, Anna Stainer, Sara Conti, Fabiana Madotto, Federica De Giacomi, Matteo Della Zoppa, Ada Vancheri et al. "Differences between Acute Exacerbations of Idiopathic Pulmonary Fibrosis and Other Interstitial Lung Diseases". Diagnostics 11, n. 9 (6 settembre 2021): 1623. http://dx.doi.org/10.3390/diagnostics11091623.

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Interstitial lung diseases (ILDs) comprise a wide group of pulmonary parenchymal disorders. These patients may experience acute respiratory deteriorations of their respiratory condition, termed “acute exacerbation” (AE). The incidence of AE-ILD seems to be lower than idiopathic pulmonary fibrosis (IPF), but prognosis and prognostic factors are largely unrecognized. We retrospectively analyzed a cohort of 158 consecutive adult patients hospitalized for AE-ILD in two Italian university hospitals from 2009 to 2016. Patients included in the analysis were divided into two groups: non-IPF (62%) and IPF (38%). Among ILDs included in the non-IPF group, the most frequent diagnoses were non-specific interstitial pneumonia (NSIP) (42%) and connective tissue disease (CTD)-ILD (20%). Mortality during hospitalization was significantly different between the two groups: 19% in the non-IPF group and 43% in the IPF group. AEs of ILDs are difficult-to-predict events and are burdened by relevant mortality. Increased inflammatory markers, such as neutrophilia on the differential blood cell count (HR 1.02 (CI 1.01–1.04)), the presence of pulmonary hypertension (HR 1.85 (CI 1.17–2.92)), and the diagnosis of IPF (HR 2.31 (CI 1.55–3.46)), resulted in negative prognostic factors in our analysis. Otherwise, lymphocytosis on the differential count seemed to act as a protective prognostic factor (OR 0.938 (CI 0.884–0.995)). Further prospective, large-scale, real-world data are needed to support and confirm the impact of our findings.

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Kimura, Toru, T. Nojiri, H. Hosoda, Y. Shintani, M. Inoue, M. Miyazato, M. Okumura e K. Kangawa. "P-227ESTABLISHMENT OF A NEW MOUSE MODEL FOR POSTOPERATIVE ACUTE EXACERBATION OF INTERSTITIAL LUNG DISEASES". Interactive CardioVascular and Thoracic Surgery 21, suppl_1 (agosto 2015): S62. http://dx.doi.org/10.1093/icvts/ivv204.227.

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Saraya, Takeshi, Hirokazu Kimura, Daisuke Kurai, Masaki Tamura, Yukari Ogawa, Sunao Mikura, Mitsuru Sada et al. "Clinical significance of respiratory virus detection in patients with acute exacerbation of interstitial lung diseases". Respiratory Medicine 136 (marzo 2018): 88–92. http://dx.doi.org/10.1016/j.rmed.2018.02.003.

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Salonen, Johanna, Hannu Vähänikkilä, Minna Purokivi e Riitta Kaarteenaho. "Causes of acute respiratory hospitalizations predict survival in fibrosing interstitial lung diseases". PLOS ONE 15, n. 11 (30 novembre 2020): e0242860. http://dx.doi.org/10.1371/journal.pone.0242860.

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Acute exacerbation of ILD (AE-ILD) is a common reason for hospitalization; it is also associated with significant mortality. Less is known about the prognostic significance of other events causing acute, non-elective hospitalizations in ILD patients. ILD patients hospitalized due to acute respiratory worsening were collected from medical records. Reasons for respiratory deterioration were classified into AE-ILDs and other causes. Clinical features and survival data of idiopathic pulmonary fibrosis (IPF) and other types of ILDs were evaluated and compared. In all, 237 patients (138 with IPF and 99 with other ILD) fulfilled the inclusion criteria. Of the non-IPF ILD types, the most prevalent subgroups were connective tissue disease-associated ILD (n = 33) and asbestosis (n = 22). The most common cause for hospitalization was AE-ILD explaining 41% of hospitalizations. Lower respiratory tract infection (22%), subacute progression of ILD (12%) and cardiovascular causes (7.2%) were other common reasons for hospital treatment. Patients with a lower respiratory tract infection had a more favorable prognosis compared with patients with AE-ILD. AE-ILDs were less fatal than cardiovascular or concurrent non-ILD-related causes for hospitalizations in non-IPF patients. High Gender-Age-Physiology (GAP) index was a marker for shortened survival and earlier AE-ILDs in all patients. IPF patients had a significantly shorter overall and post-hospitalization survival time compared with other ILDs. Most respiratory hospitalizations in ILD patients were related to causes other than AE-ILD, which highlights the importance of accurate differential diagnosis in order to target the appropriate treatment for each ILD patient.

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Banuls, Lorrain, Juliette Vanoverschelde, Fanny Garnier, Matthieu Amalric, Samir Jaber, Jonathan Charbit, Kevin Chalard et al. "Interstitial Lung Disease Worsens Short- and Long-Term Outcomes of Systemic Rheumatic Disease Patients Admitted to the ICU: A Multicenter Study". Journal of Clinical Medicine 10, n. 5 (3 marzo 2021): 1037. http://dx.doi.org/10.3390/jcm10051037.

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Critically ill patients with systemic rheumatic diseases (SRDs) have a fair prognosis, while those with interstitial lung disease (ILD) have a poorer outcome. However, the prognosis of SRD patients with ILD admitted to the intensive care unit (ICU) remains unclear. We conducted a case–control study to investigate the outcomes of critically ill SRD-ILD patients. Consecutive SRD-ILD patients admitted to five ICUs from January 2007 to December 2017 were compared to SRD patients without ILD. Mortality rates were compared between groups, and prognostic factors were then identified. One hundred and forty critically ill SRD patients were included in the study. Among the 70 patients with SRD–ILD, the SRDs were connective tissue diseases (56%), vasculitis (29%), sarcoidosis (13%), and spondylarthritis (3%). Patients were mainly admitted for acute exacerbation of SRD-ILD (36%) or infection (34%). ICU, in-hospital, and one-year mortality rates in SRD-ILD patients were higher than in SRD patients without ILD (n = 70): 40% vs. 16% (p < 0.01), 49% vs. 19% (p < 0.01), and 66% vs. 40% (p < 0.01), respectively. Hypoxemia, high sequential organ failure assessment (SOFA) score, and admission for ILD acute exacerbation were associated with ICU mortality. In conclusion, ILD worsened the outcomes of SRD patients admitted to the ICU. Admissions related to SRD-ILD acute exacerbation and the severity of the acute respiratory failure were associated with ICU mortality.

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von der Beck, Daniel, Friedrich Grimminger, Werner Seeger, Andreas Günther e Benjamin Löh. "Interstitial Lung Disease: Seasonality of Hospitalizations and In-Hospital Mortality 2005–2015". Respiration 101, n. 3 (8 ottobre 2021): 253–61. http://dx.doi.org/10.1159/000519214.

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Background: The overall incidence of interstitial lung disease and disease-associated mortality have been found on the rise. Hospitalizations for interstitial lung disease are typically caused by airway infection or the acute exacerbation of the underlying disease. Seasonal variance in ambient air pollution has recently been linked to exacerbation and mortality. We sought to examine the seasonal pattern of hospitalizations in Germany, use of mechanical ventilation, and in-hospital mortality on a year-by-year basis to identify their overall trend and to characterize seasonal patterns. Methods: The national in-patient database of the federal statistical office of Germany was searched for cases of interstitial lung disease. Results: A total of 130,366 hospitalizations for ILD occurred from 2005 to 2015. Time series data were examined for seasonality using X-11 statistics. The incidence of hospitalizations, mechanical ventilation, and in-hospital mortality show clear seasonal peaks in the cold season. The observed seasonality cannot be attributed to the variance of selected comorbidities. Also, there is a significant overall upward trend regarding hospitalization counts, especially in the use of non-invasive ventilation. Conclusion: Time series analysis of in-hospital data shows an ILD-related rise of hospitalizations, in-hospital mortality, and non-invasive ventilation. This emphasizes a growing importance of interstitial lung diseases for health-care systems. Strong seasonality is seen in these variables. Data therefore support previous studies of ILD exacerbation. More research on infectious causes and environmental factors is warranted.

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Anwar, Khadeeja, Ummara Siddique, Irfanullah Khan, Abdullah Safi, Hadia Abid, Ayesha Anwar e Karishma Israr. "Diagnostic Accuracy of Chest X-Ray in Interstitial Lung Diseases, Keeping High Resolution Computed Tomography Scan as Gold Standard". Journal of Gandhara Medical and Dental Science 11, n. 1 (31 dicembre 2023): 64–67. http://dx.doi.org/10.37762/jgmds.11-1.559.

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OBJECTIVES To determine the accuracy of the plain chest radiograph in diagnosing interstitial lung diseases (ILDs), keeping a high-resolution CT scan (HRCT) as the gold standard. METHODOLOGY A cross-sectional study was conducted. A total of 75 patients who visited the Department of Radiology department over two years were assessed by prospective analysis of their radiology reports. All the HRCTs and Chest X-ray images were reviewed. Data collected was recorded on a specially designed proforma and entered into Microsoft Excel and SPSS (Version 22.0. IBM Corp., Armonk, NY). Patients with a history of acute exacerbation of symptoms were excluded. RESULTSThe median age of the patients was 59 years, with SD 12.2. Chest radiographs detected interstitial lung disease (ILD) in 42/75 (56%).). The chest radiograph’s sensitivity, specificity, Positive Predictive Value (PPV), and Negative Predictive Value (NPV) were 76%, 84%, 86.3% and 76.7%. A plain chest X-ray's positive likelihood ratio (LR+) was 4.75, while the negative likelihood ratio (LR-) was 0.28. The overall accuracy of CXR was calculated as 78.6%. CONCLUSION Our study concluded that chest X-ray is the ideal initial investigation for diagnosing Interstitial lung disease (ILDS) with an accuracy of 78.6% compared to HRCT.

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Suda, Takafumi. "Up-to-Date Information on Rheumatoid Arthritis-Associated Interstitial Lung Disease". Clinical Medicine Insights: Circulatory, Respiratory and Pulmonary Medicine 9s1 (gennaio 2015): CCRPM.S23289. http://dx.doi.org/10.4137/ccrpm.s23289.

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Pulmonary involvement is common in rheumatoid arthritis (RA) and affects all the components of the lung. Interstitial lung disease (ILD) is the most predominant pulmonary manifestation and has been identified as the main cause of morbidity and mortality in RA. Clinically significant RA-ILD occurs in approximately 10% of RA patients. Several risk factors, such as old age, male gender, and smoking, have been reported to date. Histologically, the proportion of the usual interstitial pneumonia (UIP) pattern is higher in RA-ILD than in ILD associated with other connective tissue diseases, and RA-ILD also shows nonspecific interstitial pneumonia and organizing pneumonia patterns. High-resolution computed tomography scans are highly predictive of the histological UIP pattern with a specificity of 96%-100%. Acute exacerbation, which is the acute deterioration of the respiratory status characterized by newly developed bilateral infiltrates with unknown etiologies, has been reported in RA-ILD. Although acute exacerbation of RA-ILD has high mortality, similar to that of idiopathic pulmonary fibrosis, its incidence is lower in RA-ILD than in idiopathic pulmonary fibrosis. A consensus treatment has not yet been established. Current therapeutic regimens typically include corticosteroids with or without cytotoxic agents. Recent large longitudinal studies reported that the prognosis of RA-ILD was poor with a median survival of 2.6-3.0 years. Furthermore, histological and/or radiological patterns, such as UIP or non-UIP, have significant prognostic implications. RA-ILD patients with histological or radiological UIP patterns have poorer prognoses than those with non-UIP patterns. This review assessed the characteristics of RA-ILD by overviewing recent studies in the field and focused on the clinical significance of histological and/or radiological patterns in RA-ILD.

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Izuka, S., H. Yamashita, Y. Takahashi e H. Kaneko. "SAT0040 RISK FACTORS FOR DEVELOPING AND MORTALITY FOR ACUTE EXACERBATION OF RHEUMATOID ARTHRITIS-ASSOCIATED INTERSTITIAL LUNG DISEASE". Annals of the Rheumatic Diseases 79, Suppl 1 (giugno 2020): 950.2–951. http://dx.doi.org/10.1136/annrheumdis-2020-eular.411.

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Background:Among collagen vascular diseases, rheumatoid arthritis-associated interstitial lung disease (RA-ILD) is the most commonly associated with ILD with acute exacerbation (AE) [1]. One study reported that ILD diagnosis at an older age, the usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography, and methotrexate (MTX) use were associated with AE in patients with RA-ILD [2]. However, because these studies included few patients, the risk factors and prognosis of AE in patients with RA-ILD remain unclear. Therefore, this study examined the characteristics of RA-ILD patients with AE, and the variables associated with mortality due to AE of RA-ILD.Objectives:To investigate the risk factors for AE and mortality of RA-ILD.Methods:We retrospectively collected the clinical data of 165 RA-ILD patients admitted to our hospital between July 2010 and October 2019. We compared clinical characteristics between patients who developed AE (AE group) and those who did not (non-AE group), and identified the variables significantly associated with AE occurrence. We also compared the admission characteristics of those who survived (survivor group) and those who died (non-survivor group) after admission for AE. AE was defined using previously proposed criteria [3], which were modified slightly for application to RA-ILD.Results:The mean patient age was 73.6 ± 9.7 years and 97 (71.9%) patients were female. Thirty (22.2%) patients developed AE, of whom thirteen (43.3%) died (mean follow-up, 64.9 months). In univariate analyses UIP pattern and MTX were not associated with AE. However, in multivariate analyses, UIP pattern was associated with AE (OR 2.68, 95% CI 1.10–6.52,p=0.03). Median age (70vs. 80 years,p=0.003), non-use of MTX (70.6%vs. 23.1%,p=0.025), and C reactive protein level (median 9.38vs. 18.12 mg/dL,p=0.02) on admission were significantly higher in patients who died of AE. In the Cox proportional hazard model, UIP pattern (HR 4.67, 95% CI 1.02–21.5,p=0.048) and non-use of MTX (HR 0.16, 95% CI 0.04–0.72,p=0.016) were associated with death.Conclusion:Our data suggest that the UIP pattern is related to AE, and non-use of MTX and UIP pattern are related to death due to AE of RA-ILD.References:[1] Suda T, Kaida Y, Nakamura Y et al. Acute exacerbation of interstitial pneumonia associated with collagen vascular diseases.Respir Med2009;103:846-53.[2] Hozumi H, Nakamura Y, Johkoh T et al. Acute exacerbation in rheumatoid arthritis-associated interstitial lung disease: a retrospective case control study.BMJ Open2013;3:e003132.[3] Collard HR, Moore BB, Flaherty KR et al. Idiopathic pulmonary fibrosis clinical research network investigators. Acute exacerbations of idiopathic pulmonary fibrosis.Am J Respir Crit Care Med2007;176:636-43.Disclosure of Interests:None declared

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Kershaw, Corey D., Kiran Batra, Jose R. Torrealba e Lance S. Terada. "Characteristics and evaluation of acute exacerbations in chronic interstitial lung diseases". Respiratory Medicine 183 (luglio 2021): 106400. http://dx.doi.org/10.1016/j.rmed.2021.106400.

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Yasuda, Yuto, Takashi Nomizo, Hiroaki Ozasa, Tomoko Funazo, Takahiro Tsuji, Hironori Yoshida, Yuichi Sakamori et al. "Retrospective analysis of acute exacerbation of interstitial lung diseases with nanoparticle albumin-bound paclitaxel in patients with advanced lung cancer with preexisting interstitial lung disease". Molecular and Clinical Oncology 7, n. 4 (11 agosto 2017): 677–80. http://dx.doi.org/10.3892/mco.2017.1373.

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Cano-Jiménez, Esteban, Fernanda Hernández González e Guadalupe Peloche. "Comorbidities and Complications in Idiopathic Pulmonary Fibrosis". Medical Sciences 6, n. 3 (30 agosto 2018): 71. http://dx.doi.org/10.3390/medsci6030071.

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Though idiopathic pulmonary fibrosis (IPF) is characterized by single-organ involvement, many comorbid conditions occur within other organ systems. Patients with IPF may present during evolution different complications and comorbidities that influence the prognosis and modify the natural course of their disease. In this chapter, we highlight common comorbid conditions encountered in IPF, discuss disease-specific diagnostic modalities, and review the current treatment data for several key comorbidities. The diagnosis and treatment of these comorbidities is a challenge for the pulmonologist specialized in interstitial lung diseases (ILDs). We will focus on pulmonary emphysema, lung cancer, gastroesophageal reflux, pulmonary hypertension, obstructive sleep apnea (sleep disorders), and acute exacerbation of IPF.

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Manfredi, Andreina, Marco Sebastiani, Stefania Cerri, Caterina Vacchi, Roberto Tonelli, Giovanni Della Casa, Giulia Cassone et al. "Acute exacerbation of interstitial lung diseases secondary to systemic rheumatic diseases: a prospective study and review of the literature". Journal of Thoracic Disease 11, n. 4 (aprile 2019): 1621–28. http://dx.doi.org/10.21037/jtd.2019.03.28.

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Korolev, A. M. "Blood changes in chronic nonspecific lung diseases under the influence of hemotherapy". Kazan medical journal 50, n. 4 (31 marzo 2022): 67. http://dx.doi.org/10.17816/kazmj101353.

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We observed 50 patients aged 15 to 70 years. There were 15 people with prolonged acute pneumonia, 15 with bronchial asthma, 11 with chronic interstitial pneumonia, 9 with pneumosclerosis. There were 23 men, 27 women. Patients with chronic interstitial pneumonia also suffered from bronchiectasis, often multiple, with periodic exacerbations. Bronchial asthma in 15 people was accompanied by severe attacks in recent years, in a number of patients it was complicated by pneumosclerosis in the stage of emphysema and bronchiectasis.

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Kimura, Toru, Takashi Nojiri, Hiroshi Hosoda, Yasushi Shintani, Masayoshi Inoue, Mikiya Miyazato, Meinoshin Okumura e Kenji Kangawa. "Exacerbation of bleomycin-induced injury by lipopolysaccharide in mice: establishment of a mouse model for acute exacerbation of interstitial lung diseases". European Journal of Cardio-Thoracic Surgery 48, n. 4 (29 luglio 2015): e85-e91. http://dx.doi.org/10.1093/ejcts/ezv261.

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Lee, Jae Ha, Ji Hoon Jang, Jin Han Park, Hang-Jea Jang, Chan Sun Park, Sunggun Lee, Seong-Ho Kim, Ji Yeon Kim e Hyun Kuk Kim. "The role of interleukin-6 as a prognostic biomarker for predicting acute exacerbation in interstitial lung diseases". PLOS ONE 16, n. 7 (27 luglio 2021): e0255365. http://dx.doi.org/10.1371/journal.pone.0255365.

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Background Interstitial lung diseases (ILDs) are chronic, parenchymal lung diseases with a variable clinical course and a poor prognosis. Within various clinical courses, acute exacerbation (AE) is a devastating condition with significant morbidity and high mortality. The aim of this study was to investigate the role of interleukin-6 (IL-6) to predict AE and prognosis in patients with ILD. Methods Eighty-three patients who were diagnosed with ILD from 2016 to 2019 at the Haeundae Paik Hospital, Busan, South Korea, were included and their clinical data were retrospectively analyzed. Results The median follow-up period was 20 months. The mean age was 68.1 years and 65.1% of the patients were men with 60.2% of patients being ever-smokers. Among ILDs, idiopathic pulmonary fibrosis was the most common disease (68.7%), followed by connective tissue disease-associated ILD (14.5%), cryptogenic organizing pneumonia (9.6%), and nonspecific interstitial pneumonia (6.0%). The serum levels of IL-6 were measured at diagnosis with ILD and sequentially at follow-up visits. During the follow-ups, 15 (18.1%) patients experienced an acute exacerbation (AE) of ILD and among them, four (26.7%) patients died. In the multivariable analysis, high levels of IL-6 (OR 1.014, 95% CI: 1.001–1.027, p = 0.036) along with lower baseline saturations of peripheral oxygen (SpO2) were independent risk factors for AE. In the receiver operating characteristic curve analysis, the area under the curve was 0.815 (p < 0.001) and the optimal cut-off value of serum IL-6 to predict AE was 25.20 pg/mL with a sensitivity of 66.7% and specificity of 80.6%. In the multivariable Cox analysis, a high level of serum IL-6 (HR 1.007, 95% CI: 1.001–1.014, p = 0.018) was only an independent risk factor for mortality in ILD patients. Conclusions In our study, a high level of serum IL-6 is a useful biomarker to predict AE and poor prognosis in patients with ILD.

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Beghé, Bianca, Stefania Cerri, Leonardo M. Fabbri e Alessandro Marchioni. "COPD, Pulmonary Fibrosis and ILAs in Aging Smokers: The Paradox of Striking Different Responses to the Major Risk Factors". International Journal of Molecular Sciences 22, n. 17 (27 agosto 2021): 9292. http://dx.doi.org/10.3390/ijms22179292.

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Aging and smoking are associated with the progressive development of three main pulmonary diseases: chronic obstructive pulmonary disease (COPD), interstitial lung abnormalities (ILAs), and idiopathic pulmonary fibrosis (IPF). All three manifest mainly after the age of 60 years, but with different natural histories and prevalence: COPD prevalence increases with age to >40%, ILA prevalence is 8%, and IPF, a rare disease, is 0.0005–0.002%. While COPD and ILAs may be associated with gradual progression and mortality, the natural history of IPF remains obscure, with a worse prognosis and life expectancy of 2–5 years from diagnosis. Acute exacerbations are significant events in both COPD and IPF, with a much worse prognosis in IPF. This perspective discusses the paradox of the striking pathological and pathophysiologic responses on the background of the same main risk factors, aging and smoking, suggesting two distinct pathophysiologic processes for COPD and ILAs on one side and IPF on the other side. Pathologically, COPD is characterized by small airways fibrosis and remodeling, with the destruction of the lung parenchyma. By contrast, IPF almost exclusively affects the lung parenchyma and interstitium. ILAs are a heterogenous group of diseases, a minority of which present with the alveolar and interstitial abnormalities of interstitial lung disease.

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Yamakawa, Hideaki, Takashi Ogura, Hideto Kameda, Tomoo Kishaba, Tae Iwasawa, Tamiko Takemura e Kazuyoshi Kuwano. "Decision-Making Strategy for the Treatment of Rheumatoid Arthritis-Associated Interstitial Lung Disease (RA-ILD)". Journal of Clinical Medicine 10, n. 17 (25 agosto 2021): 3806. http://dx.doi.org/10.3390/jcm10173806.

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Rheumatoid arthritis (RA) is a common type of autoimmune arthritis. Patient clinical outcomes might be influenced by numerous respiratory diseases, but interstitial lung disease (ILD) is the most important comorbidity. RA-associated ILD (RA-ILD) is divided into acute/subacute and chronic forms. In the acute/subacute course, if the disease is severe as indicated by a diffuse alveolar damage pattern, high-dose corticosteroids combined with antimicrobial agents should be promptly initiated while considering the differential diagnoses, primarily acute exacerbation (AE) of RA-ILD, drug-induced pneumonitis, and Pneumocystis pneumonia. As initial therapeutic management in the chronic course, the RA itself should be stabilized without delay; thereafter, the activity of ILD itself can be stabilized, considering the safety of each anti-rheumatic drug. The formation of the usual interstitial pneumonia (UIP) pattern is the most important determinant because lung function can worsen more quickly with this pattern. However, because clinicians can fail to identify specific radiological patterns, it is important to determine whether each patient with RA-ILD has UIP-like lesions such as subpleural reticulation, traction bronchiectasis, and honeycombing especially progressively enlarged cysts. In patients with progressive RA-ILD and high risk for infection or AE of ILD in whom fibrosis is dominant, clinicians should consider starting an anti-fibrotic agent.

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Ricci, Alberto, Alessandra Pagliuca, Morgana Vermi, Dario Pizzirusso, Marta Innammorato, Raffaele Sglavo, Francesco Scarso et al. "The Role of Lung Colonization in Connective Tissue Disease-Associated Interstitial Lung Disease". Microorganisms 9, n. 5 (27 aprile 2021): 932. http://dx.doi.org/10.3390/microorganisms9050932.

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Connective tissue diseases (CTDs) may frequently manifest with interstitial lung disease (ILD), which may severely impair quality and expectation of life. CTD-ILD generally has a chronic clinical course, with possible acute exacerbations. Although several lines of evidence indicate a relevant role of infections in the acute exacerbations of CTD-ILD, little information is available regarding the prevalence of infections in chronic CTD-ILD and their possible role in the clinical course. The aim of the present retrospective study was the identification of lung microbial colonization in broncho-alveolar lavage from patients affected by stable CTD-ILD with radiologically defined lung involvement. We demonstrated that 22.7% of patients with CTD-ILD display microbial colonization by Pseudomonas aeruginosa, Haemophilus influenzae, and non-tuberculous mycobacteria. Moreover, these patients display a major radiologic lung involvement, with higher impairment in lung function tests confirmed in a multivariate logistic regression analysis. Overall, the present study provides new information on lung colonization during CTD-ILD and its possible relationship with lung disease progression and severity.

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Nakashima, Kazuki, Toyoshi Yanagihara, Sae Ishida, Naruhiko Ogo, Ayaka Egashira, Tatsuma Asoh e Takashige Maeyama. "Three cases of sequential treatment with nintedanib following pulsed-dose corticosteroids for acute exacerbation of interstitial lung diseases". Respiratory Medicine Case Reports 33 (2021): 101385. http://dx.doi.org/10.1016/j.rmcr.2021.101385.

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Manfredi, Andreina, Marco Sebastiani, Stefania Cerri, Caterina Vacchi, Roberto Tonelli, Giovanni Della Casa, Giulia Cassone et al. "Erratum to acute exacerbation of interstitial lung diseases secondary to systemic rheumatic diseases: a prospective study and review of the literature". Journal of Thoracic Disease 12, n. 10 (ottobre 2020): 6411. http://dx.doi.org/10.21037/jtd-2020-64.

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Clement, Annick, Jacques de Blic, Ralph Epaud, Laurie Galeron, Nadia Nathan, Alice Hadchouel, Angelo Barbato et al. "Management of children with interstitial lung diseases: the difficult issue of acute exacerbations". European Respiratory Journal 48, n. 6 (30 novembre 2016): 1559–63. http://dx.doi.org/10.1183/13993003.01900-2016.

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Rodrigues, Inês, Ricardo Estêvão Gomes, Lígia Maria Coutinho, Maria Teresa Rego, Firmino Machado, António Morais e Helder Novais Bastos. "Diagnostic yield and safety of transbronchial lung cryobiopsy and surgical lung biopsy in interstitial lung diseases: a systematic review and meta-analysis". European Respiratory Review 31, n. 166 (5 ottobre 2022): 210280. http://dx.doi.org/10.1183/16000617.0280-2021.

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Introduction:Transbronchial lung cryobiopsy (TBLC) is increasingly being used as an alternative to video-assisted thoracoscopic surgery (VATS) biopsy to establish the histopathologic pattern in interstitial lung disease (ILD).Methods:A systematic literature search of the PubMed and Embase databases, from October 2010 to October 2020, was conducted to identify studies that reported on diagnostic yield or safety of VATS or TBLC in the diagnosis of ILD.Results:43 studies were included. 23 evaluated the diagnostic yield of TBLC after multidisciplinary discussion, with a pooled diagnostic yield of 76.8% (95% confidence interval (CI) 70.6–82.1), rising to 80.7% in centres that performed ≥70 TBLC. 10 studies assessed the use of VATS and the pooled diagnostic yield was 93.5% (95% CI 88.3–96.5). In TBLC, pooled incidences of complications were 9.9% (95% CI 6.8–14.3) for significant bleeding (6.9% for centres with ≥70 TBLC), 5.6% (95% CI 3.8–8.2) for pneumothorax treated with a chest tube and 1.4% (95% CI 0.9–2.2) for acute exacerbation of ILD after TBLC. The mortality rates were 0.6% and 1.7% for TBLC and VATS, respectively.Conclusions:TBLC has a fairly good diagnostic yield, an acceptable safety profile and a lower mortality rate than VATS. The best results are obtained from more experienced centres.

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Araujo, Mariana Sponholz, Frederico Leon Arrabal Fernandes, Fernando Uliana Kay e Carlos Roberto Ribeiro Carvalho. "Pneumomediastinum, subcutaneous emphysema, and pneumothorax after a pulmonary function testing in a patient with bleomycin-induced interstitial pneumonitis". Jornal Brasileiro de Pneumologia 39, n. 5 (settembre 2013): 613–19. http://dx.doi.org/10.1590/s1806-37132013000500012.

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Spontaneous pneumomediastinum is an uncommon event, the clinical picture of which includes retrosternal chest pain, subcutaneous emphysema, dyspnea, and dysphonia. The pathophysiological mechanism involved is the emergence of a pressure gradient between the alveoli and surrounding structures, causing alveolar rupture with subsequent dissection of the peribronchovascular sheath and infiltration of the mediastinum and subcutaneous tissue with air. Known triggers include acute exacerbations of asthma and situations that require the Valsalva maneuver. We described and documented with HRCT scans the occurrence of pneumomediastinum after a patient with bleomycin-induced interstitial lung disease underwent pulmonary function testing. Although uncommon, the association between pulmonary function testing and air leak syndromes has been increasingly reported in the literature, and lung diseases, such as interstitial lung diseases, include structural changes that facilitate the occurrence of this complication.

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Inotani, S., Y. Kondo, S. Takanashi, S. Ishigaki, S. Saito, M. Takeshita, J. Kikuchi, H. Hanaoka, K. Suzuki e Y. Kaneko. "POS1220 TRANSITION IN SYMPTOMS, ORGAN INVOLVEMENT, AND CLINICAL DIAGNOSIS OF PATIENTS POSITIVE FOR ANTI-ARS ANTIBODIES". Annals of the Rheumatic Diseases 82, Suppl 1 (30 maggio 2023): 945.1–945. http://dx.doi.org/10.1136/annrheumdis-2023-eular.1345.

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BackgroundMyositis-specific autoantibodies are useful biomarkers to make a diagnosis and predict prognosis of idiopathic inflammatory myopathies. Anti-aminoacyl transfer RNA synthetase antibodies (anti-ARS) is one of the most representative myositis-specific autoantibodies, however, organ involvement in patients positive for anti-ARS is not limited to the muscles but also skin, joints, and lungs, which constructs spectrums of idiopathic inflammatory myopathies called anti-synthetase syndrome. While individual anti-ARS antibodies have been known to form distinct clinical subsets, little is known regarding their chronological clinical presentation patterns and diagnosis during clinical course.ObjectivesTo clarify the clinical characteristics of patients positive for anti-ARS and transition in manifestations and diagnosis during their clinical courses.MethodsWe reviewed consecutive patients with anti-ARS who had visited our hospital between 1998 and 2022 retrospectively. Anti-ARS antibodies were detected and categorized with RNA immunoprecipitation assays. We collected patient clinical characteristics and laboratory parameters including transition of diagnosis, manifestations and, organ involvement from their medical records chronologically. All statistical analyses were performed using JMP 15 (SAS Institute Inc., Cary, NC, USA).ResultsWe included 97 patients positive for anti-ARS in the analysis. The mean age was 53.2 years old, and 73 (75%) were female. The types of anti-ARS Ab were anti-Jo-1 (37%), anti-EJ (32%), anti-PL-7 (11%), anti-PL-12 (12%), anti-KS (4%), and anti-OJ (3%). Half of the patients had co-existing other autoantibodies such as anti-SS-A/Ro, anti-SS-B/La, anti-dsDNA, anti-RNP, and/or rheumatoid factor, but the combination of antibodies was not different among each anti-ARS. The initial diagnoses of the patients were polymyositis (22%), dermatomyositis (33%), amyopathic dermatomyositis (13%), and interstitial pneumonia with autoimmune features (27%). Raynaud’s phenomenon were observed in 25% of the patients; 19% in patients with anti-Jo-1, 36% in anti-EJ, 11% in anti-PL-7, 30% in anti-PL-12, 25% in anti-KS, and 33% in anti-OJ antibodies. Interstitial lung disease was observed in all patients except for three (two with anti-Jo-1 and one with anti-PL-12). Myositis was diagnosed initially in 72% of patients with anti-Jo-1, 50% with anti-EJ, 40% with anti-PL-7, 42% with anti-PL-12, none with anti-KS, and 67% with anti-OJ antibodies, but additional 6%, 4%, 40%, 8%, 0%, and 0% with those individual antibodies, respectively, were diagnosed with myositis during the mean observation period of 9.6 years. Acute or subacute exacerbation of interstitial lung disease requiring immunosuppressive treatment intensification were observed in 28% of patients with anti-Jo-1, 33% with anti-EJ, 40% with anti-PL-7, 8% with anti-PL-12, 33% with anti-KS, and 67% with anti-OJ antibodies.ConclusionAlmost all patients positive for anti-ARS positive had interstitial lung disease irrelevant of myositis diagnosis. Patients with anti-Jo-1 were suffering from typical myositis complicated with interstitial lung diseases at initial diagnosis, while patients with anti PL-7 tended to have precedent interstitial lung disease followed by development of myositis during clinical courses. The incidence of acute exacerbation of interstitial lung disease was the lowest in patients with anti-PL12.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of InterestsNone Declared.

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van den Bosch, Laura, Fabrizio Luppi, Giovanni Ferrara e Marco Mura. "Immunomodulatory treatment of interstitial lung disease". Therapeutic Advances in Respiratory Disease 16 (gennaio 2022): 175346662211170. http://dx.doi.org/10.1177/17534666221117002.

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Interstitial lung diseases (ILDs) other than idiopathic pulmonary fibrosis (IPF) have an array of immunomodulatory treatment options compared with IPF, due to their inflammatory component. However, there is a relative paucity of guidance on the management of this heterogeneous group of diseases. In ILDs other than IPF, immunosuppression is the cornerstone of therapy, with varying levels of evidence for different immunomodulatory agents and for each specific ILD. Classification of ILDs is important for guiding treatment decisions. Immunomodulatory agents mainly include corticosteroids, mycophenolate mofetil (MMF), azathioprine, methotrexate, cyclophosphamide and rituximab. In this review, the available evidence for single agents in the most common ILDs is first discussed. We then reviewed practical therapeutic approaches in connective tissue disease–related ILD and interstitial pneumonia with autoimmune features, scleroderma-related ILD, vasculitis and dermatomyositis with hypoxemic respiratory failure, idiopathic non-specific interstitial pneumonia, hypersensitivity pneumonitis sarcoidosis, fibrosing organizing pneumonia and eosinophilic pneumonia. The treatment of acute exacerbations of ILD is also discussed. Therapy augmentation in ILD is dictated by the recognition of progression of disease. Criteria for the evaluation of progression of disease are then discussed. Finally, specific protocol and measures to increase patients’ safety are reviewed as well, including general monitoring and serologic surveillance, Pneumocystis jirovecii prophylaxis, patients’ education, genetic testing for azathioprine, MMF serum levels and cyclophosphamide administration protocols. Immunomodulatory therapies are largely successful in the management of ILDs and can be safely managed with the application of specific protocols, precautions and monitoring.

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Tandjaoui-Lambiotte, Y., F. Gonzalez, M. Boubaya, O. Freynet, C. Clec´h, N. Bonnet, G. Van Der Meersch et al. "Two-year follow-up of 196 interstitial lung disease patients after ICU stay". International Journal of Tuberculosis and Lung Disease 25, n. 3 (1 marzo 2021): 199–205. http://dx.doi.org/10.5588/ijtld.20.0706.

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OBJECTIVE: Interstitial lung diseases (ILDs) are associated with poor prognosis in the intensive care unit (ICU). We aimed to assess factors associated with hospital mortality in ILD patients admitted to the ICU and to investigate long-term outcome.MATERIAL AND METHODS: This was a retrospective study in a teaching hospital specialised in ILD management. Patients with ILD who were hospitalised in the ICU between 2000 and 2014 were included. Independent predictors of hospital mortality were identified using logistic regression.RESULTS: A total of 196 ILD patients were admitted to the ICU during the study period. Overall hospital mortality was 55%. Two years after ICU admission, 70 (36%) patients were still alive. Of the 196 patients, 108 (55%) required invasive mechanical ventilation, of whom 21 (20%) were discharged alive from hospital. Acute exacerbation of ILD and multi-organ failure were highly associated with hospital mortality (OR 5.4, 95% CI 1.9–15.5 and OR 12.6, 95% CI 4.9–32.5, respectively).CONCLUSION: Hospital mortality among ILD patients hospitalised in the ICU was high, but even where invasive mechanical ventilation was required, a substantial number of patients were discharged alive from hospital. Multi-organ failure could lead to major ethical concerns.

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Laneri, Alessia, Stefania Cerri, Giovanni Della Casa, Antonio Moretti, Andreina Manfredi, Marco Sebastiani, Enrico Clini e Carlo Salvarani. "COVID-19, A New Possible Mimicker of Interstitial Lung Disease Related to Primary Sjögren’s Syndrome". Case Reports in Medicine 2023 (9 dicembre 2023): 1–6. http://dx.doi.org/10.1155/2023/9915553.

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Introduction. Acute exacerbation of interstitial lung disease (ILD) and COVID-19 pneumonia show many similarities, but also COVID-19 sequelae, mainly when fibrotic features are present, can be difficult to distinguish from chronic ILD observed in connective tissue diseases. Case Report. In 2018, a 52-year-old woman, was diagnosed with primary Sjogren’s syndrome (pSS). The patient did not show respiratory symptoms, and a chest X-ray was normal. During March 2020, the patient was hospitalized for acute respiratory failure related to COVID-19 pneumonia. Three months later, follow-up chest high-resolution computed tomography (HRCT) showed ground glass opacity (GGO) and interlobular interstitial thickening. Pulmonary function tests (PFTs) showed slight restrictive deficit and mild reduction in diffusion lung of carbon monoxide (DLCO). The patient complained of asthenia and exertional dyspnoea. A multidisciplinary discussion including rheumatologist, pulmonologist, and thoracic radiologist did not allow a definitive differential diagnosis between COVID-19 persisting abnormalities and a previous or new-onset pSS-ILD. A “wait and see” approach was decided, monitoring clinical conditions, PFTs, and chest HRCT over time. Only 2 years after the hospitalization, improvement of clinical symptoms was reported; PFT also improved, and HRCT showed almost complete resolution of GGO and interlobular interstitial thickening, confirming the diagnostic hypothesis of long-COVID lung manifestations. Discussion. In the above-reported case report, 3 differential diagnoses were possible: a COVID-19-related ILD, a preexisting pSS-ILD, or a new-onset pSS-ILD triggered by COVID-19. Regardless of the diagnosis, the persistence of clinical and PFT alterations, suggested a chronic disease but, surprisingly, clinical and radiologic manifestations disappeared 2 years later.

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Han, Ji-yeon, e Jae Ha Lee. "Application and Interpretation of High-Resolution Computed Tomography for the Diagnosis of Interstitial Lung Disease (ILD): ILD Protocol". Korean Journal of Medicine 98, n. 6 (1 dicembre 2023): 294–99. http://dx.doi.org/10.3904/kjm.2023.98.6.294.

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Interstitial lung disease (ILD) encompasses a heterogeneous group of over 200 diffuse parenchymal lung diseases. Accurate diagnosis of ILD is essential for appropriate treatment but can be challenging to achieve. For patients with suspected ILD, high-resolution computed tomography (HRCT) is a minimally invasive approach that allows for convenient repeat imaging. Compared to a conventional computed tomography (CT) protocol, the HRCT protocol for ILD patients involves two additional series with different technical requirements: a prone inspiration scan, and a supine expiration scan. The prone scan is useful when dependent opacification is observed on a supine chest CT scan. It facilitates the diagnosis of honeycombing, reducing observer variation in diagnosing idiopathic pulmonary fibrosis. The expiratory scan is useful for identifying air trapping, which is essential for the diagnosis of hypersensitivity pneumonitis. It is used to assess the three-density sign, which is specific for hypersensitivity pneumonitis and characterized by patchy distribution of normal-appearing lobules, ground glass opacities, and lobules with reduced lung density and vessel size. The HRCT ILD protocol should be performed regularly to assess changes in the extent of fibrosis and to diagnose lung cancer or acute exacerbation when clinically indicated.

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Sharma, Neha, Chandran Nagaraj, Bence M. Nagy, Leigh M. Marsh, Natalie Bordag, Diana Zabini, Malgorzata Wygrecka et al. "RGS5 Determines Neutrophil Migration in the Acute Inflammatory Phase of Bleomycin-Induced Lung Injury". International Journal of Molecular Sciences 22, n. 17 (28 agosto 2021): 9342. http://dx.doi.org/10.3390/ijms22179342.

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Abstract (sommario):

The regulator of G protein signaling (RGS) represents a widespread system of controllers of cellular responses. The activities of the R4 subfamily of RGSs have been elucidated in allergic pulmonary diseases. However, the R4 signaling in other inflammatory lung diseases, with a strong cellular immune response, remained unexplored. Thus, our study aimed to discern the functional relevance of the R4 family member, RGS5, as a potential modulating element in this context. Gene profiling of the R4 subfamily showed increased RGS5 expression in human fibrosing lung disease samples. In line with this, RGS5 was markedly increased in murine lungs following bleomycin injury. RGS knock-out mice (RGS-/-) had preserved lung function while control mice showed significant combined ventilatory disorders three days after bleomycin application as compared to untreated control mice. Loss of RGS5 was associated with a significantly reduced neutrophil influx and tissue myeloperoxidase expression. In the LPS lung injury model, RGS5-/- mice also failed to recruit neutrophils into the lung, which was accompanied by reduced tissue myeloperoxidase levels after 24 h. Our in-vitro assays showed impaired migration of RGS5-/- neutrophils towards chemokines despite preserved Ca2+ signaling. ERK dephosphorylation might play a role in reduced neutrophil migration in our model. As a conclusion, loss of RGS5 preserves lung function and attenuates hyperinflammation in the acute phase of bleomycin-induced pulmonary fibrosis and LPS-induced lung injury. Targeting RGS5 might alleviate the severity of exacerbations in interstitial lung diseases.

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