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Journal articles on the topic 'IgA Vasculitis'

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Author: Grafiati

Published: 25 July 2024

Last updated: 31 July 2024

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1

Sugino, Hitomi, Yu Sawada, and Motonobu Nakamura. "IgA Vasculitis: Etiology, Treatment, Biomarkers and Epigenetic Changes." International Journal of Molecular Sciences 22, no. 14 (July 14, 2021): 7538. http://dx.doi.org/10.3390/ijms22147538.

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IgA, previously called Henoch-Schönlein vasculitis, is an essential immune component that drives the host immune response to the external environment. As IgA has the unique characteristic of a flexible response to broad types of microorganisms, it sometimes causes an autoreactive response in the host human body. IgA vasculitis and related organ dysfunction are representative IgA-mediated autoimmune diseases; bacterial and viral infections often trigger IgA vasculitis. Recent drug developments and the presence of COVID-19 have revealed that these agents can also trigger IgA vasculitis. These findings provide a novel understanding of the pathogenesis of IgA vasculitis. In this review, we focus on the characteristics of IgA and symptoms of IgA vasculitis and other organ dysfunction. We also mention the therapeutic approach, biomarkers, novel triggers for IgA vasculitis, and epigenetic modifications in patients with IgA vasculitis.

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Sotoodian, Bahman, Janet Robert, Muhammad N. Mahmood, and Elaine Yacyshyn. "IgA Cutaneous Purpura Post–Renal Transplantation in a Patient With Long-Standing IgA Nephropathy." Journal of Cutaneous Medicine and Surgery 19, no. 5 (April 15, 2015): 498–503. http://dx.doi.org/10.1177/1203475415582135.

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Background: IgA vasculitis is a small-vessel vasculitis caused by deposition of IgA antibodies in tissues. IgA nephropathy and IgAV have long been considered related conditions. Objective: To assess the prevalence and implications of new-onset Henoch-Schönlein purpura (HSP) after renal transplant in patients with underlying IgA nephropathy. Methods: The PubMed database was searched for keywords such as IgAV, IgA vasculitis, Henoch-Schönlein purpura, HSP, IgA nephropathy, and renal transplant. Results: Two cases of new-onset IgA vasculitis post–renal transplant after stopping the prednisone or receiving seasonal influenza vaccine have been reported. We report the case of new-onset IgA cutaneous vasculitis in a renal transplant patient with IgA nephropathy after reduction in his prednisone dosage. Conclusion: The new development of cutaneous IgA vasculitis is unusual in renal transplant patients with IgA nephropathy. Despite these patients’ being immunosuppressed, the presence of IgA vasculitis could signal the recurrence of IgA nephropathy.

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Levanon, S., V. Gotlieb, Y. Kraus, I. Novofastovski, S. Brikman, A. Fawaz, M. Aghbariyya, et al. "POS0831 IgA VASCULITIS IN ADULTS, PEDIATRICS AND NON-VASCULITIC IgA NEPHROPATHY." Annals of the Rheumatic Diseases 81, Suppl 1 (May 23, 2022): 706.2–707. http://dx.doi.org/10.1136/annrheumdis-2022-eular.3368.

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BackgroundIgA vasculitis (IgAV) has been extensively studied in children, while its natural history remains poorly studied in adults. Sparse data comparing childhood and adult-onset disease has shown significant differences in their clinical presentation, especially in the severity of renal involvement, which accounts for the major long-term morbidity. IgAV shares similar renal histologic features with IgA nephropathy (IgAN), while clinically IgAN is a chronic kidney disease which may lead to end stage renal disease and dialysis. The extent of kidney injury among adults with IgAV is still a matter of uncertainty.ObjectivesWe aimed to evaluate clinical manifestations, laboratory data, treatment patterns and long-term outcomes of pediatric and adult-onset IgAV in comparison to IgAN.MethodsThis retrospective collaborative study examined medical records of adults and children with IgAV and IgAN adult patients admitted to rheumatology clinic and in hospital pediatric departments in a 13-year period (2007-2019). Diagnosis of adults with IgAV relied on the Ankara criteria and was confirmed by a consistent skin biopsy with positive IgA staining by immunofluorescence. Children with IgAV were included in our study on a clinical basis. All IgAN patients had a kidney biopsy proven disease. We analyzed and compared frequencies of clinical manifestations, laboratory findings, treatment regimens and long-term outcomes at one year follow-up. Finally, we assessed long term outcomes, such as time to dialysis and all-cause mortality, till the end of the follow-up time.ResultsA total of 60 adult IgAV, 60 pediatric IgAV and 45 IgAN patients were included in our study. There were significantly more males in the IgAN group compared to the adult and pediatric IgAV groups (77.8%, 41.7% and 55% respectively, p=0.01). Adult IgAV patients were significantly older than IgAN patients (53.1±17.4 years vs. 45.1±15.7 years, p=0.02) and had significantly higher rates of diabetes (43.3% vs. 24.4%, p=0.05) and ischemic heart disease (18.3% vs. 4.4%, p=0.03). The pediatric IgAV group had a statistically higher rate of previous infection compared to the adult IgAV group (44.8% vs. 20%, p=0.02). At one year follow-up, IgAN patients had higher levels of serum creatinine compared to the adult IgAV group (2.002 vs. 1.100, p<0.01). Data observed until the end of the follow-up time showed no difference in time to dialysis (IgAV adults: 9.8-12.4 years, IgAN: 5.0-6.6 years, p>.41). Nevertheless, IgAV adult patients had significantly shorter survival time (5.5 years, 95% CI: 4.8-6.2 years) than IgAN patients (7.0 years, 95% CI: 6.6-7.5 years, p<.01).ConclusionThis retrospective study demonstrates that IgAV in adults presents substantial clinical manifestations, including high risk of progression to persistent renal impairment and possesses higher mortality rate in comparison with pediatric-onset disease and IgAN.References[1]Blanco R, Martínez-Taboada VM, Rodríguez-Valverde V, García-Fuentes M, González-Gay MA. Henoch-Schönlein purpura in adulthood and childhood: two different expressions of the same syndrome. Arthritis Rheum. 1997 May;40(5):859-64. doi: 10.1002/art.1780400513. PMID: 9153547.[2]Nossent J, Raymond W, Isobel Keen H, Preen D, Inderjeeth C. Morbidity and mortality in adult-onset IgA vasculitis: a long-term population-based cohort study. Rheumatology (Oxford). 2021 Dec 24;61(1):291-298. doi: 10.1093/rheumatology/keab312. PMID: 33779729.Figure 1.Disclosure of InterestsNone declared

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4

Marro, Julien, Andrew J. Chetwynd, Samuel Edwards, Rachael D. Wright, and Louise Oni. "Increased Urinary IgA in Paediatric IgA Vasculitis Nephritis." International Journal of Molecular Sciences 23, no. 23 (November 22, 2022): 14548. http://dx.doi.org/10.3390/ijms232314548.

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IgA vasculitis (IgAV) is the most common form of paediatric vasculitis, with up to 50% of patients experiencing kidney inflammation. Much remains unknown about IgAV, but it is believed to arise due to galactose-deficient IgA1 promoting an auto-inflammatory response. This study assesses whether urinary IgA can be detected in children with IgAV to allow further evaluation of IgA1 and whether it has any relationship with nephritis. Urinary and serum IgA concentrations were measured using commercially available ELISA kits. Patients were grouped into IgAV nephritis (IgAVN) or IgAV without nephritis (IgAVwoN). Fifty-nine children were included: IgAVN n = 12, IgAVwoN n = 35, and healthy controls (HC) n = 12, with a mean age of 8.2 ± 4.1 years. Urinary IgA concentrations were statistically significantly higher in patients with IgAV (107.1 ± 136.3 μg/mmol) compared to HC (50.6 ± 26.3 μg/mmol; p = 0.027) and IgAVN (229.8 ± 226.3 μg/mmol) compared to both IgAVwoN (65.0 ± 37.8 μg/mmol; p = 0.002) and HC (p < 0.001). Urinary IgA concentrations were able to distinguish between renal status (AUC 0.838, 95%CI [0.704–0.973], p < 0.001) and did not correlate with proteinuria (r = 0.124; p = 0.407). Urinary IgA concentrations are increased in children with IgAVN, and it has the potential to act as a non-invasive biofluid to further evaluate nephritis in this disease.

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5

Nüsken, Eva, and Lutz T. Weber. "IgA vasculitis nephritis." Current Opinion in Pediatrics 34, no. 2 (February 7, 2022): 209–16. http://dx.doi.org/10.1097/mop.0000000000001120.

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6

Ozen, Seza, and Erdal Sag. "Childhood vasculitis." Rheumatology 59, Supplement_3 (April 29, 2020): iii95—iii100. http://dx.doi.org/10.1093/rheumatology/kez599.

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Abstract Vasculitis is a challenging disease for paediatricians. Certain vasculitides are quite common in children whereas others are much rarer compared with adults. The most common vasculitides in childhood are IgA-associated vasculitis (Henoch–Schönlein purpura) and Kawasaki disease, which are usually self-limiting vasculitides although children do develop complications as a result. We now have much better knowledge of how to manage these patients and prevent the deleterious complications. This review provides an up-to-date discussion on childhood vasculitides, including diagnosis, treatment and follow-up strategies, together with a comparison with vasculitides in adults. It also discusses the newly defined monogenic vasculitides that often present during early childhood.

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Winkler, Clay, Raymond Dobson, and Michael Tranovich. "Low Back Pain and Swelling as an Atypical Presentation of IgA Vasculitis." Clinical Practice and Cases in Emergency Medicine 4, no. 2 (April 14, 2020): 241–43. http://dx.doi.org/10.5811/cpcem.2019.11.44574.

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Introduction: Immunoglobulin A vasculitis (IgA vasculitis), formerly Henoch-Schonlein purpura, is the most common vasculitis in children. Case Report: A 6-year-old female presented with low back pain and swelling, difficulty ambulating, and rash two weeks after a respiratory infection. She was approached with a broad differential and ultimately diagnosed with IgA vasculitis. Discussion: Cutaneous manifestations, arthralgias, renal and gastrointestinal involvement are the most common presenting signs of IgA vasculitis. Only two cases of IgA vasculitis associated with lumbar pain and swelling were identified in the literature. Conclusion: While rash and joint pain are common presenting signs of IgA vasculitis, practitioners should be aware it can present atypically.

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Tang, Mengmeng, Xue Zhang, Xueqian Li, Lei Lei, Hejia Zhang, Chen Ling, Jie Ni, Jicheng Lv, Xiaorong Liu, and Xiangmei Chen. "Serum levels of galactose-deficient IgA1 in Chinese children with IgA nephropathy, IgA vasculitis with nephritis, and IgA vasculitis." Clinical and Experimental Nephrology 25, no. 1 (September 15, 2020): 37–43. http://dx.doi.org/10.1007/s10157-020-01968-8.

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9

Helander, S. D., F. R. De Castro, and L. E. Gibson. "Henoch-Schönlein purpura: clinicopathologic correlation of cutaneous vascular IgA deposits and the relationship to leukocytoclastic vasculitis." Acta Dermato-Venereologica 75, no. 2 (March 1, 1995): 125–29. http://dx.doi.org/10.2340/0001555575125129.

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Significant cutaneous vascular IgA deposits are common in Henoch-Schönlein purpura but not in other vasculitides. The specificity for IgA vascular deposits for Henoch-Schönlein purpura is not well defined. To examine the specificity of IgA vascular deposits for this disease, we compared clinicopathologic features of 92 cases with IgA vascular deposits and a direct immunofluorescence impression of vasculitis with 90 similar cases without IgA deposits. Henoch-Schönlein purpura was diagnosed in 24% of cases with vascular IgA deposits on direct immunofluorescence examination. IgA deposits were frequent in erythema nodosum and venous stasis-related problems and in cryoglobulinemia, coagulopathic vasculopathies, and livedoid vasculitis. Of our cases, 78% exhibited vascular fluorescence with multiple conjugates. No histologic or immunofluorescence pattern alone was specific. The diagnostic specificity for Henoch-Schönlein purpura is improved if gastrointestinal involvement, upper respiratory infection, or age < 20 years is present. We propose diagnostic criteria for Henoch-Schönlein purpura incorporating clinical findings yielding sensitivity and specificity > 90%.

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Takeuchi, Sora, Tamihiro Kawakami, Tatsuro Okano, Haruki Shida, Daigo Nakazawa, Utano Tomaru, Akihiro Ishizu, and Takafumi Kadono. "Elevated Myeloperoxidase-DNA Complex Levels in Sera of Patients with IgA Vasculitis." Pathobiology 89, no. 1 (November 23, 2021): 23–28. http://dx.doi.org/10.1159/000519869.

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Introduction: IgA vasculitis is a systemic disease that results from the entrapment of circulating IgA-containing immune complexes in small-vessel walls in the skin, kidneys, and gastrointestinal tract. An excessive formation of neutrophil extracellular traps (NETs) is involved in the pathogenesis of vasculitis, especially in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. This study aimed to clarify whether NETs are implicated in IgA vasculitis. Methods: Twenty-two patients with IgA vasculitis and 4 healthy volunteers were enrolled in this study. Serum levels of myeloperoxidase (MPO)-DNA complex, a fragment derived from NETs, were determined by enzyme-linked immunosorbent assay (ELISA), and the association between MPO-DNA complex levels and clinical parameters was examined. The presence of the ANCA was also assessed by ELISA specific for MPO and proteinase 3 (PR3) and indirect immunofluorescence (IIF), followed by assessing the differences in clinical parameters with and without the ANCA. Results: Serum MPO-DNA complex levels were significantly higher in patients with IgA vasculitis than those in healthy controls. A significant positive correlation between the serum MPO-DNA complex and IgA levels was noted. Interestingly, 63.6% of IgA vasculitis patients were ANCA-positive in IIF with an atypical pattern, whereas neither MPO-ANCA nor PR3-ANCA was detected by ELISA. These findings indicated that some IgA vasculitis patients possessed the so called minor ANCA. Serum IgA and MPO-DNA complex levels and the frequency of hematuria in the minor ANCA-positive group were significantly higher than in the minor ANCA-negative group. Conclusion: The collective findings suggested that NETs are certainly involved in the pathogenesis of IgA vasculitis.

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Nikolaishvili, Mariam, Ani Pazhava, and Vito Di Lernia. "Viral Infections May Be Associated with Henoch–Schönlein Purpura." Journal of Clinical Medicine 12, no. 2 (January 16, 2023): 697. http://dx.doi.org/10.3390/jcm12020697.

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Henoch–Schönlein purpura or IgA vasculitis is the most common type of pediatric vasculitis that may affect adults as well. It is classified as a type of small-vessel vasculitis. It can cause cutaneous and systemic symptoms with a minority of patients developing kidney failure. Little is known about the specific pathophysiology of this disorder, except that it is believed to occur in individuals with abnormally glycosylated IgA1. Serum aberrant IgA1 may form large antigen–antibody complexes which, due to a defective clearance, are able to deposit in the small vessels of the skin, kidney, gut, and joints. A variety of factors, including infectious agents, drugs, and vaccines, have been identified as potential triggers. The majority of cases are preceded by upper respiratory tract infections, and seasonal variations suggest a link with many pathogens. The etiologic agent most frequently associated with IgA vasculitis historically have been group A β-hemolytic streptococcus and common respiratory tract viruses. However, during the current coronavirus pandemic, SARS-CoV-2 infection was identified as a main trigger factor. In addition, IgA vasculitis has been observed following COVID-19 immunization. This review provides insights into the state of the art on the relationship between viral infections, viral vaccines, and Henoch–Schönlein purpura.

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Fedorczak, Anna, Dorota Szałowska-Woźniak, and Krzysztof Zeman. "Acute pancreatitis in IgA vasculitis." Pediatria i Medycyna Rodzinna 17, no. 2 (June 4, 2021): 164–67. http://dx.doi.org/10.15557/pimr.2021.0025.

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IgA vasculitis, also known as the Henoch–Schönlein purpura, is the most common systemic vasculitis in children. Inflammation most often involves small blood vessels of the skin, joints, kidneys and the gastrointestinal tract, but other organs may also be involved. Acute pancreatitis is a rare clinical manifestation of IgA vasculitis. We present a case of a 12-year-old girl with cutaneous and abdominal symptoms, who was diagnosed with acute pancreatitis during IgA vasculitis. In patients with IgA vasculitis who report abdominal pain acute pancreatitis should be taken into account and pancreatic enzymes measurement should be considered.

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Pillebout, Evangeline. "IgA Vasculitis and IgA Nephropathy: Same Disease?" Journal of Clinical Medicine 10, no. 11 (May 25, 2021): 2310. http://dx.doi.org/10.3390/jcm10112310.

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Many authors suggested that IgA Vasculitis (IgAV) and IgA Nephropathy (IgAN) would be two clinical manifestations of the same disease; in particular, that IgAV would be the systemic form of the IgAN. A limited number of studies have included sufficient children or adults with IgAN or IgAV (with or without nephropathy) and followed long enough to conclude on differences or similarities in terms of clinical, biological or histological presentation, physiopathology, genetics or prognosis. All therapeutic trials available on IgAN excluded patients with vasculitis. IgAV and IgAN could represent different extremities of a continuous spectrum of the same disease. Due to skin rash, patients with IgAV are diagnosed precociously. Conversely, because of the absence of any clinical signs, a renal biopsy is practiced for patients with an IgAN to confirm nephropathy at any time of the evolution of the disease, which could explain the frequent chronic lesions at diagnosis. Nevertheless, the question that remains unsolved is why do patients with IgAN not have skin lesions and some patients with IgAV not have nephropathy? Larger clinical studies are needed, including both diseases, with a common histological classification, and stratified on age and genetic background to assess renal prognosis and therapeutic strategies.

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Penido, Maria Goretti Moreira Guimarães, and Lilian Monteiro Pereira Palma. "IgA vasculitis in children." Brazilian Journal of Nephrology 44, no. 1 (March 2022): 3–5. http://dx.doi.org/10.1590/2175-8239-jbn-2022-e002.

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15

Fukushima, Kazuaki, and Naoki Yanagisawa. "Duodenal Ulcer - IgA Vasculitis." Internal Medicine 56, no. 4 (2017): 461–62. http://dx.doi.org/10.2169/internalmedicine.56.7735.

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KONG, Norella CT. "IgA nephropathy: A vasculitis?" Nephrology 3, no. 1 (February 1997): 23–26. http://dx.doi.org/10.1111/j.1440-1797.1997.tb00183.x.

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17

Moran, Sarah M., and Heather N. Reich. "IgA Vasculitis in Adults." Current Treatment Options in Rheumatology 4, no. 1 (February 13, 2018): 119–32. http://dx.doi.org/10.1007/s40674-018-0088-0.

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18

Wu, Jiali, Lagu He, Le Bai, Li Tan, and Min Hu. "Apolipoprotein M Serum Levels Correlate with IgA Vasculitis and IgA Vasculitis Nephritis." Disease Markers 2019 (December 11, 2019): 1–7. http://dx.doi.org/10.1155/2019/1825849.

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Objective. IgA vasculitis (lgAV) is the most frequent vessel vasculitis in children, and the prognosis is related to the children’s age and degree of nephritis. This study is aimed at investigating serum apolipoprotein M (apoM) levels in patients with lgAV patients and at evaluating the association between apoM and disease severity. Methods. A total of 109 lgAV patients and 76 age- and sex-matched healthy controls were included. The age and gender of the study participants were matched. ApoM levels were measured by an enzyme-linked immunosorbent assay. Additionally, the serum levels of lipids, apolipoproteins, kidney biochemical profiles, immunoglobulins (IgA, IgG, IgM, and IgE), and the complements (C3 and C4) were assessed using an automatic biochemical analyzer. Results. ApoM was increased significantly in lgAV patients compared to healthy controls. ApoM, meanwhile, was lower in patients with nephritis than in those without nephritis. The apoM levels were higher in classes I and II IgA vasculitis nephritis (lgAVN) patients than in classes III and IV. Besides, the apoM serum level<24.81 mg/L was an independent predictive factor for lgAVN and can be independently associated with the presence of nephritis in lgAV patients. Meanwhile, the serum apoM concentration negatively correlated with the ISKDC grading score in lgAVN patients. Conclusions. Serum apoM was elevated in lgAV patients and decreased gradually with the ISKDC grading score. ApoM (OR=0.32, 95%CI=0.12‐0.85, p=0.023) was identified as a protective factor for nephritis in all lgAV patients.

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Naifa, George, George Totikidis, Sonia Alexiadou, Christina Kolona, and Elpis Mantadakis. "Intravenous γ Globulin for Intractable Abdominal Pain due to IgA Vasculitis." Case Reports in Pediatrics 2020 (October 17, 2020): 1–4. http://dx.doi.org/10.1155/2020/8867621.

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IgA vasculitis (formerly known as Henoch–Schönlein purpura or anaphylactoid purpura) is a usually benign vasculitis that affects children of school age. The disease is characterized by the tetrad of palpable purpura, arthralgia/arthritis, abdominal pain, and hematuria. Treatment of IgA vasculitis is mainly supportive, with administration of simple analgesics. Corticosteroids have been shown to reduce and/or ameliorate the occurrence of abdominal pain which may be severe. We present two children with IgA vasculitis and severe abdominal pain despite corticosteroid administration, who responded promptly to intravenous γ globulin (IVIg) with complete resolution of their symptoms and review of the relevant medical literature. Given the toxicity and/or need for long-term administration of other second-line immunosuppressive therapies in corticosteroid-resistant IgA vasculitis, such as rituximab, cyclosporine, cyclophosphamide, azathioprine, or colchicine, we propose that IVIg may be a useful and safe treatment option, although randomized controlled clinical trials are needed in order to clarify its role in the treatment of abdominal pain in IgA vasculitis.

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Wei, Lin-Yan, Chao Liu, Ya-Li Zhang, and Guo-Liang Li. "IgA nephropathy with leucocytoclastic vasculitis." Journal of International Medical Research 46, no. 7 (June 10, 2018): 3009–14. http://dx.doi.org/10.1177/0300060518775814.

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Leucocytoclastic vasculitis is a rare type of allergic disease caused by immune complexes. IgA nephropathy is a glomerulopathy characterized by recurrent episodes of gross haematuria or microscopic haematuria and IgA deposition in the glomerular mesangial region. IgA nephropathy complicating leucocytoclastic vasculitis is rare documented. We present a case of IgA nephropathy in a 47-year-old woman with leucocytoclastic vasculitis and discuss the clinical and pathological data, aiming to promote the diagnosis and treatment of this specific clinical manifestation.

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Kvacheniuk, Olena, and Olena Okhotnikova. "Modern features of the evolution of IgA-vasculitis in children according to catamestic study." 8, no. 8 (December 29, 2021): 41–50. http://dx.doi.org/10.26565/2617-409x-2021-8-04.

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Abstract. There is a tendency to an increase in the frequency of hospitalization of children with mixed forms of IgA-vasculitis with pronounced abdominal syndrome and involvement in the pathological process of the kidneys, which is possibly associated with an increase in the level of comorbid pathology of the gastrointestinal tract, and with a premorbid state of the mucosa membranes of the digestive system. There is a need to study the course and evolution of IgA vasculitis in children with gastrointestinal manifestations in order to improve the diagnostic search, accurate prognosis and create a standardized treatment protocol for IgA vasculitis in children. Objective. To investigate the clinical and diagnostic manifestations and evolution of IgA vasculitis in children with lesions of the gastrointestinal tract. Materials and method. The surveys of patients and their parents and retrospective analysis of 58 maps of inpatients with IgA vasculitis and digestive lesions aged 0 to 18 years, who were hospitalized in the somatic departments of the National Children's Specialized Hospital " OKHMATDYT" Kyiv, Ukraine in the period from 2008 to 2018. Results. The mean age of children with IgA vasculitis was 8,03 years, and the ratio of boys to girls was 1,32: 1. Skin syndrome occurred in 58/58 patients (100%), and joint symptoms were observed in 34/58 (58,62%) patients. There were 29/58 patients (50,0%) with kidney disease. The incidence of renal pathology was higher in children with severe symptoms of the digestive system and in persons older than 7 years. 23/29 (79,31%) patients had manifestations of kidney damage in the first month after the onset of IgA vasculitis with gastrointestinal symptoms. Conclusions. In the study among hospitalized children, clinical and laboratory features and the evolution of IgA vasculitis in children with gastrointestinal lesions were similar to the literature. In 79,31% of patients, the onset of renal syndrome occurred in the first month after the onset of IgA vasculitis. In 20,69% of patients there was an evolution of kidney damage into chronic kidney disease. Therefore, careful monitoring of renal impairment should be performed to assess the progression of chronic kidney disease in patients who develop IgA vasculitis over 7 years of age and in those children who have severe symptoms of gastrointestinal damage.

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Lee, Keum Hwa, Sung Hwi Hong, Jinhae Jun, Youngheun Jo, Woogyeong Jo, Dayeon Choi, Jeongho Joo, et al. "Treatment of refractory IgA vasculitis with dapsone: a systematic review." Clinical and Experimental Pediatrics 63, no. 5 (May 15, 2020): 158–63. http://dx.doi.org/10.3345/kjp.2019.00514.

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IgA vasculitis, formerly known as Henoch-Schönlein purpura, is a systemic IgA-mediated vasculitis of the small vessels commonly seen in children. The natural history of IgA vasculitis is generally self-limiting; however, one-third of patients experience symptom recurrence and a refractory course. This systematic review examined the use of dapsone in refractory IgA vasculitis cases. A literature search of PubMed databases retrieved 13 articles published until June 14, 2018. The most common clinical feature was a palpable rash (100% of patients), followed by joint pain (69.2%). Treatment response within 1–2 days was observed in 6 of 26 patients (23.1%) versus within 3–7 days in 17 patients (65.4%). Relapse after treatment discontinuation was reported in 17 patients (65.4%) but not in 3 patients (11.5 %). Four of the 26 patients (15.4%) reported adverse effects of dapsone including arthralgia (7.7%), rash (7.7%), and dapsone hypersensitivity syndrome (3.8%). Our findings suggest that dapsone may affect refractory IgA vasculitis. Multicenter randomized placebo-controlled trials are necessary to determine the standard dosage of dapsone at initial or tapering of treatment in IgA vasculitis patients and evaluate whether dapsone has a significant benefit versus steroids or other medications.

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Mayer-Hain, Sarah, Kathleen Gebhardt, Matthias Neufeld, Jan M. Ehrchen, Karen Molyneux, Jonathan Barratt, Eva Nattkemper, et al. "Systemic Activation of Neutrophils by Immune Complexes Is Critical to IgA Vasculitis." Journal of Immunology 209, no. 6 (September 15, 2022): 1048–58. http://dx.doi.org/10.4049/jimmunol.2100924.

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Abstract In IgA vasculitis (IgAV) perivascular deposition of IgA1 immune complexes (IgA-ICs) is traditionally considered the fundamental trigger for polymorphonuclear neutrophil (PMN)–mediated damage. We propose that IgA-IC deposition, although mandatory, is not sufficient alone for IgAV. Serum IgA-IC levels and IgA-IC binding to PMNs were quantified in IgAV patients and controls. Activation of PMNs was evaluated by neutrophil extracellular trap (NET) release, adherence, and cytotoxicity assays and in a flow system to mirror conditions at postcapillary venules. In vitro results were related to findings in biopsies and a mouse vasculitis model. During acute IgAV flares we observed elevated serum levels of IgA-ICs and increased IgA-IC binding to circulating PMNs. This IgA-IC binding primed PMNs with consequent lowering of the threshold for NETosis, demonstrated by significantly higher release of NETs from PMNs activated in vitro and PMNs from IgAV patients with flares compared with surface IgA-negative PMNs after flares. Blocking of FcαRI abolished these effects, and complement was not essential. In the flow system, marked NETosis only occurred after PMNs had adhered to activated endothelial cells. IgA-IC binding enhanced this PMN tethering and consequent NET-mediated endothelial cell injury. Reflecting these in vitro findings, we visualized NETs in close proximity to endothelial cells and IgA-coated PMNs in tissue sections of IgAV patients. Inhibition of NET formation and knockout of myeloperoxidase in a murine model of IC vasculitis significantly reduced vessel damage in vivo. Binding of IgA-ICs during active IgAV primes PMNs and promotes vessel injury through increased adhesion of PMNs to the endothelium and enhanced NETosis.

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Mizerska-Wasiak, Małgorzata, Maria Winiarska, Karolina Nogal, Karolina Cichoń-Kawa, Małgorzata Pańczyk-Tomaszewska, and Jadwiga Małdyk. "IgA Vasculitis Complicated by Both CMV Reactivation and Tuberculosis." Pediatric Reports 13, no. 3 (July 22, 2021): 416–20. http://dx.doi.org/10.3390/pediatric13030048.

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Immunoglobulin A (IgA) vasculitis is the most common systemic vasculitis in the pediatric population. We present the case of a patient with IgA vasculitis with nephritis who developed cytomegalovirus (CMV) infection followed by Mycobacterium tuberculosis infection. In the literature, there are a few cases of IgA nephropathy accompanied by reactivation of CMV or tuberculosis. To the best of our knowledge, this is the first reported case of IgA vasculitis complicated by both CMV reactivation and tuberculosis. It is important to detect infections in patients with IgA vasculitis because they can induce and exacerbate the symptoms of the disease. Effective antimicrobial treatment facilitates the management of proteinuria and slows down the decline of renal function. Immunosuppressive therapy is a risk factor for reactivation of latent infections and makes patients more susceptible to its generalized and complicated course. This can be prevented by actively screening for hidden sites of infection.

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Maye, James A., Hsu Pheen Chong, Vivek Rajagopal, and William Petchey. "Reactivation of IgA vasculitis following COVID-19 vaccination." BMJ Case Reports 14, no. 11 (November 2021): e247188. http://dx.doi.org/10.1136/bcr-2021-247188.

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A 23-year-old man presented to the acute assessment unit with acute-onset haematuria within 24 hours of receiving his second dose of the Pfizer-BioNTech COVID-19 vaccine. He had been diagnosed with IgA vasculitis 8 months previously. IgA vasculitis is an autoimmune condition characterised by palpable purpura affecting the lower limbs, abdominal pain, arthralgia and renal disease. He was diagnosed with an acute exacerbation of IgA vasculitis and was discharged with oral prednisolone. Reactivation or first presentation of IgA vasculitis is a rare but increasingly recognised complication of COVID-19 vaccination. This is an important new differential in the assessment of patients with haematuria following COVID-19 vaccination.

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El-Reshaid, Kamel, and Shaima Al-Bader. "The limited role and risky profile of Rituximab in nephritis associated with Henoch-Schönlein purpura." Journal of Drug Delivery and Therapeutics 9, no. 6-s (December 15, 2019): 12–15. http://dx.doi.org/10.22270/jddt.v9i6-s.3744.

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Adult-onset IgA vasculitis or Henoch-Schönlein purpura (HSP) is a systemic vasculitis characterized by IgA1-dominant deposits. The symptoms include cutaneous purpura, ankle arthritis, enteritis and nephritis. HSP nephritis (HSPN) can be severe and refractory to corticosteroids with/without immunosuppressive agents. The concept of depletion of antibody producing B cell with Rituximab is appealing despite the uncertainity of HSP pathogenesis. In the present case report; we describe our experience with Rituximab treatment in a patient with this disease. Our patient had different triggering factors for her relapses and lately Rituximab itself. Review of the literature indicates that autoantibodies to Gd-IgA1 did not decrease with Rituximab therapy and others indicated an inherited predisposition for higher levels in patients with progressive disease. Our findings confirm the limited role and risky profile of Rituximab in treatment of HSP. Keywords: HSP, IgA, Rituximab, vasculitis.

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Pant, Anil Dev, and Nitesh Raj Pokharel. "Utility of Immunofluorescence in Cutaneous Vasculitis." Saudi Journal of Pathology and Microbiology 8, no. 08 (August 8, 2023): 193–96. http://dx.doi.org/10.36348/sjpm.2023.v08i08.001.

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Vasculitis may be due to various causes, including connective tissue disorders, medications, and infections. Histopathology shows similar features in different diseases so DIF (direct immunofluorescence) helps to categorize these vasculitic lesions on basis of positivity of different immunoglobulins. In this study, 40 cases of suspected vasculitis were confirmed by histopathology. Females were more commonly affected and the age range was from 9 to 71 years. Among these cases, 21 were immune complex mediated vasculitis of which c3 and IgG were the most commonly found immunoglobulins. Two of these cases were IgA mediated vasculitis. DIF was of great importance for the diagnosis of the disease for appropriate treatment.

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Choi, Yunjung, Chang Hun Lee, Kyoung Min Kim, and Wan-Hee Yoo. "Sudden Onset of IgA Vasculitis Affecting Vital Organs in Adult Patients following SARS-CoV-2 Vaccines." Vaccines 10, no. 6 (June 9, 2022): 923. http://dx.doi.org/10.3390/vaccines10060923.

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IgA vasculitis is an immune complex-mediated small-vessel vasculitis that mainly occurs in children and is characterized by palpable purpura, arthralgia, abdominal pain, and glomerulonephritis. We report three cases of new-onset IgA vasculitis involving major organs in adult patients after they received either the ChAdOx1 viral vector (Oxford/AstraZeneca) vaccine or the messenger RNA-1273 (Moderna) vaccine. These cases suggest that COVID-19 vaccines have the potential to trigger IgA vasculitis and indicate that physicians need to monitor for this possible complication.

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Matsumura, Mariko, Yoriaki Komeda, Tomohiro Watanabe, and Masatoshi Kudo. "Purpura-free small intestinal IgA vasculitis complicated by cytomegalovirus reactivation." BMJ Case Reports 13, no. 7 (July 2020): e235042. http://dx.doi.org/10.1136/bcr-2020-235042.

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IgA vasculitis (Henoch-Schönlein purpura) affects various organs, including the skin, gastrointestinal (GI) tract, joints and kidneys. Its clinical course typically consists of two phases: initial appearance of purpura and delayed onset of arthralgia, GI symptoms and haematuria. We report the case of an adult patient with IgA vasculitis of the small bowel, without skin involvement, complicated by cytomegalovirus (CMV) enteritis following prednisolone administration. Single-balloon enteroscopy revealed mucosal oedema, redness, erosions and transverse ulcers of the duodenum and jejunum. Jejunal biopsy specimens showed IgA deposition in the capillary walls. CMV reactivation was confirmed by PCR and immunostaining using jejunal biopsy specimens. This case report strongly suggests that adult patients with IgA vasculitis can present with isolated GI involvement, without characteristic skin purpura. Furthermore, CMV reactivation needs to be considered in patients with IgA vasculitis showing poor response to glucocorticoids.

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Kaur, Gurwant, and Catherine Abendroth. "Paraneoplastic IgA nephropathy and IgA vasculitis in mesothelioma." International Journal of Case Reports and Images 10 (2019): 1. http://dx.doi.org/10.5348/101063z01kb2019cr.

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Gmachowska, Katarzyna. "Meningismus in IgA vasculitis (Henoch–Schönlein purpura)." Pediatria i Medycyna Rodzinna 16, no. 4 (December 31, 2020): 414–17. http://dx.doi.org/10.15557/pimr.2020.0074.

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IgA vasculitis is the most common acute systemic vasculitis in children. Typical symptoms include cutaneous purpura not associated with thrombocytopenia, acute-onset abdominal pain, arthritis, renal symptoms and, less commonly, neurological symptoms. The disease is usually mild and self-limiting. Although the aetiology of IgA vasculitis is unknown, autoimmune involvement is suspected. Symptomatic treatment is mainly used; therapeutic method that would reduce disease duration and prevent recurrence is unknown. Meningococcal sepsis should always be ruled out in a child with cutaneous purpura. We present a case of a 4-year-old boy with IgA vasculitis who developed symptoms of meningeal irritation.

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Marques Dias, Joana, Marta Azevedo Ferreira, Ana Grilo, and Fernando Martos Gonçalves. "IgA vasculitis with severe renal manifestation." BMJ Case Reports 15, no. 6 (June 2022): e248686. http://dx.doi.org/10.1136/bcr-2021-248686.

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IgA vasculitis is a rare systemic vasculitis in adults, frequently more severe than in paediatric age. It manifests with cutaneous, articular, gastrointestinal and renal involvement.We present a case of a man in his 40s diagnosed with IgA vasculitis with cutaneous, joint, gastrointestinal and renal disease. Significant proteinuria and renal biopsy findings demonstrating crescentic glomerulonephritis led to the onset of early immunosuppression with corticoid and cyclophosphamide. This case report reflects a case of more severe renal impairment due to IgA vasculitis with good outcome with the chosen therapy. The findings in the renal biopsy after treatment supported the good response to the chosen immunosuppression.

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Sukalo, A. V., and I. A. Kazyra. "Characteristics of the course of nephritis associated with Iga-vasculitis Henoch-Schoenlein in children." Nephrology (Saint-Petersburg) 24, no. 3 (April 23, 2020): 64–71. http://dx.doi.org/10.36485/1561-6274-2020-24-3-64-71.

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INTRODUCTION. Among systemic vasopathies in children, IgA vasculitis Henoch Schoenlein (HS) is the most common, according to various authors, kidney damage is noted in 25-80 % and usually determines the prognosis of the disease.THE AIM of the study was to analyze clinical, laboratory, immunological, morphological characteristics, features of the course and treatment of nephritis associated with IgA vasculitis HS in children, as well as factors affecting the prognosis.PATIENTS AND METHODS. The study included 31 patients with morphologically verified nephritis due to IgA vasculitis HS (18 – boys, 13 – girls) aged 3 to 17 years, who were monitored at the Nephrology Department of the "2nd Children's City Clinical Hospital" of the National Center for Pediatric Nephrology and Renal Replacement therapy in Minsk from 2010 to 2019 yrs.The following parameters were analyzed: the clinical variant of kidney damage, laboratory tests (including the study of BAFF, RANTES lymphocyte activation molecules, pro-inflammatory IL1β, caspase1, TNFα, growth factors VEGF, TGF), 24 hours monitoring and office blood pressure measurements, ECHO cardiography with indicescalculation, ultrasound of the carotid arteries with the thickness of intima-media complex, morphological changes in the renal tissue, as well as treatment regimens.RESULTS. The contribution of deGal-IgA1, markers of T and B lymphocytes activation, pro-inflammatory and profibrotic molecules in the development of the disease is shown. Arterial hypertension was registered in 42 % of children, signs of heart remodeling according to the calculated indices in 19,3 %. Decrease level of adiponectin, vitamin D, leptin, increase concentration of obestatin, Pro-BNP, hs-CRP, and TSAT indicator classify patients with nephritis due to IgA vasculitis HS at moderate risk for the developmentof cardio-vascular disorders, which suggests the need for timely correction.CONCLUSION. In most cases, nephritis with IgA vasculitis HS has a benign course with rare relapses and progression to the end stage of chronic kidney disease (6,5 %).

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Rivas, Magali Noval, Daiko Wakita, Michael Franklin, Shuang Chen, Michifumi Yamashita, Timothy R. Crother, Kenichi Shimada, and Moshe Arditi. "Intestinal permeability and IgA provoke immune vasculitis linked to cardiovascular inflammation." Journal of Immunology 202, no. 1_Supplement (May 1, 2019): 67.1. http://dx.doi.org/10.4049/jimmunol.202.supp.67.1.

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Abstract Kawasaki Disease (KD), a systemic vasculitis of unknown etiology is the leading cause of acquired heart disease among children in the US. Subsets of KD children display gastrointestinal abnormalities during the acute phase of the disease and may have increased serum levels of secretory IgA (SIgA), suggestive of a “leaky gut” phenotype. Additionally, KD patients have higher frequencies of IgA producing B cells in the inflamed coronary artery and in the respiratory and gastrointestinal tracts. How intestinal permeability participates in cardiovascular lesions of KD remains unknown. We observed increased intestinal permeability and elevated circulating sIgA in KD patients as well as elevated sIgA and IgA deposition in vascular tissues in the Lactobacillus casei-cell wall extract murine model of KD vasculitis. Pharmacological blocking of intestinal permeability prevented gut permeability, vascular tissues IgA deposition, and ameliorated cardiovascular pathology. By using genetic and pharmacologic inhibition of IL-1β signaling, we demonstrated that IL-1β lies upstream of disrupted intestinal barrier function, subsequent IgA vasculitis development, and cardiac inflammation. Thus, targeting mucosal barrier dysfunction and the IL-1β pathway may be applicable to other IgA-related diseases including IgA vasculitis and IgA nephropathy. These observations enhance our current understanding of KD pathogenesis and may provide new diagnostic and therapeutic strategies for affected patients.

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Amini, Sheida, and Mohsen Jari. "Granulomatosis with Polyangiitis Misdiagnosed as IgA Vasculitis in a Child." Case Reports in Pediatrics 2023 (April 17, 2023): 1–3. http://dx.doi.org/10.1155/2023/9950855.

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Background. Granulomatosis with polyangiitis (GPA) with early manifestations simulating IgA vasculitis is a very rare childhood systemic disease. Case Presentation. A 10-year-old boy presented initially with cutaneous, skeletal, and abdominal signs suggestive of IgA vasculitis. Over time, the worsening of skin ulcers, orchitis, and renal involvement led to the diagnosis of GPA according to cytoplasmic positive antineutrophil cytoplasmic antibodies and renal biopsy. Conclusion. Clinicians should be awared of the diagnostic pitfalls when making a clinical diagnosis of IgA vasculitis in children older than 7 years.

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T, Anila Reddy, Vineeth Reddy G, and Sharvana Bhava B.S. "A CASE REPORT: IgA VASCULITIS." International Journal of Advances in Pharmacy and Biotechnology 3, no. 4 (December 15, 2017): 1–4. http://dx.doi.org/10.38111/ijapb.20170304001.

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Yeganeh, Mehrnoush Hassas, Reza Shiari, and Khosro Rahmani. "IgA vasculitis in Henoch-Schönleinpurpura." Journal of Pediatric Nephrology 4, no. 1 (February 10, 2016): 8–13. http://dx.doi.org/10.20286/jpn-04018.

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Esteban Terradillos, Sara, José Gabriel Erdozain Castiella, Juan Manuel Goiri Aparicio, Juan Antonio Ratón Nieto, Aitor Fernández Larrinoa Santamaria, and Juan Monte Armenteros. "IgA vasculitis induced by acenocoumarol." Reumatología Clínica (English Edition) 16, no. 5 (September 2020): 362–63. http://dx.doi.org/10.1016/j.reumae.2018.05.003.

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Esteban Terradillos, Sara, José Gabriel Erdozain Castiella, Juan Manuel Goiri Aparicio, Juan Antonio Ratón Nieto, Aitor Fernández Larrinoa Santamaria, and Juan Monte Armenteros. "Vasculitis IgA inducida por acenocumarol." Reumatología Clínica 16, no. 5 (September 2020): 362–63. http://dx.doi.org/10.1016/j.reuma.2018.05.006.

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Tsukui, Daisuke, and Hajime Kono. "Ultraviolet Purpura in IgA Vasculitis." Mayo Clinic Proceedings 93, no. 1 (January 2018): 122. http://dx.doi.org/10.1016/j.mayocp.2017.09.013.

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Allez, Matthieu, Blandine Denis, Jean‐David Bouaziz, Maxime Battistella, Anne‐Marie Zagdanski, Jules Bayart, Ingrid Lazaridou, et al. "COVID‐19–Related IgA Vasculitis." Arthritis & Rheumatology 72, no. 11 (September 22, 2020): 1952–53. http://dx.doi.org/10.1002/art.41428.

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Diogo, Joana, Carolina Coelho, Rita Monteiro, and �ngela Ghiletchi. "IgA Vasculitis involving four systems." Galicia Clínica 83, no. 4 (2022): 48. http://dx.doi.org/10.22546/67/2633.

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Glassock, Richard J. "Prognostic Scoring in IgA Vasculitis." Clinical Journal of the American Society of Nephrology 19, no. 4 (April 2024): 409–11. http://dx.doi.org/10.2215/cjn.0000000000000444.

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Du, Lina, Panpan Wang, Chang Liu, Shaojing Li, Shuang Yue, and Yan Yang. "Multisystemic manifestations of IgA vasculitis." Clinical Rheumatology 40, no. 1 (June 16, 2020): 43–52. http://dx.doi.org/10.1007/s10067-020-05166-5.

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Javadi Parvaneh, Vadood, Arezoo Shirzani, Khosro Rahmani, and Reza Shiari. "Pediatric Granulomatosis With Polyangiitis Mimicking IgA Vasculitis: A Case Report." Clinical Medicine Insights: Arthritis and Musculoskeletal Disorders 13 (January 2020): 117954412096737. http://dx.doi.org/10.1177/1179544120967371.

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Background: Granulomatosis with polyangiitis (GPA) is a systemic vasculitis of the upper and lower respiratory tract along with glomerulonephritis and is very rare in childhood. Its renal manifestations similarity with IgA vasculitis can be misleading. Case presentation: Herein, we report a 12-years-old girl with the clinical picture of IgA vasculitis and renal involvement at the time of presentation, over time, elevated cytoplasmic Anti-neutrophil Cytoplasmic Antibody (C-ANCA) and tissue biopsy confirmed GPA. Conclusion: In the case of a patient with an unusual presentation of IgA vasculitis, to some degree of suspicion, the GPA should be considered. Also, in approach to non-thrombocytopenic palpable petechia and purpura a wide range of differential diagnosis such as infections, ANCA associated vasculitis, and secondary vasculitis should be considered. Therefore, 2 effective method of GPA diagnosis, the high titer of C-ANCA test and tissue biopsy, should be considered simultaneously.

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Segelmark, Mårten. "Serological testing in small vessel vasculitis." Rheumatology 59, Supplement_3 (April 29, 2020): iii51—iii54. http://dx.doi.org/10.1093/rheumatology/kez633.

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Abstract Serological analysis has a central role in the diagnostic work-up of patients with suspected small vessel vasculitis, both for establishing a specific diagnosis and for the monitoring of response to therapy. Autoantibodies can be detected in all forms of primary small vessel vasculitis as well as in the most common forms of secondary vasculitis. For primary vasculitis the most important serological test is for ANCA. ANCA can be found in 75–95% of patients with pauci-immune small vessel vasculitis leading to this subgroup of vasculitides being named ANCA associated vasculitis. ANCA levels often follow this disease course, but the value of serial ANCA testing is controversial. Other important autoantibodies in primary small vessel vasculitis are anti-glomerular basement membrane antibodies, anti-C1q, anti-galactose deficient IgA and cryoglobulins. A wide variety of systemic inflammatory diseases and infections can be complicated by small vessel vasculitis and detected by serological testing. Important examples are SLE, rheumatoid arthritis, Hepatitis C and HIV.

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Fraticelli, Paolo, Devis Benfaremo, and Armando Gabrielli. "Diagnosis and management of leukocytoclastic vasculitis." Internal and Emergency Medicine 16, no. 4 (March 13, 2021): 831–41. http://dx.doi.org/10.1007/s11739-021-02688-x.

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AbstractLeukocytoclastic vasculitis (LCV) is a histopathologic description of a common form of small vessel vasculitis (SVV), that can be found in various types of vasculitis affecting the skin and internal organs. The leading clinical presentation of LCV is palpable purpura and the diagnosis relies on histopathological examination, in which the inflammatory infiltrate is composed of neutrophils with fibrinoid necrosis and disintegration of nuclei into fragments (“leukocytoclasia”). Several medications can cause LCV, as well as infections, or malignancy. Among systemic diseases, the most frequently associated with LCV are ANCA-associated vasculitides, connective tissue diseases, cryoglobulinemic vasculitis, IgA vasculitis (formerly known as Henoch–Schonlein purpura) and hypocomplementemic urticarial vasculitis (HUV). When LCV is suspected, an extensive workout is usually necessary to determine whether the process is skin-limited, or expression of a systemic vasculitis or disease. A comprehensive history and detailed physical examination must be performed; platelet count, renal function and urinalysis, serological tests for hepatitis B and C viruses, autoantibodies (anti-nuclear antibodies and anti-neutrophil cytoplasmic antibodies), complement fractions and IgA staining in biopsy specimens are part of the usual workout of LCV. The treatment is mainly focused on symptom management, based on rest (avoiding standing or walking), low dose corticosteroids, colchicine or different unproven therapies, if skin-limited. When a medication is the cause, the prognosis is favorable and the discontinuation of the culprit drug is usually resolutive. Conversely, when a systemic vasculitis is the cause of LCV, higher doses of corticosteroids or immunosuppressive agents are required, according to the severity of organ involvement and the underlying associated disease.

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Levanon, Shirel, Vera Gotloib, Yehudit Kraus, Irina Novofastovski, Shay Brikman, Abdallah Fawaz, Mohammad Egbaria, et al. "IgA vasculitis in adults, pediatrics and non-vasculitic IgA nephropathy, retrospective analysis from 2 centers." Medicine 102, no. 50 (December 15, 2023): e36521. http://dx.doi.org/10.1097/md.0000000000036521.

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Renal involvement represents the major long-term morbidity associated with IgA vasculitis (IgAV). Our aim was to evaluate clinical characteristics and long-term renal outcomes of IgAV in pediatrics and adults comparing to IgA nephropathy (IgAN). Our retrospective study included children and adults with IgAV and IgAN patients, admitted in a 13-year period (2007–2019) to rheumatology clinics and in hospital pediatric and internal medicine departments. We compared frequencies of clinical manifestations, laboratory findings, treatments, long-term outcomes at 1 year follow-up, including all-cause mortality and dialysis until the end of follow-up time. A total of 60 adult IgAV, 60 pediatric IgAV and 45 IgAN patients were evaluated. Adult IgAV patients were significantly older than IgAN patients (53.1 ± 17.4 years vs 45.1 ± 15.7 years respectively, P = .02) and had significantly higher rates of cardiovascular comorbidities. The risk and time to dialysis were similar among IgAN and adult IgAV groups. Yet, overall mortality at long term follow up was higher in IgAV adult group compared to IgAN. No dialysis or renal transplantation were reported in pediatric IgAV patients. IgAV and IgAN adult patients were comparable regarding risk of end stage renal disease. Of note, high mortality rates were observed among adult IgAV group.

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Sanjari, Mojgan, Mohammadreza Shakibi, and Moeinadin Safavi. "Henoch–Schönlein Purpura (IgA Vasculitis) in Association with Thyrotoxicosis." Case Reports in Endocrinology 2021 (May 22, 2021): 1–3. http://dx.doi.org/10.1155/2021/6669653.

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Graves’ disease is the most common cause of hyperthyroidism, which is characterized by thyroid antibodies and the following clinical manifestations: goiter, ophthalmopathy, and pretibial myxedema. On the other hand, Henoch–Schönlein purpura is an IgA-mediated small-vessel vasculitis. Review of the literature showed a relationship between propylthiouracil overdose and the following Henoch–Schönlein purpura (IgA vasculitis) as a side effect. The patient was a 31-year-old woman with a chief complaint of tremor and significant weight loss who contracted pruritic palpable purpura during her disease course. Then, she underwent the treatment of hyperthyroidism by methimazole which intensified her cutaneous lesions. The diagnosis of Henoch–Schönlein purpura (IgA vasculitis) was confirmed after skin biopsy. Finally, she was treated with colchicine, prednisolone, and radioiodine ablation, which caused her lesions to disappear. The temporal priority of pruritic palpable skin lesions to hyperthyroidism treatment with methimazole suggested that Henoch–Schönlein purpura (IgA vasculitis) was related to hyperthyroidism and was intensified by antithyroid agents in this patient.

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Yagi, Haruna, Seishiro Takahashi, Tetsuo Kibe, Kenji Shirai, Isao Kosugi, Hideya Kawasaki, Shiori Meguro, Toshihide Iwashita, and Hiroshi Ogawa. "An Autopsy Case of a 5-Year-Old Child with Acute Pancreatitis Caused by Eosinophilic Granulomatosis with Polyangiitis-like Necrotizing Vasculitis." Case Reports in Rheumatology 2019 (September 2, 2019): 1–7. http://dx.doi.org/10.1155/2019/9053747.

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In children, acute pancreatitis has been reported in IgA vasculitis, Kawasaki disease, systemic lupus erythematosus-associated vasculitis, and juvenile dermatomyositis-associated vasculitis. However, its frequency in these vasculitides has been shown to be low. In other childhood-onset vasculitides, acute pancreatitis is seldom reported. The patient was a 5-year-old Japanese boy who suddenly presented with gastrointestinal (GI) bleeding. Therapy with antiulcer drugs successfully stopped bleeding, but subsequently, high fever, leukocytosis, and hypoxia appeared. He died 12 days after he presented with GI bleeding. An autopsy unexpectedly revealed that necrotizing vasculitis with marked eosinophilic and histiocytic infiltration of the pancreas led to acute pancreatitis, and gastric ulcer with eosinophilic infiltration was shown to be the origin of GI bleeding. In addition, eosinophilic infiltration was found in the small intestine, lungs, and bone marrow. Necrotizing vasculitis with eosinophilic and histiocytic infiltration of the pancreas, eosinophilic infiltration of the airway wall, and eosinophilic gastroenteritis with gastric ulcer were histologically confirmed, suggesting that the present case may be an early stage of eosinophilic granulomatosis with polyangiitis- (EGPA-) like vasculitis. To our knowledge, this might be the first reported case of EGPA-like vasculitis presenting with acute pancreatitis in a child.

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