Academic literature on the topic 'Brito family'

BackgroundPrimary Sjogren syndrome (pSS) is a chronic autoimmune multisystem disease characterized by inflammation of exocrine glands, particularly the lacrimal and salivary glands, as well as multiple extraglandular involvements. Treatment is often challenging, and till now, no Disease-Modifying Antirheumatic Drugs (DMARD) have been approved for this condition. There is a lack of data about the efficacy of Janus kinase inhibitors in pSS. Here we report a patient with pSS successfully responding to upadacitinib.ObjectivesTo see the efficacy of upadacitinib in the management of primary Sjogren syndrome.MethodsA 68-year-old woman presented with pain in multiple joints for 16 years with intermittent use of NSAIDs. She had no low back pain and no self or family history of psoriasis or red eye. She denied dry mouth but required frequent water drinking during meals to aid swallowing and occasionally used artificial tears. MTX was started for her two-month articular flare and positive rheumatoid factor (RF). MTX caused severe vomiting and hyponatremia, and she was hospitalized. After the correction of hyponatremia, she was put on tofacitinib. We reevaluated the case when she came to us. She had grade 2-3 tenderness of most peripheral joints and was bedridden. Immunological parameters revealed RF 127 IU/ml (reference value <14), anti-SSA antibody 147 U/ml (normal <3.2), anti-SSB antibody 149 U/ml (reference value <8) and anti-CCP antibody; ANA, anti-ds-DNA Ab were negative. There was no erosion on the X-ray hands. Schirmer’s test was positive (3 mm bilaterally). Unstimulated salivary flow after five minutes was 0.04 ml/min (≤0.1 ml/min indicates salivary hypofunction). She fulfilled the 2016 ACR/EULAR classification criteria, and we diagnosed her with primary Sjogren syndrome. Considering her age, she was switched to upadacitinib, as European Medicines Agency restricted the use of tofacitinib beyond 65 years.ResultsHer EULAR Sjogren Syndrome’s Disease Activity Index (ESSDAI) [1] was six at baseline, indicating moderate disease activity. Her joint pain improved significantly within two months with infrequent use of pilocarpine in the sixth month. Six months after treatment with upadacitinib, ESSDAI came down to 2; the Patient’s Global Assessment score was 02 and achieved STAR (Sjogren’s Tool for Assessing Response) response 7 (STAR responder if score ≥5) [2].ConclusionTo our knowledge, this is the first case report on the efficacy of upadacitinib in pSS. Further high-quality clinical trials are required to confirm the benefit of upadacitinib in pSS.References[1]. Ramos-Casals M, Brito-Zerón P, Bombardieri S, Bootsma H, De Vita S, Dörner T, Fisher BA, Gottenberg JE, Hernandez-Molina G, Kocher A, Kostov B. EULAR recommendations for the management of Sjögren’s syndrome with topical and systemic therapies. Annals of the rheumatic diseases. 2020 Jan 1;79(1):3-18.[2]. Seror R, Baron G, Camus M, Cornec D, Perrodeau E, Bowman SJ, Bombardieri M, Bootsma H, Gottenberg JE, Fisher B, Hueber W. Development and preliminary validation of the Sjögren’s Tool for Assessing Response (STAR): a consensual composite score for assessing treatment effect in primary Sjögren’s syndrome. Annals of the rheumatic diseases. 2022 Jul 1;81(7):979-89.[1]. Ramos-Casals M, Brito-Zerón P, Bombardieri S, Bootsma H, De Vita S, Dörner T, Fisher BA, Gottenberg JE, Hernandez-Molina G, Kocher A, Kostov B. EULAR recommendations for the management of Sjögren’s syndrome with topical and systemic therapies. Annals of the rheumatic diseases. 2020 Jan 1;79(1):3-18.[2]. Seror R, Baron G, Camus M, Cornec D, Perrodeau E, Bowman SJ, Bombardieri M, Bootsma H, Gottenberg JE, Fisher B, Hueber W. Development and preliminary validation of the Sjögren’s Tool for Assessing Response (STAR): a consensual composite score for assessing treatment effect in primary Sjögren’s syndrome. Annals of the rheumatic diseases. 2022 Jul 1;81(7):979-89.Acknowledgements:NIL.Disclosure of InterestsNone Declared.

Background and context: It is known the chances of healing in developed countries might be of 90%, depending on the type of tumor and, especially, its early diagnosis. In Brazil, cancer is the leading cause of death by disease among ages from 1 to 19, and because it is rare, health professionals have difficulties in diagnosing and referring cases to treatment. Aim: The “Unidos pela Cura” aims to promote early diagnosis of childhood cancer in Rio de Janeiro in a coresponsible and integrated way to the principles of Brazilian Unified Health System (universalization, equity and integrality). Strategy/Tactics: This initiative included the Desiderata Institute as the leader of civil society organization, having as its bases: i) Mobilization of managers from the government, heads and professionals of the pediatric oncology services, health practitioners (primary care) and social organization to identify the main problem with pediatric oncology in the public system and to collectively think solutions. The identified problem was related to the early diagnosis; ii) Knowledge about the identified problem, with studies: one pointed out the main epidemiologic trends of childhood cancer in the state of Rio de Janeiro ( Koiffman, 2004 ); and another showed the system problems by revealing that suspicions of childhood cancer took an average of 60 days to be investigated when they arrived from primary care ( Brito, 2005 ); iii) Advocacy: the collective construction of coresponsibilities and priorities regarding childhood cancer in a term of commitment, and marked the definition of the initiative as public policy; and iv) Communication: bulletins with the results of the Unidos pela Cura are to be annually produced and sent to managers and health professionals to monitor cases and disseminate the results. Program/Policy process: Unidos pela Cura is a collective and coresponsible initiative to promote the early diagnosis of childhood cancer, created in 2005 and organized into three axes: i) Education - training health professionals in the family health strategy for cancer suspicion; ii) Flow - organization of referral flow and referral of suspected cases of childhood cancer from primary care to specialized hospitals to start the diagnostic investigation within 72 hours; and iii) Information - online system for monitoring suspected cases referred by primary care until diagnostic confirmation, with annual results disclosure. Outcomes: As results, the Unidos pela Cura has been included in the State Cancer Care Plan (2018-2021) and the Municipal Health Plan (2009-2012); 535 doctors and 3038 professionals from the family health strategy; 1311 children and adolescents were referred and 127 confirmed cases of cancer; 37 meetings of the managing group of Unidos pela Cura, among others. What was learned: Collaborative actions between society and governments can contribute to better outcomes and chances of cure.