Towards a standardized program of transitional care for adolescents with juvenile idiopathic arthritis for Turkey: a national survey study.

BACKGROUND: Juvenile idiopathic arthritis (JIA) is a prevalent childhood chronic arthritis, often persisting into adulthood. Effective transitional care becomes crucial as these patients transition from pediatric to adult healthcare systems. Despite the concept of transitional care being recognized, its real-world implementation remains inadequately explored. This study aims to evaluate the thoughts and practices of healthcare providers regarding transitional care for JIA patients. METHODS: A cross-sectional survey was conducted among pediatric and adult rheumatologists in Turkey. Based on the American Academy of Pediatrics' six core elements of transitional care, the survey included 86 questions. The respondents' demographic data, attitudes towards transitional care, and practical implementation were assessed. RESULTS: The survey included 48 rheumatologists, with 43.7% having a transition clinic. The main barriers to establishing transition programs were the absence of adult rheumatologists, lack of time, and financial constraints. Only 23.8% had a multidisciplinary team for transition care. Participants agreed on the importance of coordination and cooperation between pediatric and adult healthcare services. The timing of the transition process varied, with no consensus on when to initiate or complete it. Participants advocated for validated questionnaires adapted to local conditions to assess transition readiness. CONCLUSIONS: The study sheds light on the challenges and perspectives surrounding transitional care for JIA patients in Turkey. Despite recognized needs and intentions, practical implementation remains limited due to various barriers. Cultural factors and resource constraints affect the transition process. While acknowledging the existing shortcomings, the research serves as a ground for further efforts to improve transitional care and ensure better outcomes for JIA patients transitioning into adulthood.

Atezolizumab-induced myositis in a patient with small-cell lung cancer.

OBJECTIVE: Myositis, an inflammatory disease affecting muscles, is a rare and potentially fatal immune-related adverse event associated with immune checkpoint inhibitors. There are limited data on its clinical features and management. CASE PRESENTATION: Atezolizumab, in combination with etoposide and carboplatin, was initiated in the patient diagnosed with metastatic small-cell lung cancer. After four cycles, maintenance atezolizumab was initiated. At the third visit of the maintenance therapy, the patient reported weakness, edema, and tightness in the muscles that had progressed over the course of a week. Mild solid-food dysphagia was also observed. Neutrophilic leukocytosis with elevated creatine phosphokinase (9234 U/L), erythrocyte sedimentation rate (111 mm/h), and transaminase levels were observed. A diagnosis of myositis was considered based on clinical findings. Atezolizumab was omitted and an oral 0.5 mg/kg/day dose of methylprednisolone was administered. The myositis resolved within 10 days. During the treatment of myositis, the patient underwent prophylactic cranial irradiation. The steroid dose was tapered off within 35 days and then atezolizumab was restarted. CONCLUSION: The literature contains only a few case reports about atezolizumab-induced myositis, highlighting the challenges in defining its clinical features and management. Prompt diagnosis and treatment are crucial to prevent severe complications, such as myocarditis or respiratory muscle paralysis.

Disease severity and genotype-phenotype correlation in adult patients with familial Mediterranean fever.

OBJECTIVES: To assess the relationships of disease severity with genotype and phenotype in adult familial Mediterranean fever patients. METHODS: Two-hundred seventy-five patients included in the study were divided into four groups according to their mutations: Group 1, M694V homozygous; Group 2, M694V-other; Group 3, other-other; and Group 4, no meaningful gene variants. Disease severity was evaluated using the Pras disease severity score. The association between Pras scores and other possible predictors was assessed by the multiple linear regression analysis. RESULTS: In this study, 12.4% of all patients were in Group 1, 55.3% were in Group 2, 26.5% were in Group 3, and 5.8% were in Group 4. Pras scores were higher in Group 1 than in Groups 2, 3, and 4 (post hoc pairwise comparisons; P = .001, P < .001, and P = .001, respectively). Age at disease onset and age at diagnosis were found moderately and strongly correlated with Pras scores. Patients with moderate and severe disease were intensely involved in Group 1. CONCLUSIONS: Higher Pras scores, earlier age of symptoms and diagnosis, more frequent arthritis and erysipelas-like erythema, and higher colchicine dose are closely associated with M694V homozygous familial Mediterranean fever patients. These patients also have mostly moderate and severe disease severity.