Diagnosing and Treating Systemic Juvenile Idiopathic Arthritis and Adult-Onset Still's Disease as Part of the Still's Disease Continuum.

Aim: We have summarised the existing evidence supporting the concept that systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD) are part of the same Still's disease spectrum. Methods: A PubMed/Embase database search was conducted using specific search strings and free text words to screen for relevant articles. The search was limited to studies in humans, published up to June 2023, in English-language. Summary: sJIA and AOSD are rare autoinflammatory disorders that have similar pathophysiological and clinical features. The clinical presentations of sJIA and AOSD are highly variable, with differential diagnoses that include a broad range of malignancies, infectious diseases, and autoimmune disorders, which contribute to delays in diagnosis. Several sets of classification exist to help diagnose patients in clinical practice; the International League of Associations for Rheumatology criteria for sJIA and the Yamaguchi and Fautrel criteria for AOSD are the most-used criteria. The therapeutic strategy for Still's disease aims to relieve signs and symptoms, prevent irreversible joint damage and potentially life-threatening complications, and avoid deleterious side effects of treatment. Recently, targeted therapies such as interleukin (IL)-1 and IL-6 inhibitors have become available for the treatment of sJIA and AOSD. While these biologics were originally largely reserved for patients in whom non-steroidal anti-inflammatory drugs, corticosteroids and conventional synthetic disease-modifying anti-rheumatic drugs had failed, they are increasingly used earlier in the treatment paradigm. Among IL-1 inhibitors, canakinumab is the only biologic approved in the US for the treatment of both sJIA and AOSD.

Articular and Extra-Articular Benefits in ACR20 Non-responders at Week 104 Treated With Apremilast: Pooled Analysis of Three Randomized Controlled Trials.

INTRODUCTION: PALACE 1, 2, and 3 were phase 3 studies aimed to evaluate apremilast efficacy and safety in patients with active psoriatic arthritis (PsA) despite prior treatment with conventional disease-modifying anti-rheumatic drugs and/or biologics. The pooled analysis reported here further characterized the clinical outcomes associated with long-term apremilast exposure in patients failing to achieve ≥ 20% improvement in the American College of Rheumatology response criteria (ACR20) at Week 104. METHODS: Patients randomized to apremilast 30 mg twice daily at baseline and classified as ACR20 non-responders (ACR20NRs) or ACR20 responders (ACR20Rs) at Week 104 were included. Efficacy outcomes included change from baseline to Week 104 in ACR core components and other endpoints. RESULTS: At Week 104, a total of 109 patients were ACR20NRs and 193 were ACR20Rs. As expected, the ACR20R group had improvements in all indices assessed. The ACR20NR group demonstrated substantial mean improvements from baseline in swollen joint count (SJC; - 58%), tender joint count (TJC; - 42%), and Physician's Global Assessment of Disease Activity (PhGA; - 44%); resolution of enthesitis (34%) and dactylitis (68%); and achievement of ≥ 75% reduction from baseline Psoriasis Area and Severity Index scores (among patients with psoriasis involving ≥ 3% of the body surface area) (36%). CONCLUSION: Despite not fulfilling a formal ACR20 response at Week 104, ACR20NRs experienced sustained improvements in several PsA core domains, including SJC, TJC, enthesitis, dactylitis, and psoriasis, as well as the PhGA (visual analog scale) scores, with apremilast treatment. TRIAL REGISTRATION: ClinicalTrials.gov identifier: NCT01172938, NCT01212757, and NCT01212770.