Objective: This nationwide, multicenter study was conducted to assess the demographic and clinical features, treatment regimens, and prognosis of primary Sjögren disease (SjD) in childhood. Methods: This retrospective study included a total of 81 patients < 18 years of age from 21 pediatric rheumatology centers. Among these, 51 patients fulfilled the diagnosis of childhood SjD according to the 2016 American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) classification criteria. The remaining 30 patients—who did not fully meet these criteria but exhibited clinical and laboratory findings suggestive of SjD—were categorized as at risk for childhood SjD to highlight diagnostic challenges and the spectrum of early presentations, based on comprehensive clinical evaluation by experienced pediatric rheumatologists. Results: The cohort consisted of 81 patients (85.2% female, 14.8% male) with a median age at symptom onset of 11.4 years and a median follow-up of 24 months. Common clinical manifestations included dry mouth, dry eyes, arthralgia, fatigue, and parotitis. Fifty-one of the 81 patients met the 2016 ACR/EULAR classification criteria, whereas the remaining 30 were classified as an at-risk group. The most common clinical findings in the at-risk group were xerostomia (90%), arthralgia (56.7%), fatigue (50%), and dry eyes (43.3%). Dry mouth and peripheral nervous system involvements were found to be higher in patients categorized as at risk ( P = 0.03 and P = 0.02, respectively). Conclusion: The current classification criteria for childhood SjD appear to be inadequate, highlighting the need for pediatric-specific criteria that more accurately reflect the distinct clinical patterns observed in children.

Introduction: Chronic nonbacterial osteomyelitis (CNO) is a rare autoinflammatory bone disorder. To standardize management, the Childhood Arthritis and Rheumatology Research Alliance (CARRA) developed three consensus treatment plans (CTPs) (1: methotrexate or sulfasalazine [conventional disease-modifying antirheumatic drugs; DMARDs], 2: tumor necrosis factor (TNF)-alpha inhibitors (TNFi), and 3: bisphosphonates). The Clinical Disease Activity Score (CNO CDAS) was introduced to assess disease activity without relying solely on imaging. Objective: The objectives were to evaluate clinicians’ tendencies in applying CNO-CTPs and to assess treatment responses using CNO CDAS in real-world practice, including therapeutic strategies and outcomes at final follow-up. Methods: A retrospective cohort study of 171 pediatric patients with CNO from 18 centers was conducted. Disease activity was assessed using CDAS at diagnosis, at treatment modification, and at the last visit. This score incorporates clinically active lesions, patient pain, and global assessment. Treatments included NSAIDs, DMARDs, TNFi, and pamidronate. Results: Median age was 10 years at onset and 14 years at last follow-up, with a median follow-up of 36 months. The most frequent symptoms were bone pain (95.3%), activity limitation (67.8%), and fatigue (35.1%). All patients received NSAIDs; methotrexate (85.4%) and TNFi (54.4%) were commonly used. TNF inhibitors were the most used CTP, especially in patients with spinal involvement or higher MRI lesion counts, and they achieved the highest remission rate (40.4%). Of the 89 (52.0%) patients who changed treatment, 93.2% switched from DMARDs to TNFi. At the final follow-up, 62% were in medicated remission, 29.2% were in unmedicated remission, and 8.8% had active disease. CDAS scores significantly improved over time (p < 0.05). Conclusions: CARRA CTPs are effective in clinical practice, with anti-TNF therapy yielding the highest remission rates and marked improvement in CDAS.

Objective: This study aimed to evaluate serum phoenixin-14 (PNX-14) levels in patients with familial Mediterranean fever (FMF) and periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) during both attack and attack-free periods to investigate its potential biomarker value. Methods: In total, 140 children were included in this cross-sectional study: 46 with FMF, 48 with PFAPA, and 46 healthy children. Blood samples were collected during both febrile and attack-free periods in the patients with FMF and PFAPA, whereas samples from healthy controls were collected at a single timepoint. C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), serum amyloid A (SAA), fibrinogen, and serum PNX-14 levels were evaluated. Serum PNX-14 levels were measured using ELISA. SPSS 25 (IBM) was used for statistical analyses. Results: PNX-14 levels were significantly higher during attack periods in FMF and PFAPA patients compared to attack-free periods and healthy controls ( P < 0.001). PNX-14 levels during attack-free periods were also higher in both patient groups than in healthy controls ( P < 0.001). Although a positive correlation was observed between PNX-14 levels and CRP (ρ 0.222, P = 0.04), no significant correlation was observed with ESR, SAA, or fibrinogen. No significant associations were observed between PNX-14 levels and disease severity (Pras disease severity score), colchicine treatment duration, or daily colchicine dose in the FMF group. Conclusion: PNX-14 may be part of a neuroimmune response aimed at resolving inflammation in autoinflammatory processes and could be a potential immunomodulatory biomarker. PNX-14 may be a complementary indicator reflecting inflammatory burden or the healing process.

Liver enzyme elevations may occur during follow-up of children with familial Mediterranean fever [FMF], but their underlying mechanisms remain unclear. Thi

Panniculitis is a rare clinical finding in dermatomyositis. There are few reported cases in the medical literature. In this report, we describe a 17-year-old male patient with anti-MDA5 positive hypomyopathic dermatomyositis who, eight months after diagnosis, presented with indurated nodules on the right forearm and right thigh despite methotrexate and monthly intravenous immunoglobulin treatment. A skin biopsy revealed lobular panniculitis with lymphocytic infiltrate. His lesions were successfully controlled with hydroxychloroquine and azathioprine. This article presents a case regarding the rarity of panniculitis in juvenile dermatomyositis and its treatment strategy.