Khadija Ahmadova

Objectives: Exertional leg pain (ELP) is an underrecognized manifestation of FMF with unclear pathophysiology. This study performed a comprehensive ultrasonographic evaluation of lower-extremity joints, tendons and entheses in paediatric FMF patients with ELP, comparing findings with non-ELP patients and matched healthy controls. Methods: Paediatric FMF patients carrying homozygous or compound heterozygous pathogenic MEFV variants were enrolled in this prospective study. Demographic, anthropometric and clinical data were collected, while a blinded operator performed standardized ultrasonographic evaluations. Results: A total of 50 patients and 25 heathy controls were included (25 with ELP, 25 without). No differences were observed between the groups in demographic, anthropometric or FMF-related factors, including MEFV mutations and ISSF scores. Among the evaluated parameters, Achilles tendon thickness was significantly increased in children with ELP, independent of demographic or anthropometric factors, compared with both healthy and non-ELP group (P < 0.01). Nine patients exhibited grade 1 knee synovial effusions, predominantly in the ELP cohort, most with subclinical inflammation. One patient with ELP demonstrated enthesitis and was diagnosed with HLA-B27-negative ERA. Patellar tendon and plantar fascia thickness were comparable. Conclusion: Paediatric FMF patients with ELP demonstrated increased achilles tendon thickness. Although no standardized cut-off exists for tendinopathy, this may reflect tendon structural changes related to ELP. Moreover, FMF patients, particularly those with ELP, exhibited inter-attack knee effusions which may serve as a marker for subclinical inflammation. Larger longitudinal cohorts are needed to define ELP, elucidate its underlying pathways and clarify the clinical significance of Achilles tendon thickening.

Oligoarticular juvenile idiopathic arthritis (oJIA) is the most common JIA subtype, with variable disease courses influencing long-term outcomes. Joint involvement patterns significantly affect disease activity, treatment response, and prognosis. This study evaluates the impact of joint involvement on disease activity, inflammatory markers, treatment needs, and disease extension in patients with oJIA. We retrospectively analysed 124 oJIA patients followed for a median of 62 months. Joint involvement, disease activity (Juvenile Arthritis Disease Activity Score [JADAS-27]), inflammatory markers (erythrocyte sedimentation rate [ESR], C-reactive protein [CRP]), and treatment modalities were assessed. Persistent and extended disease courses were compared. A total of 124 patients with oJIA (63.7% female) were included, with a median age at diagnosis of 8.2 years. The knee was the most frequently involved joint (87.1%), followed by the ankle (41.1%) and hip (22.6%). Patients with ankle, wrist, and elbow involvement had significantly higher JADAS-27 scores at baseline. Notably, ankle involvement was associated with sustained higher disease activity up to the first year and higher final CRP levels. Repeated measures ANOVA demonstrated a significant decline in JADAS-27 over time across all joint subgroups (p<0.001), with no joint-specific difference in trajectory. Ankle and small hand joint (MCP/PIP) involvement were significantly associated with increased use of cDMARDs and anti-TNF agents. Patients with extended oJIA had significantly higher JADAS-27 scores at all timepoints except the final visit and were more likely to have symmetric joint involvement and receive biologic therapy compared to those with persistent oJIA (anti-TNF use: 70% vs. 25%, p<0.001). Joint involvement patterns, particularly affecting the ankle, wrist, and small hand joints, are associated with higher disease activity and increased treatment requirements in oJIA. Symmetrical and extended disease forms further indicate a more severe course. Early identification of these high-risk features may guide personalized treatment and improve long-term outcomes.