E16 Extra-articular manifestations and hip involvement in juvenile idiopathic arthritis: what is the link?

Abstract

Abstract Introduction Juvenile idiopathic arthritis (JIA) is a complex pediatric rheumatism. The systemic and structural involvement of the disease is both functional and life threatening. Hip involvement can reach 50% of cases, especially in severe and destructive cases. Objectives The objective of this work is to study the relationship between systemic and hip involvement in JIA. Methods This was a retrospective study including adults with long-standing JIA according to the International League of Associations for Rheumatology (ILAR) criteria over a period of 28 years (1994–2022). Socio-demographic, clinical, biological, and radiographic data were collected. Results There were 29 patients (17 females and 12 males), the mean age was 35.69 ± 11.72 [18–61] years. The mean age of disease onset was 11.10 ± 4.25 [2–16] years. The average diagnostic delay was 52.96 ± 95.97 [0–336] months. The average disease duration was 24.48 ± 12.76 [1–47] years. At least one extra-articular manifestation was noted in 20 patients. An ocular dry syndrome was present in 6 cases, with a delay of onset of 132.14 ± 134.18 [0–312] months. Skin involvement was present in 4 cases with a mean onset delay of 24.25 ± 47.83 [0–96] months. Three patients had a pulmonary interstitial syndrome, the onset delay of this pulmonary involvement was 228.00 ± 74.94 [0–312] months. Valvular involvement was present in 3 cases and pericarditis in only one case, the onset delay of cardiac involvement was 211.33 ± 129.40 [70–324] months. Renal involvement was noted in 2 cases: one case of proteinuria and one case of renal failure, the time of onset of renal involvement was 12.50 ± 16.26 [1–24] months. Furthermore, hematological damage such as anaemia was noted in 20 of the patients included. No patients had macrophage activation syndrome in our study population. Hip involvement was noted in 14 patients. It was bilateral in 9 cases. Twenty-three hips were affected in total. Hip involvement was diagnosed 14.5 ± 9.37 [1–28] years after disease onset. Our statistical study identified a single correlation between hip involvement and cardiac involvement during JIA (r = 0.413; P = 0.026). In contrast, no other correlation was identified between hip involvement and the rest of the extra-articular involvement in JIA. Conclusion Extra-articular and hip involvements are common in JIA. Both have a prognostic impact and must be systematically screened throughout the follow-up. In our study, hip involvement was correlated with cardiac involvement.