Familial aggregation of juvenile idiopathic arthritis with other autoimmune diseases: Impact on clinical characteristics, disease activity status and disease damage

Abstract

Objectives To evaluate the impact of family history of autoimmune diseases (FHADs) on the clinical characteristics and outcome of juvenile idiopathic arthritis (JIA). Methods We retrospectively reviewed children with JIA seen in 7 pediatric rheumatology clinics from 6 Arab countries. All included patients met the International League of Associations for Rheumatology classification criteria for JIA and had a disease duration greater than 1 year. Data were collected at the last follow‐up visit and comprised clinical findings, including FHADs. Disease activity and disease damage were assessed by Juvenile Arthritis Multidimensional Assessment Report, and juvenile arthritis damage index (JADI) respectively. Disease activity was categorized as remission off treatment, remission on treatment, or active disease. Results A total of 349 (224 females) JIA patients with a disease duration of 5 (interquartile range 2.9‐7.5) years were included. The most frequent JIA categories were polyarticular JIA and oligoarticular JIA, followed by systemic JIA. There were 189 patients with FHADs and 160 patients without FHADs. The most frequent FHADs were diabetes mellitus (21.2%), JIA (18.5%), rheumatoid arthritis (12.7%). Among patients with FHADs, 140/189 (74.1%) achieved clinical remission, while 131/160 (81.9%) patients without FHDs had clinical remission (odds ratio [OR] = 1.2, 95% CI 0.97‐1.5). Rate of consanguinity, enthesitis‐related arthritis (ERA) and psoriatic arthritis were higher in patients with FHADs (OR = 0.6, 95% CI 0.4‐0.9 and OR = 1.2, 95% CI 1.1‐1.4). Also, articular JADI correlated significantly with presence of FHADs (OR = 1.1, 95% CI 1.0‐1.1). Conclusion This study shows that autoimmune diseases cluster within families of patients with JIA with a high proportion of ERA and psoriatic arthritis. JIA patients with FHADs are likely to have more disease damage.

Prevalence of familial autoimmune diseases in juvenile idiopathic arthritis: results from the international Pharmachild registry

“…A small study by Tronconi et al [8] did not find an association between a family history of AD and the subtype and age at onset of JIA. On the contrary, the current study observed that patients with psoriatic arthritis, undifferentiated arthritis and ERA reported relatively often a family history of AD as opposed to patients with systemic arthritis and oligoarthritis, which is also in line with two previous studies [4,6]. The association between HLA-B27 and a family history of AD corresponds with the observed effect for JIA category, given that, 73.7% of ERA patients and 38.3% of undifferentiated arthritis patients in our study were HLA-B27 positive.…”

“…This can be explained by the autoinflammatory instead of autoimmune nature of this JIA category [1]. Previous studies have reported contradictory relationships between the age at JIA onset and a family history of AD [5,6,8,[39][40][41]. In this study, familial AD was associated with older age at JIA onset.…”

“…In the current study, we found no effect of familial autoimmunity on (the course of ) disease activity in the included JIA patients. Previously, two studies reported that JIA patients with a family history of AD had higher disease activity and longer active disease duration than JIA patients without such family history [5,6]. This contradiction might be a result of differences in the target population and study design, given that one of the mentioned studies included a highly consanguineous JIA population from Saudi Arabia and the other study only included JIA patients from Iran in a case-control design.…”

“…A previous study reported that 32% of 4677 JIA patients had at least one first-degree relative with an AD [4]. Furthermore, JIA patients with a family history of AD were reported to have higher disease activity and more often enthesitis-related arthritis (ERA) and psoriatic arthritis than JIA patients without such family history [5,6]. Frequently described ADs in relatives of JIA patients are insulin-dependent diabetes mellitus (IDDM), JIA, rheumatoid arthritis (RA), autoimmune thyroid disease (AITD), spondyloarthropathy and psoriasis [4][5][6][7][8][9][10][11].…”

“…Furthermore, JIA patients with a family history of AD were reported to have higher disease activity and more often enthesitis-related arthritis (ERA) and psoriatic arthritis than JIA patients without such family history [5,6]. Frequently described ADs in relatives of JIA patients are insulin-dependent diabetes mellitus (IDDM), JIA, rheumatoid arthritis (RA), autoimmune thyroid disease (AITD), spondyloarthropathy and psoriasis [4][5][6][7][8][9][10][11]. Nonetheless, the few studies about familial autoimmunity in JIA are either based on a limited number of patients, do not report prevalence rates within families or only report pre-selected ADs.…”