Background/Purpose: Localized scleroderma (LS), including morphea and linear scleroderma, is an autoimmune disease where excessive collagen deposits underneath the skin lead to thickening, scarring, and fibrosis. Often limited to the skin, affecting only the outer layer and subcutaneous tissues, this disease may be one manifestation of a diffuse systemic inflammatory process. LS coexisting with juvenile idiopathic arthritis (JIA) is less well-described but has been reported in as many as 20% in a recent cohort of 53 LS patients. Herein, we describe a cohort of 8 children with both LS and JIA. The objective of this study is to determine the characteristics of JIA in children with LS and response to treatment regimens.

Methods: A retrospective chart review was completed on patients less than 19 years of age who were diagnosed with either morphea or linear scleroderma at a single institution from 2004-2018. Patients were identified using ICD-9 and ICD-10 diagnostic codes. Records were reviewed for additional diagnostic codes, exams, and laboratory findings confirming coexisting JIA.

Results: A total of 87 patients with a diagnosis of either morphea or linear or circumscribed scleroderma were identified. Eight (9%) also had coexisting JIA per diagnostic codes with active arthritis as documented by a pediatric rheumatologist. Median age of initial rheumatic disease diagnosis was 7.5 years. A majority of patients with both LS and JIA were female (62.5%). Half of the patients (n=4, 50%) had LS lesions over arthritic joints. JIA subtype varied widely with 3 (37.5%) patients with rheumatoid factor (RF) negative polyarticular JIA, 2 (25%) patients with oligoarticular JIA, 2 (25%) patients with psoriatic JIA, and 1 (12.5%) with enthesitis-related JIA. The timing of onset of LS and JIA also varied widely. Three (37.5%) patients had LS lesions preceding the appearance of clinical arthritis on exam, and three (37.5%) patients had arthritis before the appearance of LS. Two (25%) patients had both LS and arthritis at time of diagnosis. Two (25%) of the 8 patients had a positive ANA screen, both less than or equal to 1:320 with negative extractable nuclear antigen panels. All patients received methotrexate (MTX) during their disease course with only three (37.5%) receiving systemic steroids during treatment. All 8 patients had resolution of LS lesions. With respect to active joint count, 1 patient achieved remission on MTX alone, and 1 patient achieved remission on MTX and a tumor-necrosis factor inhibitor (TNFi). Six of the 8 patients had active arthritis on combination MTX and TNFi therapy.

Conclusion: In this cohort of pediatric patients with LS, 9% had coexisting JIA, which is lower than the 20% reported in previous studies. The characteristics of this cohort who had both LS and JIA varied widely. Presence of autoantibodies, including ANA titer, was inconsistent. All patients received MTX initially with resolution of LS lesions. However, the majority failed to respond to MTX and TNFi combination therapy and continued to have active arthritis.These results suggest that JIA coexisting with LS may be less likely to respond to traditional JIA therapies.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/characteristics-of-coexisting-localized-scleroderma-and-juvenile-idiopathic-arthritis/