E36 Juvenile scleroderma in rheumatology unit Tripoli children hospital

Abstract

Abstract Introduction Juvenile scleroderma is a rare chronic systemic connective tissue disease. It is classified into two main forms: localized scleroderma and systemic sclerosis. Since it is rare in children, many aspects of the disease remain undiscussed. Aim To study the clinical and demographic characteristics of patients diagnosed with various types of scleroderma who presented at the pediatric rheumatology unit in Tripoli Children hospital from the year 2000–2022 Materials and methods This is a retrospective case series conducted by reviewing the files of patients with scleroderma diagnosed and followed up in pediatric rheumatology unit from year 2000–2022 and collecting the data regarding patients’ demographics, time of diagnosis, clinical features, and laboratory results. Results Seven patients who met preliminary classification criteria for JSS were included. 2 males and 5 females. 1 patient with systemic sclerosis, 1 patient with linear morphea, 2 patients with generalized morphea, 1 patient with Parry Romberg syndrome, 1 patient with CREST syndrome, and 1 patient with deep morphea. The mean age at diagnosis was 8.2 ± 4.5 years. The first symptom attributable to JSS was hyperpigmented skin patches in 5 cases, no one presented with Raynaud's phenomenon. Respiratory involvement occurred in one patient, and it manifested as dyspnea as well as abnormal imaging and/or pulmonary function tests showed mild restrictive lung disease. Dysphagia was the commonest gastrointestinal symptom (4 patients, 57%). The most frequent musculoskeletal symptom was arthritis found in in 4 (57.1%) patients. Uveitis in 1 (14.3%), ANA was positive in 4 (57%) children: RF positive in 2 (28.6%) patients, ENA negative in the 7 (100%) patients. Skin biopsy was done in 2 patients and was consistent with scleroderma. Regarding treatment, six patients received MTX, one patient received prednisolone and one patient received MMF. Conclusion This report is the first describing Libyan patients with scleroderma. As reported in the literature, localized forms are more common than systemic sclerosis. Also a rare form of scleroderma (Parry Romberg syndrome) was present. Data about the disease mainly based on studies on adults and we need more studies on pediatric patients. Ethics Our study was approved by local ethical committee of pediatric department at Tripoli children hospital.