Abstract
Systemic sclerosis (SSc) is a rare connective tissue disorder characterized by fibrosis, vascular alterations, and organ dysfunction. Juvenile systemic sclerosis (jSSc), an infrequent form of the disease, primarily affects children, with an incidence of 0.27 to 1 per million. This report details the case of a 10-year-old male patient diagnosed with jSSc complicated by interstitial lung disease (ILD) and myositis. The patient initially presented with characteristic signs of skin thickening and Raynaud's phenomenon, which later advanced to the emergence of ILD and myositis. The case emphasizes the importance of vigilant screening for ILD in jSSc patients, given the severity and associated increased mortality of the complication, and improves our understanding of the underlying clinical features of this disease. It also calls attention to the challenges of managing the disease effectively and necessitates further research to improve treatment outcomes for such patients.
Publisher
Al-Rafidain University College
Reference15 articles.
1. Careta MF, Romiti R. Localized scleroderma: clinical spectrum and therapeutic update. Ann Bras Dermatol. 2015;90(1):62-73. doi: 10.1590/abd1806-4841.20152890.
2. Giacomelli R, Liakouli V, Berardicurti O, Ruscitti P, Di Benedetto P, Carubbi F, et al. Interstitial lung disease in systemic sclerosis: current and future treatment. Rheumatol Int. 2017;37:853-863. doi: 10.1007/s00296-016-3636-7.
3. Scalapino K, Arkachaisri T, Lucas M, Fertig N, Helfrich DJ, Londino AV, et al. Childhood onset systemic sclerosis: classification, clinical and serologic features, and survival in comparison with adult onset disease. J Rheumatol. 2006;33(5):1004-1013. PMID: 16583463.
4. Martini G, Foeldvari I, Russo R, Cuttica R, Eberhard A, Ravelli A, et al. Systemic sclerosis in childhood: clinical and immunologic features of 153 patients in an international database. Arthritis Rheum. 2006;54(12):3971-3978. doi: 10.1002/art.22207.
5. Khanna D, Tashkin DP, Denton CP, Renzoni EA, Desai SR, Varga J. Etiology, risk factors, and biomarkers in systemic sclerosis with interstitial lung disease. Am J Respir Critic Care Med. 2020;201(6):650-660. doi: 10.1164/rccm.201903-0563CI.