Juvenile Systemic Sclerosis Complicated by Interstitial Lung Disease and Myositis: A Case Report
DOI:
https://doi.org/10.54133/ajms.v5i1S.344Keywords:
Interstitial lung disease, Juvenile systemic sclerosis, Mycophenolate mofetil, Myositis, Systemic sclerosisAbstract
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.
Downloads
References
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.
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.
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.
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.
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.
Herrick AL, Ennis H, Bhushan M, Silman AJ, Baildam EM. Incidence of childhood linear scleroderma and systemic sclerosis in the UK and Ireland. Arthritis Care Res. 2010;62(2):213-218. doi: 10.1002/acr.20070.
Céspedes-Cruz AI, Carranza-Muleiro RA, López-Rojas EL, Cruz-Domínguez MP, Espinosa-Gan H, Ramírez-Pérez J, et al. Pulmonary involvement in patients with juvenile systemic sclerosis. Bol Med Hosp Infant Mex. 2021;78(5):385-394. doi: 10.24875/BMHIM.20000301.
Yuen LK, Lai WM, Tse KC, Chiu MC. Two cases of juvenile systemic sclerosis and literature review. HK J Paediatr. 2007;12:221-226.
Tyndall AJ, Bannert B, Vonk M, Airò P, Cozzi F, Carreira PE, et al. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis. 2010;69(10):1809-1815. doi: 10.1136/ard.2009.114264.
Matuszewska G, Zaniewicz-Kaniewska K, Włodkowska-Korytkowska M, Smorawińska P, Saied F, Kunisz W, Sudoł-Szopińska I. Radiological imaging in pediatric rheumatic diseases. Pol J Radiol. 2014;79:51-58. doi: 10.12659/PJR.889816.
Torok KS. Pediatric scleroderma: systemic or localized forms. Pediatr Clin North Am. 2012;59(2):381-405. doi: 10.1016/j.pcl.2012.03.011.
Quartier P, Bonnet D, Fournet JC, Bodemer C, Acar P, Ouachee-Chardin M, et al. Severe cardiac involvement in children with systemic sclerosis and myositis. J Rheumatol. 2002;29(8):1767-1773. PMID: 12180742.
Elhai M, Boubaya M, Distler O, et al. Outcomes of patients with systemic sclerosis treated with rituximab in contemporary practice: a prospective cohort study. Ann Rheum Dis. 2019;78(7):979-987. doi:10.1136/annrheumdis-2018-214816.
Perelas A, Silver RM, Arrossi AV, Highland KB. Systemic sclerosis-associated interstitial lung disease. Lancet Respir Med. 2020;8(3):304-320. doi:10.1016/S2213-2600(19)30480-1.
Tashkin DP, Roth MD, Clements PJ, Furst DE, Khanna D, Kleerup EC, et al. Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial. Lancet Respir Med. 2016;4(9):708-719. doi: 10.1016/S2213-2600(16)30152-7.
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2023 Al-Rafidain Journal of Medical Sciences ( ISSN 2789-3219 )
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
Published by Al-Rafidain University College. This is an open access journal issued under the CC BY-NC-SA 4.0 license (https://creativecommons.org/licenses/by-nc-sa/4.0/).