Scleroderma is a rare disease with two primary
forms: localized scleroderma (LS) and systemic sclerosis (SSc). Both are
chronic conditions that can manifest in
various patterns (subtypes) and are linked to extracutaneous involvement
in pediatric patients. Juvenile SSc poses a higher risk of morbidity and mortality, with patients facing life-threatening
complications such as lung, heart, and visceral organ fibrosis, and
vasculopathy. In contrast, mortality is extremely rare in juvenile LS, but
patients are susceptible to significant morbidity, leading to severe
disfigurement and functional impairment. Treatment for scleroderma aims to
control inflammation and address specific issues. An early diagnosis significantly enhances the overall outcome. This study conducts
a retrospective descriptive analysis aiming to document the clinical
manifestations, management approaches, and outcomes of systemic sclerosis in a
cohort of nine children receiving treatment for juvenile systemic sclerosis at Pediatric B department of Mohammed VI University,
Hospital Center in Marrakech, Morocco.
References
[1]
Smith, V., Herrick, A.L., Ingegnoli, F., Damjanov, N., De Angelis, R., Denton, C.P., et al. (2020) Standardisation of Nailfold Capillaroscopy for the Assessment of Patients with Raynaud’s Phenomenon and Systemic Sclerosis. Autoimmunity Reviews, 19, Article ID: 102458. https://doi.org/10.1016/j.autrev.2020.102458
[2]
Herrick, A.L., Ennis, H., Bhushan, M., et al. (2010) Incidence of Childhood Linear Scleroderma and Systemic Sclerosis in the UK and Ireland. Arthritis Care & Research, 62, 213-218. https://doi.org/10.1002/acr.20070
[3]
Beukelman, T., Xie, F. and Foeldvari, I. (2018) Assessing the Prevalence of Juvenile Systemic Sclerosis in Childhood Using Administrative Claims Data from the United States. Journal of Scleroderma and Related Disorders, 3, 189-190.
https://doi.org/10.1177/2397198318763701
[4]
Foeldvari, I., Klotsche, J., Torok, K.S., et al. (2018) Characteristics of the First 80 Patients at Timepoint of First Assessment Included in the Juvenile Systemic Sclerosis Inception Cohort. Journal of Scleroderma and Related Disorders, 4.
[5]
Stevens, B.E., Torok, K.S., Li, S.C., et al. (2018) Clinical Characteristics and Factors Associated with Disability and Impaired Quality of Life in Children with Juvenile Systemic Sclerosis: Results from the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry. Arthritis Care & Research, 70, 1806-1813.
https://doi.org/10.1002/acr.23547
[6]
Zulian, F., Woo, P., Athreya, B.H., Laxer, R.M., Medsger Jr., T.A., Lehman, T.J., et al. (2017) The Pediatric Rheumatology European Society/American College of Rheumatology/European League against Rheumatism Provisional Classification Criteria for Juvenile Systemic Sclerosis. Arthritis & Rheumatology, 57, 203-212.
https://doi.org/10.1002/art.22551
[7]
Masi, A.T., Rodnan, G.P., Medsger Jr., T.A., Altman, R.D., D’Angelo, W.A., Fries, J.F., et al. (1980) Preliminary Criteria for the Classification of Systemic Sclerosis (Scleroderma). Arthritis & Rheumatology, 23, 581-590.
https://doi.org/10.1002/art.1780230510
[8]
van den Hoogen, F., Khanna, D., Fransen, J., Johnson, S.R., Baron, M., Tyndall, A., et al. (2013) 2013 Classification Criteria for Systemic Sclerosis: An American College of Rheumatology/European League against Rheumatism Collaborative Initiative. Annals of the Rheumatic Diseases, 72, 1747-1755.
https://doi.org/10.1136/annrheumdis-2013-204424
[9]
Zulian, F. (2017) Scleroderma in Children. Best Practice & Research Clinical Rheumatology, 31, 576-595. https://doi.org/10.1016/j.berh.2018.02.004
[10]
Foeldvari, I., Culpo, R., Sperotto, F., Anton, J., Avcin, T., Baildam, E., et al. (2021) Consensus-Based Recommendations for the Management of Juvenile Systemic Sclerosis. Rheumatology (Oxford), 60, 1651-1658.
https://doi.org/10.1093/rheumatology/keaa584
[11]
Kowal-Bielecka, O., Fransen, J., Avouac, J., Becker, M., Kulak, A., Allanore, Y., et al. (2017) Update of EULAR Recommendations for the Treatment of Systemic Sclerosis. Annals of the Rheumatic Diseases, 76, 1327-1339.
https://doi.org/10.1136/annrheumdis-2016-209909
[12]
Fernandez-Codina, A., Walker, K.M., Pope, J.E., Scleroderma Algorithm Group (2018) Treatment Algorithms for Systemic Sclerosis According to Experts. Arthritis & Rheumatology, 70, 1820-1828. https://doi.org/10.1002/art.40560
