Autoimmune Systemic Diseases in Children: Epidemiology and Clinical Phenotypes in Senegal

Abstract

Introduction: Systemic diseases (SD) include all non-organ-specific autoimmune and/or inflammatory disorders. In children, their severity is related to severe visceral damage and iatrogenic complications of treatment. This study aimed to determine the epidemiological, clinical evolutionary aspects of systemic diseases in children. Methodology: A cross-sectional, analytic study was carried out in the Dermato-pediatrics Department of the Albert Royer Hospital in Dakar from January 2020 to June 2022 (30 months). We included all children aged 0 to 16 years followed up for systemic autoimmune disease. Results: We collected 18 cases of systemic diseases in children, representing a hospital frequency of 0.36%. The SD were of the lupus type in 7 cases, dermatomyositis in 4 cases, scleroderma in 3 cases, mixed connectivity’s in 3 cases and APLS in 1 case. The sex ratio was 0.12. The mean age of the children was 10 years [4-14 years]. In lupus, lesions were acute in 5 cases, subacute in 1 case and chronic in 1 case. In dermatomyositis, the cutaneous manifestations were: periorbital erythredema, ulcer-necrotic lesions, atrophic lesions of photo-exposed areas, non-erosive cheilitis, a non-scarring alopecia, Gottron papules, poikiloderma and calcinosis. Dermatomyositis was associated with extracutaneous muscular, articular, cardiovascular and pulmonary involvement. In scleroderma, cutaneous manifestations included sclerodactyly, Raynaud's phenomenon and cutaneous sclerosis. Visceral involvement included rhythm disturbances and pulmonary fibrosis. Necrotic ulcers and cyanosis of the extremities were the circumstances in which APLS was discovered. The association lupus-dermatomyositis represented (66.7%) and lupus-APLS (33.3%). Corticosteroid therapy was administered in 38.8% of cases. The outcome was favorable in 27.77% (n=5), with death noted in 3 cases. Conclusion: Systemic autoimmune diseases of children are rare disorders. They are characterized by their clinical polymorphism and the severity of visceral damage. Early treatment and therapeutic education of parents can improve prognosis.