Abstract
Objective: The aim of this study was to describe the epidemiological, clinical and therapeutic features of systemic scleroderma at Cocody UTH. Methodology: We conducted a retrospective and descriptive study over a period of 10 years (September 15, 2008 to April 15, 2019) on the files of patients hospitalized for systemic scleroderma in the rheumatology unit of the UTH of Cocody. We used the classification criteria of the American Society of Rheumatology (1980) to retain the diagnosis. Results: Nineteen patients’ files had been collected, representing a hospital frequency of 0.32%. The average age was 37.25 ± 13.82 years old. There were 15 women and 4 men. The average consultation time was 26.44 months. The mode of revelation of the disease was mostly cutaneous and articular. All patients had cutaneous sclerosis (average Rodnan score = 27.63/11.61 (min = 4, max = 49).) Scleroderma was diffuse in 70.59% of cases; a Raynaud’s phenomenon was seen in 47.37%. The main clinical manifestations were: cutaneous (100%), articular (89.47%), pulmonary (57.89%) and digestive (63.16%). No renal damage was found. Pulmonary fibrosis (5 cases), pulmonary arterial hypertension (3 cases) and pericardial effusion (2 cases) were sometimes founded in explorations. The positivity of antinuclear antibodies (ANA) was seen in 72% of patients and anti scl70 antibodies in 42.85%. The treatment included corticosteroids and immunosuppressants, respectively used in 84.2% and 63.16% of cases. The outcome was marked by 5 cases of death attributed to respiratory distress. Conclusion: Systemic scleroderma seems to be a very rare condition in Ivorian rheumatology milieu. The main systemic manifestations were digestive and pulmonary. Treatment was very often symptomatic sometimes associated with D-penicillamine.
Highlights
Systemic sclerosis (SSc) is a rare autoimmune disease in which: three entities coexist: vasculopathy, activation of the immune system, and fibrosis [1].The prognosis of this condition is highly variable and is associated with high mortality [2] [3].There are two main forms depending on the cutaneous extension: limited forms and diffuse forms
We conducted a retrospective and descriptive study over a period of 10 years (September 15, 2008 to April 15, 2019) on the files of patients hospitalized for systemic scleroderma in the rheumatology unit of the University Teaching Hospital (UTH) of Cocody
All patients had cutaneous sclerosis (average Rodnan score = 27.63/11.61.) Scleroderma was diffuse in 70.59% of cases; a Raynaud’s phenomenon was seen in 47.37%
Summary
Systemic sclerosis (SSc) is a rare autoimmune disease in which: three entities coexist: vasculopathy, activation of the immune system, and fibrosis [1].The prognosis of this condition is highly variable and is associated with high mortality [2] [3].There are two main forms depending on the cutaneous extension: limited forms and diffuse forms. Systemic sclerosis (SSc) is a rare autoimmune disease in which: three entities coexist: vasculopathy, activation of the immune system, and fibrosis [1]. The prognosis of this condition is highly variable and is associated with high mortality [2] [3]. The SSc is associated with a significant risk of visceral involvement, digestive, pulmonary, cardiac or renal, which significantly reduces survival [4] [5]. The objective of this work was to describe the epidemiological, clinical, immunological and therapeutic characteristics of SSc in the rheumatology department of UTH at Cocody
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