Clinical and serological correlation of systemic sclerosis in Moroccan patients

Abstract

Objective Systemic sclerosis (SSc) is a connective tissue disorder characterized by excessive fibrosis of the skin and internal organs, along with microvascular damage, and is often associated with typical autoantibodies. This study aimed to analyze the correlation between specific autoantibody profiles, clinical and paraclinical features in Moroccan patients with SSc. Methods We analyzed the presence of specific autoantibodies in 46 SSc patients using indirect immunofluorescence (IIF) on Hep-2 cells and immunodot. We then correlated the type of autoantibodies with clinical and laboratory manifestations. Results Among our patients, 86.9% were females. The mean age of patients at diagnosis was 50.21 years old, with an average diagnosis delay of 5 years. The main clinical manifestations found were Raynaud’s phenomenon (89.2%), sclerodactyly (84.8%), proximal scleroderma (67.4%), gastrointestinal involvement (50%), and interstitial lung disease (30.4%). According to the specific autoantibody profile, 14 patients were anti-topo I positive (30.4%), 8 were anti-RNP(68kDa/A/C) positive (17.4%) and 6 were anti-RNA polymerase III positive (13%). We found a significant association of Anti-RNAP III with sclerodactyly and pulmonary arterial hypertension (p < 0.05). We also found an association between anti-topo I and interstitial lung disease in 30.4% of patients. There was no significant association between the positivity for the autoantibodies and other diagnosed clinical manifestations. Conclusion Some clinical manifestations of SSc may correlate positively with the presence of specific autoantibodies. Environmental factors, ethnicity, and gene interaction may also influence this correlation. Lay summary What does this mean for patients? Systemic sclerosis (SSc) is a disease that affects the connective tissues, causing excessive skin and internal organs fibrosis and microvascular damage. Specific autoantibodies, which are antibodies that attack the body's tissues, are responsible for amplifying the immune response and targeting cells involved in the development of SSc. This study aimed to investigate the relationship between specific autoantibodies and the clinical and laboratory features of SSc in 46 Moroccan patients. The majority of patients were female, middle-aged, and of Caucasian ethnicity, with a mean age of 50 years, and duration of disease between 1 and 10 years. The most common symptoms were Raynaud’s phenomenon (89.2% of patients), sclerodactyly (84.8%), proximal scleroderma (67.4%), gastrointestinal involvement (50%), arthralgia (41.3%), interstitial lung disease (30.4%), myalgia (24%) and pulmonary arterial hypertension (10.8%). We found a correlation between anti-RNAP III and Sclerodactyly and pulmonary arterial hypertension, while anti-topo I autoantibodies were associated with interstitial lung disease. The study also noted that environmental factors, ethnicity, and genetic interactions may influence the clinical and autoantibody profile in SSc patients.