Clinical presentation in patients with systemic sclerosis

Abstract

Introduction: systemic sclerosis is an autoimmune disease characterized by endothelial damage, and skin, vessel and internal organ fibrosis and inflammation. There are differences in terms of frequency, severity and prognosis for the different ethnic groups, what reinforces the importance of the study in each geographical region with the purpose of enabling early diagnosis of its incipient symptoms.
Methods: we conducted a descriptive and retrospective study form March 2006 through March 2008, including patients with a final diagnosis of systemic sclerosis, who are treated at the Systemic Autoimmune Diseases Unit at the Clínicas Hospital.
Results: 31 women were included in the study, average follow-up of patients was 39.2 months, and average age at the time of diagnosis was 47.6 years. Eleven patients (35,5%) presented diffuse disease and 20 (64.5%) of them evidenced limited disease. Thirty patients presented Raynaud’s phenomenon. In 92% of cases capilaroscopy showed a sclerodermiform pattern. In terms of the respiratory system, we found interstilial pathology in 25% of cases, pulmonary arterial hypertension in 22.2 % and a restrictive pattern in respiratory function studies in 35.5%. Also, 67.7% presented digestive manifestations and 9.6% developed sclerodermic renal crisis. We found anti-nuclear antibodies (ANA) in 29 out of 31 patients (93,5%) patients; 16 presented anticentromere antibodies and five anti-topoisomerasa-I antibodies. The four patients (12.9%) who died during follow-up presented common elements such as diffuse sclerosis, digital ulcers and severe respiratory compromise.
Conclusions: the clinical and immune characteristics found in our study were similar to those described in other series. Should there be no specific treatment, it is essential to perform regular assessment of visceral impact in order to control and delay complications which result in high morbimortality rates.