Systemic sclerosis. Clinical and epidemilogycal assessment of the patients from two reference centers in Cuba

Introduction: Systemic Sclerosis is a serious and potentially mortal disease, causing marked fibrosis of the skin and multiple vital organs. Systemic Sclerosis evolves with different grades of severity and increased mortality. Objectives: to determine sociodemographic features, clinical-epidemiological characteristics, therapeutic handling and complications of Systemic Sclerosis cuban subjects from two reference centers. Method: clinical variables, affected organs and systems, laboratories tests and immunology were picked up. Endoscopy GD, heart ecography, cathetherism, computerized axial tomography of high resolution, pulmonary function testing, and others. It was assessed the therapies, complications and outcome of the serie. Results: Diffuse form of the illness was present in 20 patients (58.8 %), 11 (32.3 %) had limited forms, 2 CREST syndromes (5.88 %) and a one patient with overlap syndrome. The time of disease duration average was 7.4anos DS/5.3. Musculoskeletal participation; 16 (47 %) and, joint retractions, digital ulcers and resorption of phalanges 4 (12.5 %). Gastrointestinal (GI) participation was seen in 56.2 %. Esophageal involvement was seen in 16 / 19 patients (85 %), which was severe in 5 (31.2 %). Respiratory insufficiency; 9 cases (26.4 %), heart was affected in 8 patients (23 %), renal dysfunction occurred in 6 (17.6 %), among these, 2 patients developed renal crisis, 68 % of the patients had Raynaud phenomenon. Treatment with anti fibrotic drugs like D'Pencilamina 19 (55.8 %), pro-kinetic medication 16 (47.4 %) and gastroprotectors 8 (23.5 %). Corticosteroids 9 (26.4 %), angiotensin - converting enzyme inhibitors (ACE) 7 (20.5 %), sildelnafil 3 (8.82 %) and others. Conclusions: Affected vital organs with large GI and repiratory involvement was observed. In general, there was severe evolution of SSc subjects. It is similar to published studies.