Clinical and sociodemographic features of Puerto Ricans with systemic sclerosis.

INTRODUCTION: Systemic sclerosis is an autoimmune disease of unknown etiology characterized by fibrotic changes in the skin, blood vessels, and various internal organs. The disease has a wide spectrum of presentation and a variable clinical course that includes limited skin involvement to life-threatening disease. Clinical manifestations and disease severity differs among ethnic groups. The objective of this study is to describe the clinical and sociodemographic features of patients with well-characterized systemic sclerosis from Puerto Rico.
METHODS: A structured questionnaire was completed for each patient to gather information about demographic factors, clinical manifestations, laboratory findings, diagnostic studies, and pharmacologic treatments.
RESULTS: Of the 24 patients with systemic sclerosis, 96% were females, 83% had Raynaud's phenomenon, 67% had gastrointestinal involvement, 63% had skin hypopigmentation, 50% had digital pitting scars, 46% had arterial hypertension, 11% had pulmonary hypertension, and 4.8% had renal involvement. The overall median modified Rodnan skin score was 24.5 (inter-quartile range 16.0-31.3). Pulmonary function tests resulted in abnormal in 60% of 14 patients, of which 57% had restrictive lung disease (FVC < 70%) and 42.9% had decreased diffusion capacity. Serologically, 66.7% were positive for antinuclear antibody and 62.5% were positive for anti-centromere.
CONCLUSIONS: In this study, the predominant clinical features of Puerto Ricans with systemic sclerosis were gastrointestinal involvement, Raynaud's phenomenon, digital pitting scars, and lung disease. Patients had a moderate severity of skin disease. The presence of renal involvement and pulmonary hypertension were low in our group. No significant differences were found between systemic sclerosis disease subsets.