The objective of this study was to determine the lipoprotein profile of limited cutaneous systemic sclerosis (LcSSc). Fasting lipids were determined in 24 female patients and 24 healthy age-matched and sex-matched controls. Exclusion criteria were conditions that induce an altered lipid profile. Lipoprotein levels of risk were determined in accordance with the National Cholesterol Education Program (NCEP). Significantly lower levels of high-density lipoprotein (HDL) cholesterol (47.6+/-12.4 mg dL(-1) vs. 58.2+/-12.3 mg dL(-1); P=0.003) and total cholesterol (197.0+/-40.7 mg dL(-1) vs. 222.0+/-34.0 mg dL(-1); P=0.02) were observed in LcSSc patients than in controls. The presence of anti-centromere antibodies (ACA) was also associated with lower HDL levels (45.0+/-12.1 mg dL(-1)) compared to ACA-negative patients and controls (50.2+/-12.6 and 58.2+/-12.3 mg dL(-1), respectively, P=0.01). The only clinical variable associated with low HDL levels was pulmonary hypertension (PH) (33.6+/-2.3 mg dL(-1) vs. 49.6+/-11.9 mg dL(-1), P=0.01). No significant correlation was observed among HDL levels and ESR (r=-0.313; P=0.14), CRP (r=-0.296; P=0.16), or BMI (r=-0.263; P=0.21). Remarkably, a higher percentage of risk HDL levels was identified in LcSSc patients (41.6%) than in healthy controls (8.3%) (P=0.02). Our data suggest that LcSSc patients, particularly those who are ACA positive, have an adverse lipid profile characterized by low HDL levels, a known independent risk for CAD in women. The relevance of this finding for the development of atherosclerosis in this disease must be confirmed by epidemiological studies.