ANTIENDOTHELIAL CELL ANTIBODIES IN SCLERODERMA CORRELATE WITH SEVERE DIGITAL ISCHEMIA AND PULMONARY ARTERIAL-HYPERTENSION

Objective. To determine the prevalence of IgG antiendothelial cell ant ibodies (AECA) in patients with scleroderma (systemic sclerosis, SSc) and to correlate it with clinical spectrum and autoantibody profile. M ethods. Seventy-six patients with SSc and 50 matched healthy controls were studied. Immunological variables were antinuclear antibody (ANA), rheumatoid factor (RF), and Scl-70. IgG-AECA was measured by cellular ELISA. Results. The prevalence of IgG-AECA was 27.6% in patients with SSc compared to 6% in controls (p < 0.01). Forty percent of patients with diffuse disease had this antibody, versus 13.5% of those with lim ited cutaneous involvement (p < 0.05). Patients with AECA had signific antly higher incidence of digital infarcts and gangrene (p < 0.01) and pulmonary arterial hypertension (p < 0.001) than those without. In th e AECA positive group, mean IgG-AECA levels (measured by absorbance va lues) were significantly higher in patients with digital infarcts (0.9 1 +/- 0.31 vs 0.60 +/- 0.05; p < 0.01) and pulmonary arterial hyperten sion (1.14 +/- 0.37 vs 0.68 +/- 0.13; p < 0.001) compared to those wit hout these features. Conclusion. IgG-AECA appears to be an important m arker for disease severity in scleroderma.