ANTIENDOTHELIAL CELL ANTIBODIES - USEFUL MARKERS OF SYSTEMIC-SCLEROSIS

BACKGROUND: Systemic sclerosis (SS) encompasses a wide spectrum of cli nical presentations. Antiendothelial cell antibodies (AECA) in patient s with primary Raynaud's phenomenon (PRP), limited SS (ISSc), or diffu se SS (dSSc) may help to determine the long-term prognosis of the dise ase. METHODS: Twenty-seven normal controls, 13 patients with PRP, 36 w ith ISSc, and 31 with dSSc were included in the study. Sera were exami ned for the presence of AECA, using a cellular enzyme-linked immunosor bent assay (ELISA). Angiotensin-converting enzyme (ACE) activity, plas ma von Willebrand factor antigen (vWfAg), and thrombomodulin (Tm) conc entrations were also evaluated. The medical records of 50 of the ISSc and dSSc patients were reviewed and the organ system involvement noted . RESULTS: Antiendothelial cell antibodies were present in 3 patients with PRP, 16 patients with ISSc, and 26 patients with dSSc. These auto antibodies were mainly of the IgG isotype. There was no difference in ACE activity between patients and controls. In contrast, vWfAg and Tm concentrations were higher in patients with PRP relative to controls, and higher in patients with ISSc compared with those with PRE. The pre sence of AECA was associated with digital scars and ulcers (P < 0.004 and P < 0.003, respectively), severe RP (P < 0.01), grade 3 tortuosity of vessels (P < 0.0004), and lung involvement (P < 0.02). CONCLUSION: The significant trend for AECA to increase with disease severity acro ss the three groups of patients studied suggests that the AECA test ca n identify subsets of SSc with differing prognoses. (C) 1997 by Excerp ta Medica, Inc.