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. 1992 Nov;71(6):327-36.
doi: 10.1097/00005792-199211000-00001.

The clinical and immunogenetic features of patients with autoantibodies to the nucleolar antigen PM-Scl

C Marguerie  1 , C C Bunn, J Copier, R M Bernstein, J M Gilroy, C M Black, A K So, M J Walport
Affiliations

The clinical and immunogenetic features of patients with autoantibodies to the nucleolar antigen PM-Scl

C Marguerie et al. Medicine (Baltimore). 1992 Nov.

Abstract

The clinical and laboratory features of 32 patients with anti-PM-Scl were studied. Patients with this rare autoantibody suffered from a homogenous overlap connective tissue disease defined by Raynaud phenomenon (32/32), features of scleroderma (31/32), arthritis (31/32, erosive in 9/32), myositis (28/32), lung restriction (25/32), calcinosis (15/32), and sicca (11/32). Significant renal and neurologic involvement was uncommon. All patients examined (22/22) had HLA-DR3, and 50% of these patients were homozygous. Our patients responded favorably to moderate immunosuppression and, with therapy, the disease generally has a good prognosis; over 50% of our series (17/32) remained well on minimal or no immunosuppression after a median follow-up of 8 years.

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