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

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 (2...

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Bibliographic Details
Published in:Medicine (Baltimore) 1992-11, Vol.71 (6), p.327-336
Main Authors: Marguerie, C, Bunn, C C, Copier, J, Bernstein, R M, Gilroy, J M, Black, C M, So, A K, Walport, M J
Format: Article
Language:English
Subjects:
Adolescent
Adult
Aged
Arthritis - immunology
Autoantibodies - immunology
Autoantigens - immunology
Child
Connective Tissue Diseases - genetics
Connective Tissue Diseases - immunology
Dermatomyositis - immunology
Exoribonucleases
Exosome Multienzyme Ribonuclease Complex
Female
HLA-DR3 Antigen - genetics
Humans
Kidney Diseases - immunology
Lung Diseases - immunology
Lupus Erythematosus, Systemic - immunology
Male
Middle Aged
Polymyositis - immunology
Raynaud Disease - immunology
Scleroderma, Systemic - immunology
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Summary: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.
ISSN:0025-7974
DOI:10.1097/00005792-199211000-00001