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 (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.
Marguerie
C
C
Rheumatology Unit, Royal Postgraduate Medical School, Hammersmith Hospital, London, U.K.
Bunn
C C
CC
Copier
J
J
Bernstein
R M
RM
Gilroy
J M
JM
Black
C M
CM
So
A K
AK
Walport
M J
MJ
D000293:Adolescent; D000328:Adult; D000368:Aged; D001168:Arthritis / Q000276:immunology; D001323:Autoantibodies / Q000276:immunology*; D001324:Autoantigens / Q000276:immunology*; D002648:Child; D003240:Connective Tissue Diseases / Q000235:genetics / Q000276:immunology*; D003882:Dermatomyositis / Q000276:immunology; D005095:Exoribonucleases; D063326:Exosome Multienzyme Ribonuclease Complex; D005260:Female; D015803:HLA-DR3 Antigen / Q000235:genetics; D006801:Humans; D007674:Kidney Diseases / Q000276:immunology; D008171:Lung Diseases / Q000276:immunology; D008180:Lupus Erythematosus, Systemic / Q000276:immunology; D008297:Male; D008875:Middle Aged; D017285:Polymyositis / Q000276:immunology; D011928:Raynaud Disease / Q000276:immunology; D012595:Scleroderma, Systemic / Q000276:immunology
D001323:Autoantibodies; D001324:Autoantigens; D015803:HLA-DR3 Antigen; D005095:Exoribonucleases; D063326:Exosome Multienzyme Ribonuclease Complex; C077044:EXOSC10 protein, human