title: The clinical and immunogenetic features of patients with autoantibodies to the nucleolar antigen PM-Scl.
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.
authors.affiliation: Rheumatology Unit, Royal Postgraduate Medical School, Hammersmith Hospital, London, U.K.
authors.forename: C
authors.initials: C
authors.lastname: Marguerie
authors.forename: C C
authors.initials: CC
authors.lastname: Bunn
authors.forename: J
authors.initials: J
authors.lastname: Copier
authors.forename: R M
authors.initials: RM
authors.lastname: Bernstein
authors.forename: J M
authors.initials: JM
authors.lastname: Gilroy
authors.forename: C M
authors.initials: CM
authors.lastname: Black
authors.forename: A K
authors.initials: AK
authors.lastname: So
authors.forename: M J
authors.initials: MJ
authors.lastname: Walport
mesh_terms: 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
chemical_list: D001323:Autoantibodies; D001324:Autoantigens; D015803:HLA-DR3 Antigen; D005095:Exoribonucleases; D063326:Exosome Multienzyme Ribonuclease Complex; C077044:EXOSC10 protein, human