title: [Pulmonary manifestation of progressive systemic scleroderma: prognostic value of centromere antibodies and antibodies to Scl-70 nucleoprotein].
abstract: We examined 74 patients with systemic sclerosis. 21 of them (= 28%) had Scl-70 antibodies in their blood serum and 12 (= 16%) were anticentromere antibody positive, whereas in 41 cases (= 56%) none of these antibodies could be detected. In patients with Scl-70 antibodies lung function tests showed a decrease in vital capacity (p less than 0.05), total capacity (p less than 0.01), and diffusing capacity (p greater than 0.05/n.s.) as compared to ACA-positive patients. A limitation of pulmonary function was seen in 76% of Scl-70-positive patients, in 44% of antibody-negative patients, and in only 33% in the ACA-positive group. A disabling respiratory limitation was found in 14% of Scl-70-positive patients, and in 7% of antibody-negative patients, whereas none of the ACA-positive patients had a higher degree of respiratory insufficiency. We conclude that pulmonary involvement in systemic sclerosis is most frequent and severe in patients with Scl-70 antibodies; it is relatively rare and of minor severity in ACA-positive patients. Antibody-negative patients are in between these extremes.
authors.affiliation: Pneumologische Abteilung, Medizinischen Klinik, Klinikum Grosshadern.
authors.forename: J
authors.initials: J
authors.lastname: Behr
authors.forename: G
authors.initials: G
authors.lastname: König
authors.forename: M
authors.initials: M
authors.lastname: Meurer
authors.forename: T
authors.initials: T
authors.lastname: Krieg
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000032:analysis*; D002503:Centromere / Q000276:immunology*; D002875:Chromosomes / Q000276:immunology*; D004264:DNA Topoisomerases, Type I; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009687:Nuclear Proteins / Q000276:immunology*; D011379:Prognosis; D011658:Pulmonary Fibrosis / Q000276:immunology*; D012595:Scleroderma, Systemic / Q000276:immunology*
chemical_list: D001323:Autoantibodies; D009687:Nuclear Proteins; C048100:Scl 70 antigen, human; D004264:DNA Topoisomerases, Type I
vernacular_title: Pulmonale Manifestation bei progressiver, systemischer Sklerodermie: Prognostische Wertigkeit der Zentromerantikörper und Antikörper gegen Scl-70 Nukleoprotein.