title: Anti-topoisomerase II alpha autoantibodies in systemic sclerosis-association with pulmonary hypertension and HLA-B35.
abstract: We have previously detected autoantibodies against topoisomerase II alpha (anti-topo II alpha) in sera from patients with idiopathic pulmonary fibrosis. To determine whether anti-topo II alpha is also present in systemic sclerosis (SSc) patients with pulmonary involvement, we screened sera from 92 patients and 34 healthy controls. Presence of anti-topo II alpha was investigated with respect to clinical and serological features, including the frequencies of HLA class I and II alleles. Anti-topo II alpha was detected in 20/92 (21.7%) patients. No association was found with either anti-topoisomerase I (Scl-70 or anti-topo I) or anti-centromere antibodies. However, anti-topo II alpha was associated with the presence of pulmonary hypertension (PHT) (as opposed to pulmonary fibrosis), and with a decrease of carbon monoxide diffusing capacity. Anti-topo II alpha was strongly associated with the presence of the class I antigen HLA-B35. No significant association was found with HLA class II antigens. HLA-B35 also turned out to be associated with the presence of PHT. These results indicate that in SSc patients, the presence of anti-topo II alpha is associated with PHT, and that the simultaneous presence of HLA-B35 seems to add to the risk of developing PHT.
authors.affiliation: Immunology and Genetics Laboratory, Codivilla Putti Research Institute, IRCCS Istituti Ortopedici Rizzoli, Italy.
authors.forename: B
authors.initials: B
authors.lastname: Grigolo
authors.forename: I
authors.initials: I
authors.lastname: Mazzetti
authors.forename: R
authors.initials: R
authors.lastname: Meliconi
authors.forename: S
authors.initials: S
authors.lastname: Bazzi
authors.forename: R
authors.initials: R
authors.lastname: Scorza
authors.forename: M
authors.initials: M
authors.lastname: Candela
authors.forename: A
authors.initials: A
authors.lastname: Gabrielli
authors.forename: A
authors.initials: A
authors.lastname: Facchini
mesh_terms: D000328:Adult; D000483:Alleles; D001323:Autoantibodies / Q000097:blood*; D016022:Case-Control Studies; D004250:DNA Topoisomerases, Type II / Q000276:immunology*; D005260:Female; D015797:HLA-B35 Antigen / Q000235:genetics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000235:genetics / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000150:complications* / Q000235:genetics / Q000276:immunology*
chemical_list: D001323:Autoantibodies; D015797:HLA-B35 Antigen; D004250:DNA Topoisomerases, Type II