Anti-topoisomerase II α autoantibodies in systemic sclerosis - Association with pulmonary hypertension and HLA-B35

B. Grigolo, I. Mazzetti, R. Meliconi, S. Bazzi, R. Scorza, M. Candela, A. Gabrielli, A. Facchini

Research output: Contribution to journalArticlepeer-review


We have previously detected autoantibodies against topoisomerase II α (anti-topo II α) in sera from patients with idiopathic pulmonary fibrosis. To determine whether anti-topo II α 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 α was investigated with respect to clinical and serological features, including the frequencies of HLA class I and II alleles. Anti-topo II α 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 α 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 α 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 α is associated with PHT, and that the simultaneous presence of HLA-B35 seems to add to the risk of developing PHT.

Original languageEnglish
Pages (from-to)539-543
Number of pages5
JournalClinical and Experimental Immunology
Issue number3
Publication statusPublished - 2000


  • Anti-topoisomerase II α
  • Pulmonary hypertension
  • Systemic sclerosis

ASJC Scopus subject areas

  • Immunology


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