Post-Menopause is the Main Risk Factor for Developing Isolated Pulmonary Hypertension in Systemic Sclerosis

  1. RAFFAELLA SCORZA1,*,
  2. MONICA CARONNI1,
  3. SONIA BAZZI1,
  4. FILIPPO NADOR2,
  5. LORENZO BERETTA1,
  6. RITA ANTONIOLI1,
  7. LAURA ORIGGI1,
  8. ALESSANDRA PONTI1,
  9. MAURIZIO MARCHINI1,
  10. MASSIMO VANOLI1

Article first published online: 24 JAN 2006

DOI: 10.1111/j.1749-6632.2002.tb04221.x

Additional Information

How to Cite

SCORZA, R., CARONNI, M., BAZZI, S., NADOR, F., BERETTA, L., ANTONIOLI, R., ORIGGI, L., PONTI, A., MARCHINI, M. and VANOLI, M. (2002), Post-Menopause is the Main Risk Factor for Developing Isolated Pulmonary Hypertension in Systemic Sclerosis. Annals of the New York Academy of Sciences, 966: 238–246. doi: 10.1111/j.1749-6632.2002.tb04221.x

Author Information

  1. 1

    Clinical Immunology and Allergology Unit, IRCCS Ospedale Maggiore and University of Milan, Milan, Italy

  2. 2

    Cardiology Unit, IRCCS Ospedale Maggiore di Milano, Milan, Italy

*Address for correspondence: Prof. Raffaella Scorza, Servizio di Imunologia Clinica ed Allergologia e Centro di Riferimento per le Malattie Autoimmuni Sistemiche, Via F. Sforza 35 - 20122 Milano, Italy. Voice/fax: +39 2 5503 3568; raffaella.scorza@unimi.it.

Publication History

  1. Issue published online: 24 JAN 2006
  2. Article first published online: 24 JAN 2006

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Keywords:

  • scleroderma;
  • systemic sclerosis, etiology;
  • pulmonary hypertension;
  • menopause;
  • hormonal replacement therapy;
  • estrogens

Abstract: In scleroderma patients, isolated pulmonary hypertension (PHT) has been associated with selected HLA haplotypes, severe impairment of the diffusing capacity for carbon monoxide and the diagnosis of CREST. Most patients with CREST have a late-age onset of the disease, corresponding to the perimenopausal or postmenopausal period. We conducted a retrospective cohort study to determine the role of post-menopause and of the other known clinical and biological markers in the development of isolated pulmonary hypertension in Italian patients with systemic sclerosis. 189 female patients with scleroderma who had no ecographic signs of pulmonary hypertension (PHT) and radiographic signs of lung fibrosis at the first visit and did not develop significant pulmonary fibrosis during the observation time were included. Sixty-three out of 189 patients (33.3%) presented isolated pulmonary hypertension. A severe impairment of diffusing capacity for carbon monoxide at admission was found to be an early predictive element for its development. An increased risk was associated with postmenopausal condition (RR= 5.2, p= 0.000), CREST syndrome (RR= 2.8, p= 0.001) and haplotype HLA-B35 (RR= 2.8; p= 0.002). A significant positive interaction between postmenopausal condition and either HLA-B35 (RR= 15.2; p= 0.000) or the diagnosis of CREST (RR= 14.1; p= 0.000) was found. Postmenopausal condition alone or in combination with HLA-B35 and CREST syndrome is the main risk-factor for developing primary pulmonary hypertension in scleroderma patients. This suggests that hormonal replacement therapy could play a role in preventing isolated PHT in patients with systemic sclerosis.

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