Antiendothelial Cells Antibodies in Patients with Systemic Sclerosis in Relation to Pulmonary Hypertension and Lung Fibrosis

  • K. Lewandowska
  • M. Ciurzynski
  • E. Gorska
  • P. Bienias
  • K. Irzyk
  • M. Siwicka
  • K. Zycinska
  • P. Pruszczyk
  • U. Demkow
Conference paper
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 756)

Abstract

Although scleroderma is generally considered a fibrosing disease, it is now recognized that the underlying vascular pathology is playing a fundamental role in its pathogenesis. The present study was aimed at testing the prevalence of anti-endothelial cell antibodies (AECA) in systemic scleroderma (SSc) patients with and without pulmonary hypertension (PH) and in relation to the presence of pulmonary fibrosis. Fifty four SSc patients (50 females and 4 male, mean age 55.7 ± 16.3 years) were prospectively screened. All patients underwent transthoracic echocardiography with the estimation of pulmonary artery pressure (PAP) and tricuspid regurgitant peak gradient (TRPG). All patients suspected to have pulmonary hypertension were referred for right heart catheterization. Restrictive lung disease was confirmed by HRCT. A healthy control group included (n = 27; 7 men and 20 women, mean age 49.8 ± 12.1 years). The study of AECA was performed using the indirect immunofluorescence method on commercially available human umbilical vein endothelial cells. The HRCT scans in patients with suspected interstitial lung disease revealed signs of lung fibrosis in 15 (out of the 36 examined patients). TRPG at rest of 31 mmHg was demonstrated in 14 (21%) patients. During cardiac catheterization, arterial PH was found in two patients. Resting venous PH was found in one patient and an excessive post capillary PAP elevation at rest was demonstrated in 11 patients. At the baseline, 14/54 patients (26%) were positive for AECA. In the control group, the frequency of the antibodies was 3/27 (11%). No statistical correlation between antibody titter and the presentation of the disease existed. AECA were highly prevalent in a subgroup of patients suffering from interstitial pulmonary fibrosis. Out of the 15 patients suffering from lung fibrosis, 7 were AECA positive. The presence of AECA correlated very well with antinuclear antibodies (ANA), but was not related to the profile of ANA. Our findings support evidence that endothelial cell damage is involved in SSc, as there was increased prevalence of circulating AECA of the IgG isotype in SSc patients. AECA may also be related to the complications of SSc, like pulmonary fibrosis.

Keywords

Anti-endothelial cell antibodies Interstitial lung disease Pulmonary fibrosis Pulmonary hypertension Systemic scleroderma 

Notes

References

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Copyright information

© Springer Science+Business Media Dordrecht 2013

Authors and Affiliations

  • K. Lewandowska
    • 1
  • M. Ciurzynski
    • 2
  • E. Gorska
    • 1
  • P. Bienias
    • 2
  • K. Irzyk
    • 2
  • M. Siwicka
    • 3
  • K. Zycinska
    • 4
  • P. Pruszczyk
    • 2
  • U. Demkow
    • 1
  1. 1.Department of Laboratory Diagnostics and Clinical Immunology of Developmental AgeMedical University of WarsawWarsawPoland
  2. 2.Department of Internal Medicine and CardiologyMedical University of WarsawWarsawPoland
  3. 3.Department of DermatologyMedical University of WarsawWarsawPoland
  4. 4.Department of Familial MedicineMedical University of WarsawWarsawPoland

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