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Interstitial lung disease increases mortality in systemic sclerosis patients with pulmonary arterial hypertension without affecting hemodynamics and exercise capacity

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Abstract

Published data suggest that coexisting interstitial lung disease (ILD) has an impact on mortality in patients with systemic sclerosis (SSc) and pulmonary arterial hypertension (PAH), but there is scarce knowledge if this is reflected by hemodynamics, exercise capacity, autoantibody profile, or pulmonary function. In this partially retrospective study, 27 SSc-PAH patients were compared to 24 SSc-PAH patients with coexisting ILD respecting to survival, pulmonary function, hemodynamics, exercise capacity, and laboratory parameters. Survival was significantly worse in SSc-PAH-ILD patients than in SSc patients with isolated PAH (1, 5, and 10-year survival rates 86, 54, and 54% versus 96, 92, and 82%, p = 0.013). Compared to isolated SSc-PAH patients, patients with SSc-PAH-ILD revealed lower forced expiratory volume after 1 s (FEV1) values at the time of PAH diagnosis as well as 1 and 2 years later (p = 0.002) without significant decrease in the PAH course in both groups. At PAH diagnosis, diffusion capacity for carbon monoxide (DLCO) values were lower in the ILD-PAH group. Coexisting ILD was not associated with lower exercise capacity, different FEV1/forced vital capacity (FVC) ratio, higher WHO functional class, or reduced hemodynamics. Higher levels of antibodies against angiotensin and endothelin receptors predict mortality in all SSc-PAH patients but could not differentiate between PAH patients with and without ILD. Our study confirmed an impact of ILD on mortality in SSc-PAH patients. Pulmonary function parameters can be used to distinguish PAH from PAH-ILD. The higher mortality rate cannot be explained by differences in hemodynamics, exercise capacity, or autoantibody levels. Mechanisms of mortality remain to be studied.

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Acknowledgments

Prof. Riemekasten has recieved financial support from the Mirjam Lichy Foundation. The study was supported by the German Network of Systemic Sclerosis (DNSS), by the DFG grant RI 1056-11/1-2, and by Actelion Deutschland Pharmaceuticals GmbH (INDIZ project).

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Authors and Affiliations

  1. Department of Anesthesiology, University Hospital Bonn, Bonn, Germany

    M. Michelfelder

  2. Department of Rheumatology, Charité University Hospital Berlin, Berlin, Germany

    M. Michelfelder, M. Becker, E. Siegert & G. Riemekasten

  3. Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland

    M. Becker

  4. Department of Neurology, Asklepios Fachklinikum Teupitz, Teupitz, Germany

    A. Riedlinger

  5. Department of Pulmonology, University Hospital Lübeck, Lübeck, Germany

    D. Drömann

  6. Priority Area Asthma and Allergy, Research Center Borstel, Borstel, Germany

    X. Yu, F. Petersen & G. Riemekasten

  7. Department of Rheumatology, University Hospital Lübeck, Lübeck, Germany

    G. Riemekasten

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  1. M. Michelfelder
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Correspondence to G. Riemekasten.

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Conflict of interest

Prof. Riemekasten has recieved financial support from CellTrend, Actelion, and GSK and is an advisor of CellTrend, Bayer, Novartis, GSK, Actelion, Pfizerveranstaltung, and BMS. There is no conflict of interest to declare with regard to the work for Marissa Michelfelder, Mike Becker, Arne Riedlinger, Elise Siegert, Daniel Drömann, Frank Petersen, and Xinhua Yu.

Ethical standards

The study was approved by the local ethics committee and has been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments and included written consent of each patient.

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Michelfelder, M., Becker, M., Riedlinger, A. et al. Interstitial lung disease increases mortality in systemic sclerosis patients with pulmonary arterial hypertension without affecting hemodynamics and exercise capacity. Clin Rheumatol 36, 381–390 (2017). https://doi.org/10.1007/s10067-016-3504-6

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  • DOI: https://doi.org/10.1007/s10067-016-3504-6

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