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Isolated DLco/VA reduction in systemic sclerosis patients: a new patient subset?

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Abstract

Introduction

Diffusing capacity for carbon monoxide (DLco) reduction is the first detectable pulmonary functional test (PFT) change in systemic sclerosis (SSc)–related pulmonary complications. reduction in patients without cardiopulmonary alterations has also been observed; a good characterisation of these patients is lacking. The objective of this study is to describe the characteristics of SSc patients with isolated DLco reduction and compare these patients to SSc patients with DLco reduction with a known cause.

Methods

SSc patients with DLco < 80% predicted were included and classified into cases (isolated DLco reduction) and controls (DLco reduction in the presence of known pulmonary pathology). SSc clinico-serological data, PFT and echocardiography features were collected and analysed.

Results

From a total SSc cohort of 115 patients, 75 patients were included: 20 cases (26.7%) and 55 controls (73.3%). Cases were predominantly limited skin subset (90% vs 60%, p < 0.001), were anti-centromere antibody (ACA)-positive (95% vs 40%, p < 0.001) and had an infrequent oesophageal involvement (45% vs 74%; p = 0.016). The mean DLco reduction of cases was mild (65.60% ± 10.56). Only 1 out of 20 patients had normal DLco/VA values, and tricuspid regurgitation was more frequent (85% vs 53.8%, p = 0.014).

Conclusion

There is a subgroup of SSc patients with mild isolated DLco and DLco/VA reduction, predominantly limited SSc with ACA seropositivity, which could identify a particular SSc subset. We hypothesise that isolated DLco/VA reduction could indicate a pulmonary vascular involvement. Nevertheless, a close follow-up is mandatory, as a pre-PAH situation cannot be excluded.

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Author information

Authors and Affiliations

  1. Servicio Reumatología, Consultas Externas, Hospital del Mar, Passeig Marítim 25-29, 08003, Barcelona, Spain

    Patricia Corzo & Anna Pros

  2. Rheumatology Section, Department of Internal Medicine, Hospital Plató, Barcelona, Spain

    Patricia Corzo

  3. Department of Rheumatology, Hospital Universitary Sagrat Cor, Barcelona, Spain

    Patricia Corzo

  4. Department of Respiratory Medicine, Parc de Salut Mar-IMIM, Hospital del Mar, Barcelona, Spain

    Juana Martinez-Llorens & Eva Balcells

  5. Universitat Pompeu Fabra (UPF), Barcelona, Spain

    Juana Martinez-Llorens & Eva Balcells

  6. CIBER Enfermedades Respiratorias (CIBERES), Bunyola, Illes Balears, Spain

    Juana Martinez-Llorens & Eva Balcells

  7. IMIM (Hospital del Mar Medical Research Institute), Barcelona, Spain

    Juana Martinez-Llorens, Luis Molina & Eva Balcells

  8. Department of Cardiology, Parc de Salut Mar-IMIM, Hospital del Mar, Barcelona, Spain

    Luis Molina

  9. Centre for Musculoskeletal Research, The University of Manchester, Manchester, UK

    Stephanie Fenxi Ling

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  1. Patricia Corzo
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Corresponding author

Correspondence to Patricia Corzo.

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The study does not contain recognisable patient data. For this type of study format, consent is not required.

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Key Messages

• There is a SSc patient subgroup with isolated DLco and DLco/VA reduction, predominantly lSSc with ACA-positive.

• This DLco/VA reduction could indicate the presence of a pulmonary microvascular involvement

• The prognosis of this patients has not beet yet established; therefore, a close pulmonary follow-up is mandatory

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Corzo, P., Pros, A., Martinez-Llorens, J. et al. Isolated DLco/VA reduction in systemic sclerosis patients: a new patient subset?. Clin Rheumatol 37, 3365–3371 (2018). https://doi.org/10.1007/s10067-018-4342-5

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  • DOI: https://doi.org/10.1007/s10067-018-4342-5

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