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Isolated diffusing capacity reduction in systemic sclerosis.

V D Steen1, G Graham, C Conte

  • 1Department of Medicine, University of Pittsburgh School of Medicine, Pennsylvania.

Arthritis and Rheumatism
|July 11, 1992
PubMed
Summary
This summary is machine-generated.

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Systemic sclerosis patients with reduced diffusing capacity for carbon monoxide (DLCO) generally have good outcomes. However, a small subset with very low DLCO may develop pulmonary hypertension, indicating a need for careful monitoring.

Area of Science:

  • Pulmonary Medicine
  • Rheumatology
  • Cardiology

Background:

  • Systemic sclerosis (SSc) is a complex autoimmune disease affecting connective tissues.
  • Pulmonary involvement is a major cause of morbidity and mortality in SSc.
  • Isolated reduction in diffusing capacity for carbon monoxide (DLCO) is a recognized pulmonary function test abnormality in SSc.

Purpose of the Study:

  • To investigate the long-term prognosis of patients with SSc presenting with an isolated reduction in DLCO.
  • To identify predictors of adverse cardiopulmonary outcomes in this patient group.

Main Methods:

  • Retrospective analysis of 815 SSc patients with initial pulmonary function tests (PFTs).
  • Identification of patients with isolated DLCO reduction (normal forced vital capacity [FVC] and FEV1/FVC ratio).

Related Experiment Videos

  • Longitudinal follow-up including repeat PFTs, assessment of cardiopulmonary events, and survival.

    • Main Results:

    • 19% of SSc patients had isolated DLCO reduction; 11% developed pulmonary hypertension with reduced survival.
    • An initial DLCO <55% predicted and FVC/DLCO ratio >1.4 predicted pulmonary hypertension.
    • Among patients with isolated DLCO reduction, 48% had follow-up PFTs, with only 8% developing serious pulmonary disease.
    • Half of patients showed significant DLCO improvement at follow-up, unexplained by initial factors.

    Conclusions:

    • Isolated DLCO reduction is common in SSc and generally associated with a good prognosis.
    • A subset of patients with very low DLCO (<55% predicted) are at risk for developing isolated pulmonary hypertension.
    • The FVC/DLCO ratio may help identify SSc patients requiring closer monitoring for pulmonary hypertension.