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Improved pulmonary function in systemic sclerosis after treatment with cyclophosphamide

A Akesson1, A Scheja, A Lundin

  • 1Department of Rheumatology, Lund University Hospital, Sweden.

Arthritis and Rheumatism
|May 1, 1994
PubMed
Summary

Cyclophosphamide treatment improved lung function and reduced inflammation in patients with systemic sclerosis (SSc)-related pulmonary fibrosis. Further trials are recommended for patients with elevated acute-phase proteins.

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Area of Science:

  • Rheumatology
  • Pulmonology
  • Immunology

Background:

  • Pulmonary fibrosis is a significant complication of systemic sclerosis (SSc), contributing to patient morbidity and mortality.
  • Alveolitis is hypothesized to be a critical factor in the development of pulmonary fibrosis in SSc.
  • Investigating immunosuppressive therapies is crucial for managing SSc-associated lung disease.

Purpose of the Study:

  • To evaluate the efficacy of immunosuppressive therapy in improving pulmonary function in patients with SSc.
  • To assess the impact of cyclophosphamide and corticosteroids on lung function parameters in SSc patients with pulmonary involvement.

Main Methods:

  • Eighteen patients with progressive pulmonary dysfunction due to SSc received a 1-year treatment course of cyclophosphamide and corticosteroids.

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  • Patients were assessed for changes in vital capacity (VC) and static lung compliance (Cst).
  • Subgroup analysis was performed based on pre-therapy levels of acute-phase proteins and erythrocyte sedimentation rate (ESR).

    • Main Results:

    • Vital capacity (VC) improved in 14 out of 18 patients, with a median increase from 74% to 80% of predicted.
    • Static lung compliance (Cst) improved in 8 out of 12 patients, with a median increase from 59% to 66% of predicted.
    • Patients with elevated acute-phase proteins and ESR showed significant improvements in VC and Cst, and resolution of pulmonary opacities.

    Conclusions:

    • Cyclophosphamide demonstrates a potential beneficial effect on pulmonary fibrosis in SSc patients, particularly those with elevated acute-phase proteins.
    • The findings suggest that immunosuppressive therapy may be a viable treatment option for pulmonary SSc.
    • Controlled trials focusing on SSc patients with elevated inflammatory markers are warranted to confirm these results.