Cyclophosphamide is associated with pulmonary function and survival benefit in patients with scleroderma and alveolitis.

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  • Author(s): White B;White B; Moore WC; Wigley FM; Xiao HQ; Wise RA
  • Source:
    Annals of internal medicine [Ann Intern Med] 2000 Jun 20; Vol. 132 (12), pp. 947-54.
  • Publication Type:
    Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • Language:
    English
  • Additional Information
    • Source:
      Publisher: American College of Physicians--American Society of Internal Medicine Country of Publication: United States NLM ID: 0372351 Publication Model: Print Cited Medium: Print ISSN: 0003-4819 (Print) Linking ISSN: 00034819 NLM ISO Abbreviation: Ann Intern Med Subsets: MEDLINE
    • Publication Information:
      Publication: <2001->: Philadelphia, PA : American College of Physicians--American Society of Internal Medicine
      Original Publication: Philadelphia [etc.] American College of Physicians.
    • Subject Terms:
      Pulmonary Alveoli*/physiopathology; Cyclophosphamide/*therapeutic use ; Immunosuppressive Agents/*therapeutic use ; Pneumonia/*drug therapy ; Scleroderma, Systemic/*complications; Biopsy ; Bronchoalveolar Lavage ; Carbon Monoxide ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Pneumonia/mortality ; Pneumonia/physiopathology ; Pulmonary Diffusing Capacity ; Retrospective Studies ; Scleroderma, Systemic/mortality ; Treatment Outcome ; Vital Capacity
    • Abstract:
      Background: Lung inflammation (alveolitis) may cause lung fibrosis in scleroderma.
      Objective: To determine whether cyclophosphamide treatment is associated with retention of lung function and improved survival in scleroderma patients with alveolitis.
      Design: Retrospective cohort study.
      Setting: Johns Hopkins and University of Maryland Scleroderma Center.
      Patients: 103 patients with scleroderma who had bronchoalveolar lavage or lung biopsy.
      Intervention: Cyclophosphamide therapy.
      Measurements: 1) Serial measurement of forced vital capacity (FVC) and carbon monoxide diffusing capacity and 2) survival.
      Results: During a median follow-up of 13 months after bronchoalveolar lavage or biopsy, patients with alveolitis who did not receive cyclophosphamide therapy experienced a decrease in FVC (mean difference, -0.28 L [95% Cl, -0.41 to -0.16 L] and -7.1% of the predicted value [Cl, -10.9% to -4.0%]). Carbon monoxide diffusing capacity also decreased in these patients (mean difference, -3.3 x mmol min(-1) x kPa(-1) [Cl, -4.6 to -2.1 mmol x min(-1) x kPa(-1)] and -9.6% of the predicted value [Cl, -16.7% to -2.4%]). During a median follow-up of 16 months, patients with alveolitis who received cyclophosphamide were more likely to have a good outcome (stabilization or improvement) in FVC (relative risk, 2.5 [Cl, 1.5 to 4.1]) and diffusing capacity (relative risk, 1.5 [Cl, 1.0 to 2.2]). These patients also had improved survival; the median survival rate was 89% (25th, 75th percentiles, 84%, 94%) compared with 71% (25th, 75th percentiles, 55%, 86%) in untreated patients (P = 0.01, log-rank test).
      Conclusions: The presence of lung inflammation identifies patients with scleroderma who are more likely to have worsening lung function. Lung function outcomes and survival are improved in patients with alveolitis who receive cyclophosphamide.
    • Grant Information:
      1RO1HL54163 United States HL NHLBI NIH HHS
    • Accession Number:
      0 (Immunosuppressive Agents)
      7U1EE4V452 (Carbon Monoxide)
      8N3DW7272P (Cyclophosphamide)
    • Publication Date:
      Date Created: 20000617 Date Completed: 20000614 Latest Revision: 20190619
    • Publication Date:
      20221213
    • Accession Number:
      10.7326/0003-4819-132-12-200006200-00004
    • Accession Number:
      10858177