Histopathologic Subsets of Fibrosing Alveolitis in Patients With Systemic Sclerosis and Their Relationship to Outcome

Am J Respir Crit Care Med. 2002 Jun 15;165(12):1581-6. doi: 10.1164/rccm.2106012.

Abstract

Fibrosing alveolitis associated with systemic sclerosis (FASSc) has a better prognosis than idiopathic pulmonary fibrosis. In view of recent reports that idiopathic nonspecific interstitial pneumonia (NSIP) has a better prognosis than idiopathic usual interstitial pneumonia (UIP), we classified histologic appearances of surgical lung biopsies performed in 80 patients with FASSc. NSIP (n = 62, 77.5%), subcategorized as cellular NSIP (n = 15) and fibrotic NSIP (n = 47) was much more prevalent than UIP (n = 6), end-stage lung disease (ESL, n = 6), or other patterns (n = 6). There were 25 deaths (NSIP 16/62, 26%; UIP/ESL 6/12, 50%). Five-year survival differed little between NSIP (91%) and UIP/ESL (82%); mortality was associated with lower initial carbon monoxide diffusing capacity (DL(CO)) and FVC levels (p = 0.004 and p = 0.007, respectively). Survival and serial FVC and DL(CO) trends did not differ between cellular and fibrotic NSIP. Increased mortality in NSIP was associated with lower initial DL(CO) levels (p = 0.04), higher BAL eosinophil levels (p = 0.03), and deterioration in DL(CO) levels during the next 3 years (p < 0.005). We conclude that NSIP is the histopathologic pattern in most patients with FASSc. However, outcome is linked more strongly to disease severity at presentation and serial DL(CO) trends than to histopathologic findings.

Publication types

  • Comparative Study
  • Evaluation Study

MeSH terms

  • Adult
  • Aged
  • Anti-Inflammatory Agents / therapeutic use
  • Biopsy
  • Bronchoalveolar Lavage Fluid / cytology
  • Female
  • Follow-Up Studies
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Leukocytes / metabolism
  • London
  • Lung / pathology
  • Male
  • Middle Aged
  • Predictive Value of Tests
  • Prognosis
  • Pulmonary Diffusing Capacity / physiology
  • Pulmonary Fibrosis / diagnosis
  • Pulmonary Fibrosis / etiology*
  • Pulmonary Fibrosis / mortality
  • Respiratory Function Tests
  • Retrospective Studies
  • Scleroderma, Systemic / complications*
  • Scleroderma, Systemic / diagnosis
  • Scleroderma, Systemic / mortality
  • Severity of Illness Index
  • Steroids
  • Survival Analysis
  • Treatment Outcome
  • Vital Capacity / physiology

Substances

  • Anti-Inflammatory Agents
  • Immunosuppressive Agents
  • Steroids