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

Royal Brompton Hospital; Royal Free Hospital, London, United Kingdom; and Mayo Clinic, Scottsdale, Arizona

Correspondence and requests for reprints should be addressed to Athol Wells, M.D., Interstitial Lung Disease Unit, National Heart and Lung Institute, at Imperial College, Emmanuel Kaye Bld, 1 Manresa Rd., London SW3 6LR, UK. E-mail: a.wells{at}rbh.nthames.nhs.uk

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.

Key Words: fibrosing alveolitis • systemic sclerosis • nonspecific interstitial pneumonia • histopathology • survival

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