Cryptogenic fibrosing alveolitis: Assessment by graded trephine lung biopsy histology compared with clinical, radiographic, and physiological features

Abstract

Summary Sixty-two patients with abnormal chest radiographs in whom trephine lung biopsy showed changes of cryptogenic fibrosing alveolitis are reported; 47 were male and 15 female. The clinical, radiographic, physiological and histological features before treatment have been graded and correlated with progress and response to corticosteroids. Thirty-one patients (50%) have died, 30 are known to be alive after an average of five and a half years (range two to 12 years) from diagnosis and one could not be traced. Forty patients were treated with corticosteroids; 24 (60%) improved symptomatically; half of these also improved radiographically. Radiographic improvement was associated with the presence of more intra-alveolar large mononuclear cells in biopsy specimens and less fibrosis. Younger patients had less fibrosis in their biopsies and more commonly showed radiographic improvement. All three treated patients aged less than 40 years showed such a response. Symptomatic improvement showed no significant correlation with the clinical, physical and radiological features analysed. Compared to the general population the survival of the patients as a group was shortened by deaths from fibrosing alveolitis and bronchial carcinoma. The risk of death from fibrosing alveolitis was greater among those with worse effort intolerance, more profuse radiographic opacities and more fibrosis in biopsies. The risk of death from carcinoma was independent of these factors. This study suggests that the less invasive procedure of trephine biopsy may be as informative as open lung biopsy in the assessment of cryptogenic fibrosing alycolitis.