Fibrosing alveolitis and polymyositis.
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An inspiratory musical sound ("squawk") was recorded in 14 patients with diffuse pulmonary fibrosis. These were divided into two groups: nine patients suffering from extrinsic allergic alveolitis (seven with bird fancier's lung and two with farmer's lung) and five patients with pulmonary fibrosis due to other causes, including rheumatoid disease, Wegener's granulomatosis, systemic sclerosis, and sarcoidosis. Clinical studies and phonopneumographic analysis of 10 consecutive squawks in each patient showed that the sound in the group with extrinsic allergic alveolitis was of shorter duration, occurred later in inspiration, and tended to be of higher frequency than the sound heard in the other group. Inspiratory crackles were present in all patients and in eight a single loud crackle preceded the squawk. We suggest that squawks, like crackles, result from the opening of airways and that the differences between the squawks in the two groups may reflect the size of the affected airways.
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The history of patients with idiopathic pulmonary fibrosis (IPF) shows that the disease may be preceded by a viral-like illness. Although viruses have not been demonstrated, it is possible that viruses were not detected in culture because they do not replicate during latency. We investigated the presence of adenovirus in IPF and interstitial pneumonia associated with collagen vascular disease (CVD-IP), using the nested polymerase chain reaction (PCR) and in situ hybridization (ISH) for the E1A region of the adenovirus genome. Studies were performed on lung tissues obtained by transbronchial lung biopsy from 19 patients with IPF, 10 patients with CVD-IP and, for comparison, 20 patients with sarcoidosis. The E1A DNA was present in 3 out of 19 (16%) cases of IPF, in 5 of 10 (50%) cases of CVD-IP, and in 2 of 20 (10%) cases of sarcoidosis. The incidence of E1A DNA in CVD-IP was significantly higher than that in sarcoidosis (p < 0.05). In patients with IPF and CVD-IP, E1A DNA was more prevalent in patients treated with corticosteroids (6 out of 9 cases; 67%) than in those without it (2 out of 20 cases; 10%) (p < 0.01). ISH studies showed that 1 out of 8 cases of IPF and CVD-IP, in which E1A DNA was detected by PCR, was positive for E1A DNA. We conclude that adenovirus E1A is unlikely to be aetiologically involved in the pathogenesis of idiopathic pulmonary fibrosis or interstitial pneumonia associated with collagen vascular disease. However, a latent adenovirus infection may be reactivated or may newly infect the host following corticosteroid administration.
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