CRYPTOGENIC FIBROSING ALVEOLITIS - RELATIONSHIPS OF PULMONARY PHYSIOLOGY AND BRONCHOALVEOLAR LAVAGE TO RESPONSE TO TREATMENT AND PROGNOSIS

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Abstract
A minority of patients with cryptogenic fibrosing alveolitis (idiopathic pulmonary fibrosis) respond to treatment with corticosteroids. To identify factors other than histologic features that relate to responsiveness and prognosis, an analysis was made of 120 patients on whom data were prospectively collected. The relationships between pulmonary function measurements, differential cell counts on bronchoalveolar lavage fluid, physiologic response to treatment and survival are discussed. Patients responding to treatment with corticosteroids or immunosuppressive drugs had more severe initial impairment of the FVC [forced vital capacity] (P < 0.0001) and a more recent onset of disease (P < 0.001). Lavage cell counts showed that increased proportions of lymphocytes were associated with responsiveness to corticosteroids and good prognosis (P < 0.001); increased eosinophils or increased neutrophils without increased lymphocytes were associated with failure to respond (P < 0.025) and increased eosinophils were associated with a greater likelihood of progressive deterioration (P < 0.05). Life table comparisons of survival in different groups showed improved survival in patients responding to treatment (P < 0.05). Among nonresponders, those with more severe impairment of the FVC had shorter survival (P < 0.05). Physiologic and bronchoalveolar lavage data allow predictions of responsiveness to treatment and prognosis that are of practical value to clinicians.