Cryptogenic fibrosing alveolitis: response to corticosteroid treatment and its effect on survival

Abstract

We have studied retrospectively 220 patients with cryptogenic fibrosing alveolitis (CFA) who were first seen between 1955-73 and had been followed up for at least four years until 1977. Seventy-seven patients had received no treatment and 143 had received corticosteroids. The only clinical difference between the groups was the age at presentation (untreated mean age 61 years± 11 SD; treated mean age 56 years± 11 SD p < 0001). The influence of corticosteroid treatment has been assessed both in terms of the clinical, radiographic, and physiological response after four to eight weeks and in terms of survival, using a log rank method of analysis. Of 143 treated cases 127 had detailed follow-up information. Seventy-two (57 %) had substantial subjective improvement of breathlessness but only 22 (17 %) of the total showed additional objective improvement. The only factors significantly distinguishing the 72 subjective responders from the 55 non-responders, and which therefore might be used as predictors of response, were a younger age at presentation (p < 0 001) and less dyspnoea (p < 0 02). Analysis of survival data demonstrated that treated patients who showed an early good response to steroids had a markedly better survival than non-responders (p <0 0 1). The survival curve for the untreated group lay between the two steroid groups. Analysis of the survival data suggests that steroid responsiveness relates particularly to a subgroup with an inherently better prognosis, which is characterised by a younger age and probably by a cellular histology not however necessarily typical of desquamative interstitial pneumonia. Nevertheless treatment appears to have an additional influence on survival especially in early disease, which is not observed in the untreated cases. A large number of factors are not related either to steroid responsiveness or to survival and these include the presence of associated "connective tissue" diseases, autoantibodies, and the duration of dyspnoea at presentation. It is concluded that biopsy information is valuable in assessment of potential to respond to steroids and that if steroid treatment is to be helpful it must be used at an early stage. The response is very variable and titration of the steroid dose against an individual patient's response is likely to give better results than adherence to arbitrary dose schedules.

Pulmonary Function Testing in Idiopathic Interstitial Pneumonias

“…In a separate study, a subgroup of patients participating in a therapeutic trial underwent spirometric measurement during a mean duration of 3 wk (52); none of 81 patients with IPF had more than a 10% decrease in percentage of predicted FVC whereas only 1 patient had a 10% or greater increase in FVC. To date, most investigators have defined clinically significant changes in spirometry for patients with IPF as a change in FVC exceeding 10-15% (38,(53)(54)(55)(56)(57)(58)(59). In general, the Dl CO change believed to be clinically significant has been greater than 20% (38,53,54,56).…”

A Role for the Receptor for Advanced Glycation End Products in Idiopathic Pulmonary Fibrosis

“…This treatment produces an objective response in only 10 to 20% of patients and has a minimal effect on the fatal course of IPF. [2][3][4] Thus, the need for new therapeutic modalities is evident.…”

Idiopathic pulmonary fibrosis: treatment options in pursuit of evidence-based approaches

“…While this may be appropriate for treatment with lone corticosteroid therapy [2], colchicine [3,4], cyclophosphamide [5,6] and d-penicillamine [7], care should be taken in generalising the notion to treatment with all currently available drugs. For instance, several years ago, combined low-dose prednisone plus azathioprine, compared with low-dose prednisone alone, did produce significant positive signals in a prospective, double-blind, randomised, placebo-controlled study in patients with newly diagnosed IPF [8].…”