Cyclosporin A in idiopathic chronic fibrosing interstitial pneumonia without idiopathic pulmonary fibrosis

摘要

Background: We have sometimes observed interstitial pneumonia which had chronic course and unknown causes but not diagnosed as idiopathic pulmonary fibrosis (IPF). However, the treatment strategy was not established definitely. To clarify the usefulness of cyclosporin A (CsA) in idiopathic chronic fibrosing interstitial pneumonia (iCFIP) without IPF, we examined longitudinal changes in pulmonary physiology. Methods: Japanese patients with iCFIP without IPF treated with CsA were identified retrospectively. Linear mixed-effects models were used to evaluate changes in pulmonary physiology after adjusting for age, sex, and smoking history. Primary outcomes were longitudinal trajectories of the percent predicted forced vital capacity (%FVC), percent predicted diffusing capacity for carbon monoxide (%DLco), and composite physiologic index (CPI) before and after CsA. Results: Thirty-three patients were included. Before CsA initiation, %FVC, %DLco, and CPI declined at rates of 9.1%, 8.6% and ?7.1 per 1 year, respectively. After CsA initiation, the gradient of %FVC showed significant improvements in 0–1 years (6.2%±3.0%; P0.01) and in 1–2 years (10.0%±3.6%; P0.01); %DLco improved in 0–1 year (4.0%±4.6%; P=0.09) and in 1–2 years (7.0%±5.6%; P=0.02); and CPI improved in 0–1 year (3.2%±3.3%; P=0.06) and in 1–2 years (4.6%±4.1%; P=0.03). Conclusions: CsA for iCFIP without IPF may be associated with improvements in pulmonary physiology in 2 years. Further studies are needed to determine the role of CsA in iCFIP without IPF.