EXPERIENCE IN MANAGING A PATIENT WITH IDIOPATHIC PLEUROPARENCHYMAL FIBROELASTOSIS: FEATURES OF DIAGNOSIS AND TREATMENT

Abstract

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare form of idiopathic interstitial pneumonia the etio¬logy of which is often unknown, and the course of the disease is usually rapidly progressive. The disease occurs with equal frequency in both men and women. Its onset usually is observed in the second half of life. Complaints are nonspecific: patients suffer from dry cough, shortness of breath, low body mass. A distinctive feature of IPPFE is the involvement of the apices of the lungs and development of platythorax, which can be easily detected in these patients during computed tomography. There is no specific treatment, but there is an evidence of positive experience with the use of antifibrotic drugs.