CHEST Guidelines-Chronic-Progressive-Dyspnea-in-a-71-Year-Old-Man

Chronic-Progressive-Dyspnea-in-a-71-Year-Old-Man_c (1)

Pdf Summary

This document details the diagnostic journey of a 71-year-old man with chronic progressive dyspnea, eventually diagnosed with pleuroparenchymal fibroelastosis (PPFE) after eight years of consultations. The patient, an ex-bus driver with a history of smoking, presented with chronic dry cough and progressive exertional dyspnea. Over three years, he lost about 20 kg and developed proximal muscle weakness. His medical history included gastric adenocarcinoma, coronary disease, and previous surgeries.<br /><br />Various diagnostic tests over the years, including CT scans, PET scans, and biopsies, yielded inconclusive results until it was finally diagnosed as PPFE, a rare lung disease characterized by fibrosis of the upper lobe pleura and subpleural lung parenchyma. This condition lacks widely accepted diagnostic criteria and can be idiopathic or associated with other factors such as chronic lung infections, chemotherapy, or radiation therapy.<br /><br />The diagnosis involved reviewing past CT scans, analyzing the patient's lung function tests, and utilizing a previously collected biopsy sample. The characteristic clinical and radiologic findings, combined with exclusion of other conditions, led to the diagnosis. PPFE is marked by upper lobe pleural thickening, traction bronchiectasis, and volume loss, leading to a distinctive chest CT appearance.<br /><br />Despite the diagnosis, no effective treatment exists for PPFE other than lung transplantation. Supportive care, including managing pulmonary infections and providing psychosocial support, remains the primary approach. Unfortunately, two months after discharge, the patient passed away due to advanced-stage disease. This case underscores the complexity of diagnosing PPFE and highlights the need for improved diagnostic criteria and treatment options.

Keywords

pleuroparenchymal fibroelastosis

PPFE

chronic dyspnea

lung disease

diagnostic journey

fibrosis

chronic cough

lung transplantation

radiologic findings

supportive care