A 62 year-old man presented with decreased exercise capacity and chronic cough over the last year. High resolution computed tomography(HRCT) of the chest, pulmonary function testing, and cardiopulmonary exercise testing were performed with non-specific results, eventually followed by video assissted thorascopic surgical(VATS) lung biopsy that led to a diagnosis of pleuroparenchymal fibroelastosis. This is a rare, relatively new clinicopathologic diagnosis. The clinical presentation is similar to other chronic interstitial pneumonias, with pathologic findings that do not fit any other currently defined conditions.
The patient is a 62 year-old male who presented with complaints of decreased exercise tolerance as well as chronic cough over the past year. He continued to walk 3-4 miles several times per week, play golf, and lift weights, although he noted he could not be as vigorous as before. His cough occurred throughout the day, and was occasionally productive of clear sputum. He denied any hemoptysis, wheezing, or dyspnea at rest or with minimal exertion. His other review of systems was only positive for persistent hoarseness. He has no significant past medical or surgical history. He is a lifelong non-smoker and has no exposure history. His medications included inhaled steroids. His mother died at the age of 47 of an unknown pulmonary disease. His physical exam was unremarkable, with lungs clear to auscultation and percussion. Several prior computed tomography scans were reviewed that revealed bilateral patchy infiltrates that had been stable back to 2001. Spirometry showed a decrease in volumes without evidence of airflow obstruction and no significant change post bronchodilator. Lung volume measurements indicated a moderately severe reduction in residual volume and a mild reduction in total lung capacity. Diffusing capacity for carbon monoxide was also mildly reduced. Arterial blood gas on room air revealed a pH of 7.45, PaCO2 of 40 mmHg and a PaO2 of 96 mmHg. His 6-minute walk test was normal. A HRCT of the chest showed multiple pleural based abnormalities and bronchiectasis in the superior segment of the lower lobes. Pleural based opacities were noted in both upper lobes, as well as extensive interstitial opacities throughout both lungs. VATS biopsy subsequently revealed fibrosing interstitial pneumonia with marked pleural fibrosis and subpleural accentuation. It also showed abundant chronic inflammation with small lymphocytes present in association with the pleura, predominantly in the upper lobes.
Idiopathic pleuroparenchymal fibroelastosis is a clinicopathologic diagnosis that has been recently reported. The clinical presentation is similar to other chronic idiopathic interstitial pneumonias with symptoms of exertional dyspnea and chronic cough. Radiographs show pleural and parenchymal involvement with upper lobe predominance. Pathologic findings include intense fibrosis of the visceral pleura, prominent, homogenous subpleural fibroelastosis, sparing of the parenchyma distant from the pleura, mild, patchy lymphoplasmacytic infiltrates, and small numbers of fibroblastic foci present at the leading edge of the fibrosis. Other entities that are characterized by both pleural and parenchymal fibrosis include asbestos-related disease, connective tissue-associated disease, and lung disease related to radiation injury or drugs. Our patient did not have evidence of any of the above.
This report highlights the clinical presentation, radiographic findings, and pathology of a relatively new entity that is not classifiable as one of the currently defined chronic interstitial pneumonias. It has been termed idiopathic pleuroparenchymal fibroelastosis.
D Sams, None.