View Abstract

A6545 - Familial Pleuroparenchymal Fibroelastosis
Author Block: S. Ozkaya1, A. Dirican2, O. S. Bekdemir3, C. Yuksel4, S. Gun5, F. Yildirim6, D. Zorlu7; 1Department of Pulmonary Medicine, Bahcesehir University, faculty of Medicine, Istanbul, Turkey, 2samsun Medicalpark Hospital, Samsun, Turkey, 3Department of Thoracic Surgery, Samsun Chest Diseases and Thoracic Surgery Hospital, Department of Thoracic Surgery, Samsun, Turkey, 4Department of Pathology, Gazi State Hospital, Department of Pathology, Samsun, Turkey, 5Department of Pathology, Ondokuzmayis University, Faculty of Medicine, Department of Pathology, Istanbul, Turkey, 6Department of Pulmonary Medicine, Gazi University, Faculty of Medicine, Department of Pulmonary Medicine, Ankara, Turkey, 7Department of Pulmonary Medicine, Ahi Evren University, faculty of Medicine, Kırsehir, Turkey.

Abstract
Pleuroparenchymal fibroelastosis(PPFE) is an uncommon disease of the pleura and lungs and it was classified as a rare interstitial lung disease(ILD) in a joint American Thoracic Society/European Respiratory Society statement on the classification of the idiopathic interstitial pneumonias. It is characterized by pleural fibrosis and fibroelastosis of the subjacent lung parenchyma, affecting predominantly the upper lobes. We aimed to present and discuss three sublings with PPFE which prove to be genetic/familial.
Case 1(1st Brohter)
36 years old man admitted to hospital with hypercapnic respiratory failure. His breathing was supporting with home-type mechanical ventilation via tracheostomy due to chronic respiratory failure. The weaning is not possible because of very low tidal volume. The weaning failure with low tidal volume were attributed to the pleural thickening and surgical decortication had been planned. Although the held twice surgical decortication, weaning was unsuccessfull. The histopathologic examination of decortication samples showed the intense fibrosis of the visceral and parietal pleura with subpleural fibrosis. Rheumatologic and specific conditions were not identified for to causes of pleura-parenchymal fibrosis. Because the weaning difficulty, tracheostomy was performed andhe discharged with home-type mechanical ventilation. Two days later, he admitted to the ER in our hospital with respiratory failure. We have re-evaluted the clinic, radiologic and histopathologic features of patient. He had “platythorax” and “pleuroparenchymal fibroelastosis” was diagnosed. The mechanic ventilation and corticosteroid treatments were started. Good response was achieved to corticosteroid treatment and the respiratory failure was temporarily recorvered. And, 4 months later, the patient was readmitted to the hospital with hypercapnic and hypoxemic respiratory failure and died. The time of survival was 14 months after the diagnosis.
Case 2(2nd Brother)
34 years old man whose little brother of 1st case admitted to the hospital with complaints of cough.Figures 4-6).
All results were consisted with pleuroparenchymal fibroelastosis and we thought it might be “ ”. VATS was planned but pleura was very thickened and pleuroparenchymal biopsies were taken with minithoracotomy. The histopathologic examination revealed the intense fibrosis of the visceral and parietal pleura with subpleural fibrosis which typically consistent with pleuroparenchymal fibroelastosis(Figure 7).
Case 3(Elder Sister)
39 years old woman whose elder sister of 1st and 2nd cases was worried for health status and admitted to the our clinic without any symptoms. Two of her brothers have been diagnosed with . All results were consisted with pleuroparenchymal fibroelastosis .