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A1550 - Pleuroparenchymal Fibroelastosis a Case Report
Author Block: M. A. Ramirez Candelas1, O. Barreto1, I. Buendia2, k. Jassen Avellaneda1, M. Gaxiola2; 1Enseñanza, Instituto Nacional de Enfermedades Respiratorias, Tlalpan, Mexico, 2investigation, Instituto Nacional de Enfermedades Respiratorias, Tlalpan, Mexico.
INTRODUCTION: Pleuroparenchymal Fibroelastosis is a clinical pathological entity recently described in literature; characterized by fibrosis and septal thickening in pleural and subpleural parenchyma. Mainly, the affection is observed in upper lung lobules with loss of lung volumes and structural distortion. Fibroelastosis presents fibrosis in elastics fibers, which mark the difference from other interstitial lung diseases that present fibrosis in collagen fibers. The similarity between histologic and radiologic features for Pleuroparenchymal Fibroelastosis has been used to propose several criteria that may establish a definitive and consistent pattern for diagnosis. Clinic Case: We present a case report of Pleuroparenchymal Fibroelastosis. Data was obtained from the medical record. Computed tomography lung patterns characteristics were analyzed and histopathological images were obtained from lung biopsies. A study case of a 65-year-old patient with previous history of a brother who died at the age of 56-years-old from idiopathic pulmonary fibrosis. In her pathological backround, the patient had passive tobacco exposure for 13 years; myelodysplastic syndrome since 2010 in treatment with erythropoietin; diagnosed with ulcerative colitis 11 years ago in treatment with sulfasalazine and steroids, and idiopathic pulmonary fibrosis since 2015 in treatment with pirfenidone. The symptomatology presented by the patient was acute dyspnea and 3 years with productive cough, without improvement of her symptoms. Pulmonary function tests delivered: Forced expiratory volume in 1 second (FEV1)/Forced volume vital capacity (FVC): 0.73, FVC: 1.79 liters (54%), FEV1: 1.31 liters (53%). Diffusing capacity of the lungs for carbon monoxide: 35%. Computed tomography lung scans showed in upper lung lobules loss of lung volumes, subpleural honeycomb pattern and traction bronchiectasis. A Lung biopsy was required for diagnosis of Pleuroparenchymal Fibroelastosis. Discussion: Pleuroparenchymal Fibroelastosis is a poorly described disease and this is the first Mexican case report of this entity. This type of interstitial lung disease has been reported as a complication after bone marrow transplants, in lung transplanted patients and after the usage of some chemotherapeutic drugs (particularly in alkylating agents). The identification of this disease is very important to define prognosis and for development of different and strategic therapeutic treatments, especially due to the fast progression this entity possesses.