117Volume 3 • Issue 2 • 1000127Madridge J Intern Emerg Med.ISSN: 2638-1621

MadridgeJournal of Internal and Emergency Medicine

Case Report Open Access

Pleuroparenchymal Fibroelastosis: A Case ReportAbdulrahman Nasiri*, Reda Elsaud and Mohammed AlfifiDepartment of Internal Medicine, Security Forces Hospital (SFH), Riyadh, Saudi Arabia

Article Info*Corresponding author:Abdulrahman NasiriProfessorDepartment of Internal medicine, Security Forces Hospital (SFH)PO Box 63146, Riyadh 11516Saudi ArabiaE-mail: dramnasiri@gmail.com

Received: February 8, 2019Accepted: February 19, 2019Published: February 25, 2019

Citation: Nasiri AR, Elsaud R, Alfifi M. Pleuroparenchymal Fibroelastosis: A Case Report. Madridge J Intern Emerg Med. 2019; 3(2): 117-120.doi: 10.18689/mjiem-1000127

Copyright: © 2019 The Author(s). This work is licensed under a Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Published by Madridge Publishers

AbstractIntroduction: Pleuroparenchymal fibroelastosis (PPFE) is rare condition characterized by elastic fibrosis of the pleura and subpleuralparenchyma with upper lobe being predominantly affected. PPFE has been included in the Update of the International Multidisciplinary Classification of the Idiopathic Interstitial Pneumonias as part of the rare. The etiology of PPFE is unclear but associations with lung and bone marrow transplantation as well as chemotherapy have been proposed. In the present report, we describe a case of 72 years old male presented to our hospital with history of cough with sputum production and right chest pain for the last 3 months. Chest X-ray showed apical mass on the right lung. Surgical lung biopsy revealed PPFE in the upper lobes. To our knowledge, this is the first report in Saudi Arabia, there have been no published reports of diagnosed cases of PPFE.

Conclusion: PPFE usually shows a progressive clinical course and carries a poor prognosis. There is no effective treatment for PPFE apart from lung transplantation, other medication can be used as supportive care and includes: corticosteroids, cyclophosphamide, azathioprine, N-acetylcysteine, azithromycin, sulfamethoxazole and trimethoprim.

Keywords: Pleuroparenchymal; Fibroelastosis; Rare; Lung; Idiopathic Interstitial Pneumonias.

Abbreviation: PPFE: Pleuroparenchymal Fibroelastosis; DLCO: Carbon monoxide Diffusion capacity; CT: Computerized Tomographic scan; IIP: Idiopathic Interstitial Pneumonias.

IntroductionPleuroparenchymal fibroelastosis (PPFE) is rare condition characterized by elastic

fibrosis of the pleura and subpleural parenchyma with upper lo be being predominantly affected [1]. PPFE has been included in the update of the international multidisciplinary classification of the Idiopathic Interstitial Pneumonias as part of the rare IIP with idiopathic lymphocytic interstitial pneumonia. The etiology of PPFE is unclear but associations with lung and bone marrow transplantation as well as chemotherapy have been proposed [2,3]. To our knowledge, this is the first report in Saudi Arabia; there have been no published reports of diagnosed cases of PPFE.

Patient InformationA 72 years old male known case of diabetes mellitus type 2 and hypertension

presented to our hospital with history of cough with sputum production and right chest pain for the last 3 months.

He was a 60-pack-year former smoker and had no significant history of occupational exposure, allergy, and family history of pulmonary fibrosis, substance use and any symptoms suggestive of connective tissue diseases. Past medical history included trauma at age of 21 after which the patient had multiple surgeries on his arm. The patient had lost 15 kg in weight during that period.

ISSN: 2638-1621

Madridge Journal of Internal and Emergency Medicine

Madridge J Intern Emerg Med.ISSN: 2638-1621

118Volume 3 • Issue 2 • 1000127

Clinical FindingsExamination revealed diminished breath sounds,

bronchial breathing in the right upper lung zone, O2 saturation on room air was 92% and no clubbing was noted. Pulmonary function teat showed restrictive pattern.

Diagnostic AssessmentApart from lipid profile laboratory investigation were within

normal limits, chest x-ray showed apical mass on the right lung, no pleural effusion or pleural thickening was noted (Figure 1).

High-resolution computed tomography scan (HRCT) showed apparent enlarged lobulated mass lesion occupying apex of the right lung (Figure 2), it measures 5.13 × 5.96 × 4.48 cm. It shows tiny punctate calcification inside. It is predominant wide pleural base attachment with apical medial pleura and subpleural location, with mild encroachment upon the mediastinum, with slightly deviated trachea and esophagus toward the left side, with no signs of significant narrowing or occlusion (Figure 3). Rest of both lung fields seems to be clear with no other suspicious mass lesion. Pulmonary scarring at the right middle lobe is noted could be related to an old infection.

Figure 1. Chest x-ray showed apical mass on the right lung, no pleural effusion or pleural thickening was noted.

Figure 2. High-resolution computed tomography scan (HRCT) showed apparent enlarged lobulated mass lesion occupying apex of the right lung.

Figure 3. High-resolution computed tomography scan (HRCT) showed apparent enlarged lobulated mass lesion occupying apex

of the left lung.

Bronchoalveolar lavage was normal. There was no clinical or serological evidence of a connective tissue disease, vasculitis or extrinsic allergic alveolitis. Lung function showed a significant decrease in forced vital capacity (FVC) (Table 1). Six-minute walking test demonstrated a walking distance of 476 m with desaturations (86%).

Table 1. Pulmonary function tests.

FVC [%pred] 1.97 (54 %)

FEV1 [%pred] 1.96 (70 %)

TLCO [%pred] 7.75 (94.4 %)

The smoking history placed possibility of bronchogenic carcinoma on the top of our differential diagnosis. Other differentials considered were granuloma, hamartoma, idiopathic pulmonary fibrosis, idiopathic pleuroparenchymal fibroelastosis and chronic hypersensitivity pneumonitis. The patient was admitted and underwent thoracoscopic lung biopsy under general anesthesia. The patient did well postoperatively and discharged home in a stable condition.

The specimen was sent for histopathological examination which revealed thickened visceral pleura and prominent subpleural fibrosis characterized by elastic fiber deposition in the subpleural area and adjacent pulmonary parenchyma (Figures 4A-4C).

Because of the rare incidence the specimen was sent to another hospital and the diagnosis was confirmed.

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Figure 4. Section of a lung biopsy which displays subpleural fibroelastosis. A. Surgical lung biopsy specimen at low power

showed markedly thickened visceral pleura and prominent sub pleural fibrosis characterized by abnormal increase of elastic tissue

Figure 4. Section of a lung biopsy which displays subpleural fibroelastosis. B. Abrupt transition to normal parenchyma was also

seen.

Figure 4. Section of a lung biopsy which displays subpleural fibroelastosis. C. Parenchyma distant from the pleura was spared.

Therapeutic InterventionWe elect to use conservative treatment as daily activities

of the patient were not affected (MRC grade 1) with close follow ups every 2 months for the first year then biannual visits.

DiscussionPleuroparenchymal fibroelastosis (PPFE) is rare condition

characterized by elastic fibrosis of the pleura and subpleural parenchyma with upper lobe being predominantly affected [1]. PPFE has been included in the update of the international multidisciplinary classification of the idiopathic interstitial pneumonias (IIP) as part of the rare IIP with idiopathic lymphocytic interstitial pneumonia. The etiology of PPFE is unclear but associations with lung and bone marrow transplantation as well as chemotherapy have been proposed [2-5].

The presentation of PPFE in reported cases happens in the fifth decade of life and has no sex predilection [1-3] with symptoms of cough, shortens of breath either on exertion or in worsening course, recurrent infection and weight loss [1,5-8]. Moreover, patients with PPFE seems to have high risk for developing pneumothorax during the disease course [2,3,9].

The physical examination of patients with PPFE may reveal flattened chest, crackles are audible in about half of the reported cases and rarely clubbed finger [4,6,10,11].

Imaging of the chest might show irregularly thickened pleura on frontal view of chest x-ray. Chest CT demonstrates pleural thickening, subpleural nodular or reticular opacities in the lung parenchyma at the apex, traction bronchiectasis and subpleural and peribronchial consolidation with minimal middle and lower lobes involvement [10,12,13].

The Pulmonary Function test pattern is restrictive with decreased DLCO [10,11].

The differential diagnosis includes asbestosis, connective tissue diseases, advanced fibrosing sarcoidosis and radiation or drug induced lung disease as these disease causes both pleural and parenchymal fibrosis [8,9,14].

The diagnosis of PPFE requires a multidisciplinary approach with input involving the physician, radiologist, and pathologist [2,5].

Lung biopsy or post-mortem examination is basic for the unequivocal conclusion of PPFE, imaging discoveries are additionally basic as the initial step to the last analysis, as in different IIPs [1,10].

Criteria for the histological and radiological diagnosis of PPFE have been suggested [1,2,11,14].

PPFE usually shows a progressive clinical course and carries a poor prognosis. There is no effective treatment for PPFE apart from lung transplantation, other medication can be used as supportive care and includes: corticosteroids, cyclophosphamide, azathioprine, N-acetylcysteine, azithromycin, sulfamethoxazole and trimethoprim [1,6,8,9].

Patient perspective

Informed consent: Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

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ConclusionPPFE usually shows a progressive clinical course and carries

a poor prognosis. There is no effective treatment for PPFE apart from lung transplantation, other medication can be used as supportive care and includes: corticosteroids, cyclophosphamide, azathioprine, N-acetylcysteine, azithromycin, sulfamethoxazole and trimethoprim.

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