Idiopathic Pleuroparenchymal Fibroelastosis

Abstract

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare form of idiopathic interstitial pneumonia. The disease is characterized by fibrosis of the pleura and subpleural lung parenchyma predominantly affecting the upper lobes. Various triggers have been proposed as inciting factors in the development of the disease. Diagnosis is made clinically in conjunction with radiographic findings and histopathology when available. There are no known effective treatment options and several cases of lung transplantation have been reported. We report a case of an 86-year-old female who presented to the emergency department with worsening dyspnea and hypoxia. She had a history of unexplained pneumomediastinum and a 20 - 25 pounds unintentional weight loss over 10 months. Computed tomography (CT) of the chest without contrast revealed radiographic evidence of IPPFE. Despite symptomatic management with antibiotics, diuretics, and steroids, her condition continued to deteriorate. Unfortunately, our patient was not a candidate for a lung transplant. She was transitioned to hospice care and succumbed to her disease. IPPFE is a rare disease with an unknown prevalence. It has a median survival rate of 2 years. Usually, there is an overlap with interstitial lung diseases, making it challenging to diagnose. There are only a few cases reported in the literature, and there are currently no guidelines available on the appropriate management of this debilitating disease. We recommend more cases be reported, and further research is done to establish better criteria for diagnosis and management.

Case Report: Idiopathic pleuroparenchymal fibroelastosis

“…3 The true prevalence of IPPFE is still unknown due to the absence of set criteria for its diagnosis. 5 Shioya et al detected 29 cases (7.7%) of IPPFE out of 375 cases of IPP over a 10-year period. 1 According to a review of 78 cases published up to 2013, IPPFE had a bimodal age distribution ranging between 13 and 85 years of age, with a mean of 49 years; as was reported in our case.…”

“…1 According to a review of 78 cases published up to 2013, IPPFE had a bimodal age distribution ranging between 13 and 85 years of age, with a mean of 49 years; as was reported in our case. 5 Although gender predilection is still a controversial topic, a female predominance was found in non-smoking, younger and lightweight patients. 1 Unlike our case, indeed, IPPFE mostly occurs in nonsmokers.…”

“…2 Commonly, clinical presentation is typically characterized by exertional dyspnea, dry cough, weight loss, chest discomfort and recurrent respiratory infections. 5 Pneumothorax and pneumomediastinum are less common. 5 Our patient, considering his COPD background, had productive cough with white sputum.…”

“…Clinical exam features include inspiratory crackles on auscultation, platythorax and deepened suprasternal notch; which is contrasting with our case. 5 The typic imaging features of PPFE are highly suggestive showing symmetric, bilateral, apical and irregular pleural thickening with few calcifications and bronchiectasis in some cases. 4 Middle and lower lungs may be affected in some patients.…”

“…1 The pathogenesis is thought to involve acute or subacute lung injury, including diffuse alveolar damage with aberrant tissue repair, leading to exuberant interstitial inflammation and subsequent fibrosis. 1,5 The triggering stimuli remains unknown. 1 PPFE is schematically separated into IPPFE and PPFE secondary to a number of conditions.…”