RECURRENT PNEUMOTHORACES AS A PRESENTING SYMPTOM OF NON-SPECIFIC INTERSTITIAL PNEUMONIA IN A YOUNG MALE PATIENT

Abstract

SESSION TITLE: Fellows Diffuse Lung Disease Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: October 18-21, 2020 INTRODUCTION: Nonspecific interstitial pneumonia (NSIP) is the second most common cause of the idiopathic interstitial pneumonias and is associated with various underlying conditions including connective tissue disorders, medications and infections1. We present an unusual case of biopsy-proven NSIP presenting as recurrent pneumothoraces in a young male. CASE PRESENTATION: We present a 17-year-old male with a history of gastroesophageal reflux and an adrenal neuroblastoma status post resection with subsequent extensive chemoradiation which was curative. Years later he experienced his first of 16 pneumothoraces, each treated with the placement of a chest tube leading to resolution without complication. He later developed worsening dyspnea on exertion that limited his daily activities triggering an outpatient evaluation in which a moderate restrictive pattern was noted on his pulmonary function tests (PFTs). He was subsequently referred to our interstitial lung disease clinic for further evaluation, where a CT chest revealed ground-glass opacities with minimal interstitial changes. His PFTs continue to decline as did his symptoms and following an extensive discussion and counseling on the risks, the patient opted to undergo a biopsy to obtain a definitive diagnosis. A bronchoscopy with cryobiopsies was performed and samples were taken of the right lung. Unfortunately, he experienced a complication in the form of a pneumothorax as a result of the procedure. A chest tube was immediately placed, and he was admitted for further medical management. Despite the placement of the chest tube, he had a persistent air leak and ultimately required a VATs with pleurodesis, which resulted in the resolution of the air leak. Surprisingly the histopathology of the samples obtained showed peribronchial lymphoid aggregates, reticular fibrosis, and non-specific interstitial pneumonia, with no evidence of significant honeycombing and was consistent with an overall diagnosis of NSIP. Following these findings, a serological workup was performed to evaluate for occult connective tissue disorders but was unremarkable. DISCUSSION: While some interstitial lung diseases have been associated with the development of a pneumothorax, it is an unusual prevailing symptom with an incidence rate of 7.9% noted in a recent study2. The pathogenesis of a secondary spontaneous pneumothorax (SSP) in these cases is unclear, but it is thought to be attributed to rupture of subpleural areas of honeycombing cysts2. CONCLUSIONS: We present an unusual case of a young male with biopsy-proven NSIP, who presented following multiple pneumothoraces and moderate restriction on PFTs. Given his continued decline he was started on steroids and immunosuppressive treatment but will likely require definitive therapy in the form of a lung transplant. Reference #1: Nayfeh AS, Moore DR. Nonspecific Interstitial Pneumonitis. In: StatPearls. Treasure Island (FL): StatPearls Publishing; 2020 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK518974. Reference #2: Porcel, J.M. (2018) Secondary spontaneous pneumothorax in idiopathic pulmonary fibrosis: Grim news. Respirology, 23: 448– 449. doi: 10.1111/resp.13253. DISCLOSURES: No relevant relationships by Sruti Brahmandam, source=Web Response No relevant relationships by Sravya Brahmandam, source=Web Response No relevant relationships by Sally Suliman, source=Web Response