B43-33 Tale of Leaky Lungs: Complications of Interstitial Lung Disease With Pneumothorax and Pneumomediastinum

Abstract Introduction Interstitial lung disease (ILD) is a heterogeneous disease characterized by inflammation or fibrosis with hallmark symptoms of cough, dyspnea, and potentially hypoxemia. All connective tissue diseases have a 15% chance of becoming connective tissue disease-associated ILD (CT-ILD), most commonly associated with female patients less than 50 years old1. In this population, about 15-40% have a progressive disease2. CT-ILD can be present more acutely and develop rapidly progressive interstitial lung disease (RP-ILD), mainly associated with anti-melanoma differentiation-associated gene 5 (MDA5) or anti-aminoacyl-tRNA synthetase antibody. Furthermore, the anti-MDA5 positive patients also have increased incidence of spontaneous pneumomediastinum than other ILD subtypes3. This case puts forth a young, male patient who initially presented with MDA5 myositis and subsequently developed ILD complicated by a spontaneous pneumomediastinum and pneumothorax which required urgent lung transplantation. Case Description 41 year old male initially presented with proximal muscle weakness and hand pain with associated erythroderma. Patient was diagnosed with and treated for rheumatoid arthritis, but symptoms did not improve. Patient then screened for other autoimmune etiologies and found to have MDA5 dermatomyositis. Six months after initial diagnosis, patient developed a cough. Computed tomography (CT) scan showed diffuse, peripheral honeycombing with basilar predominance. Patient then referred to pulmonology, diagnosed with ILD, and subsequently hospitalized. Patient given pulsed dose intravenous methylprednisone and discharged on a slow prednisone taper. Two weeks after discharge, patient’s dyspnea worsened. Patient readmitted to the hospital for acute hypoxemic respiratory failure. Repeat CT scan showed worsening consolidation at the bases, acute pneumomediastinum, pneumothorax, and subcutaneous emphysema. A right pigtail chest tube was inserted for the pneumothorax, another round of pulsed dose steroids was initiated for possible ILD flare, and patient was urgently evaluated for lung transplantation. Fortunately, the patient had a bilateral lung transplant five days later and is currently doing well. Discussion of Clinical Significance Within six months of his diagnosis, the patient required urgent lung transplantation. One out of ten people with MDA5 dermatomyositis have their course complicated with spontaneous pneumomediastinum (SPM) or pneumothorax4. More specifically, this patient developed SPM within three months of his ILD diagnosis. If patients develop SPM within three months of their ILD diagnosis, then they have an increased mortality risk with an overall survival rate of 17.5%4. Thus, patients with MDA5 who develop spontaneous pneumomediastinum should be urgently evaluated for lung transplantation with or without diagnosis of RP-ILD as it can be lifesaving5. This abstract is funded by: None