C48-20 The Connection Within: Persistent Pneumothorax Unmasking Bronchopulmonary Fistula in Mixed Connective Tissue Disorder

Abstract Introduction Bronchopulmonary fistula (BPF) is a sinus tract between the bronchus and the pleural cavity. Symptoms can range from pneumonia and pneumothorax to subacute emphysema. It most commonly occurs after lung resection surgeries; other causes are lung infections, malignancies, and post-radiation treatment. We present a case of bronchopulmonary fistula resulting in spontaneous pneumothorax in an elderly patient with mixed connective tissue disease. Description An 83-year-old female with a history of asthma, hypertension, and sick sinus syndrome presented to the hospital for worsening shortness of breath. She also endorsed significant weight loss in the past 6 months but denied any history of smoking. In the ER, a CTA of the chest showed a large apical left-sided pneumothorax and bilateral dense ground glass opacities concerning acute pneumonitis or possible interstitial lung disease (ILD) with lower lobe bronchiectasis. A chest tube was placed in, Cefepime was started for suspected pneumonia, and IV methylprednisolone was started for possible concerns of ILD flare. Patient reported difficulty swallowing in the past couple of months; labs showed elevated ESR and CRP with positive rheumatoid factor, RNP, and SSA levels. Rheumatology was consulted and was determined that patient has mixed connective tissue disorder; additional labs showed normal C3, C4, CH50 and anti-DNA levels, indicating inactive autoimmune disease. Despite multiple chest tube replacements and subsequent pigtail catheter placement, tube continued to have persistent air leak. Patient underwent thoracoscopy with bullectomy and pleurodesis. A pathology report showed fibroblastic proliferation consistent with ILD. The air leak continued to persist, and the patient was transferred to higher care tertiary center where she underwent left thoracotomy, which showed bronchopulmonary fistula, the fistula was oversewn. A couple of days later, air recurred, and the patient developed subcutaneous emphysema. A placement for Heimlich valve was planned, but the patient elected for hospice care and was discharged. Discussion a bronchopulmonary fistula causing spontaneous pneumothorax is a rare but dangerous pulmonary complication, diagnosis and management of bronchopulmonary fistula remain a challenge. Early recognition is crucial to reduce morbidity and mortality. This case emphasizes the importance of considering bronchopulmonary fistula in patients with persistent pneumothorax despite proper treatment. This abstract is funded by: None