Abstract Background Connective tissue disease–associated interstitial lung disease (CTD-ILD) is a spectrum of autoimmune-mediated pulmonary disorders with overlapping radiologic patterns. Among these, systemic sclerosis (SSc)—including CREST syndrome—is a common cause of fibrosing ILD, affecting roughly 40–50% of SSc patients. High-resolution CT typically demonstrates a nonspecific interstitial pneumonia (NSIP) pattern. However, a subset of patients (20-25%) exhibits a usual interstitial pneumonia (UIP) pattern, associated with subpleural cyst formation, higher risk of spontaneous pneumothorax and poorer prognosis compared to NSIP counterparts. Case Presentation A 39-year-old Spanish-speaking male with CREST-associated ILD presented with acute pleuritic chest pain, dyspnea, and nonproductive cough. His history included Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly and serologic findings consistent with a scleroderma–lupus overlap. He was first diagnosed in 2022 after progressive dyspnea and CT imaging showing bibasilar subpleural honeycombing consistent with an UIP pattern. Initial management included oral corticosteroids, hydroxychloroquine, and mycophenolate. He was later transitioned to Rituximab due to gastrointestinal intolerance and cost-related non-adherence: initiation was delayed by over a year due to lack of insurance. Despite intermittent improvement on biannual Rituximab and low-dose prednisone 7.5 mg, he developed progressive fibrosis and severely reduced lung diffusion capacity. Chest imaging on presentation revealed a large left pneumothorax with mediastinal shift, requiring emergent tube thoracostomy. Pleurodesis was deferred to preserve transplant eligibility. Within one month, a recurrent pneumothorax occurred, secondary to subpleural bullous changes. Transplant referrals were denied due to undocumented status and socioeconomic constraints. Extensive multidisciplinary efforts have been made by pulmonology and rheumatology services to advocate for the patient’s ongoing care. Discussion Pneumothorax occurs in 6–11% of CTD-ILD cases within five years and significantly worsens prognosis, reducing ten-year survival from 80% to 30%. It signifies advanced fibrotic lung disease and presents a management dilemma between recurrence prevention (pleurodesis) and preserving transplant candidacy. This case highlights how delayed access to care - heightened by language, insurance, and immigration barriers - accelerated disease progression. Concerning our patient’s ongoing care, several ethical challenges remain - mainly systemic barriers such as immigration status and lack of insurance - all have the potential to restrict/delay access to life-saving therapies like lung transplantation. This case underscores the importance of equitable access, and patient-centered multidisciplinary and interhospital approaches in advanced CTD-ILD when considering transplantation. Multidisciplinary care and patient advocacy are essential to bridge the gap between guideline-based and real-world management. This abstract is funded by: None
Mitchell et al. (Fri,) studied this question.