CYSTS AND A CRASH: A CASE OF TUBEROUS SCLEROSIS COMPLEX-ASSOCIATED LYMPHANGIOLEIOMYOMATOSIS

TOPIC: Diffuse Lung Disease TYPE: Fellow Case Reports INTRODUCTION: Lymphangioleiomyomatosis (LAM) is a rare, multiorgan disease characterized most commonly by diffuse, cystic destruction of the lungs. LAM occurs sporadically (sporadic-LAM), almost exclusively in women, or with Tuberous Sclerosis Complex (TSC-LAM), both men and women. Sporadic-LAM an incidence 3-8 cases per 1,000,000 patients. TSC 1 5000 to 10000 live births prevalence ranges from 30-80% over lifetime. Common presentations include fatigue, dyspnea, spontaneous pneumothorax, pleural effusion. We present case middle-aged female TSC-LAM complicated respiratory failure, pneumothoraces, chylothorax. CASE PRESENTATION: The patient 52-year-old African American history epilepsy, hypertension, depression. She was her usual state health until being found obtunded hypoxic vehicle. CT chest revealed bilateral thin-walled cysts, suggestive LAM. discharged on supplemental oxygen due persistent hypoxia. diagnosis later confirmed biopsy. Two years later, she presented chylothorax which resolved tube thoracostomy drainage. poor candidate for lung transplant high risk seizures calcineurin inhibitors required post-transplant. A few months pneumothoraces requiring pleurodesis. has since been Sirolimus (3 years). Pulmonary function tests (PFTs) continue show severe obstruction diffusion defect. Her symptoms functional status have stabilized no further complications last 3 years. DISCUSSION: clinical made identifying multiple (>10), bilateral, well-defined cysts High-Resolution Computed Tomography (HRCT) TSC3. should be differential young known suspected who presents progressive and/or effusion8. Asymptomatic females undergo screening HRCT at age 18 conjunction PFT 6-minute walk test early diagnosis, accurate prognostication, improved median transplant-free survival1. Patients moderate-to-severe impairment initiated mTOR slow decline function2. refractory referred evaluation3. CONCLUSIONS: major cause morbidity mortality patients that underdiagnosed5. With advent newer therapeutics delay progression, education presenting surveillance can improve prevent length survival. REFERENCE #1: Cudzilo CJ, Szczesniak RD, Brody AS, et al. Screening Women Sclerosis. CHEST. 2013;144(2):578-585. doi:10.1378/chest.12-2813 #2: McCormack FX, Inoue Y, Moss J, Efficacy Safety Lymphangioleiomyomatosis. New England Journal Medicine. 2011;364(17):1595-1606. doi:10.1056/nejmoa1100391 #3: Gupta N, Lee H-S, Ryu JH, NHLBI Registry. 2019;155(2):288-296. doi:10.1016/j.chest.2018.06.016 DISCLOSURES: No relevant relationships Maida Hafiz, source=Web Response disclosure file Ogugua Obi; Amish Parikh,