Abstract Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease caused by abnormal proliferation of smooth muscle-like LAM cells, leading to progressive destruction of the lung parenchyma. It typically affects women of reproductive age, and its discovery in older patients is exceedingly uncommon. We report a case of sporadic LAM incidentally identified in a 69-year-old woman. Case Presentation The patient, a lifelong smoker with newly diagnosed atrial fibrillation, underwent routine imaging during evaluation for ischemic stroke. Chest X-ray: Demonstrated diffuse hyperlucency of both lungs. High-Resolution CT (HRCT): Revealed numerous, thin-walled, round cysts diffusely distributed throughout both lungs, without nodules or ground-glass opacities. Abdominal Imaging: No renal angiomyolipomas or features of tuberous sclerosis complex. Serum VEGF-D: Elevated, confirming sporadic LAM. Pulmonary Function Tests: Preserved lung volumes and diffusion capacity.The patient denied dyspnea, cough, or exercise intolerance. Discussion The disease was presumed long-standing but indolent, discovered incidentally in a postmenopausal woman whose hormonal milieu may have slowed progression. LAM may remain clinically silent for years and is sometimes detected during unrelated evaluations.Characteristic HRCT finding, uniform, thin-walled cyst combined with elevated VEGF-D levels can establish diagnosis noninvasively, avoiding the need for surgical lung biopsy. Management focuses on risk factor modification, avoidance of estrogen exposure, and mTOR inhibitor therapy for symptomatic or progressive disease. Conclusion This case highlights an atypical, late-onset presentation of sporadic LAM likely present for many years but clinically quiescent. Recognition of characteristic imaging features and use of VEGF-D biomarkers are essential for diagnosis. Awareness of such incidental, indolent cases broadens understanding of disease spectrum and underscores the central role of imaging in evaluation and follow-up. References McCormack FX, et al. N Engl J Med. 2011;364:1595-1606.Gupta N, et al. Chest. 2019;155:808-824.Taveira-DaSilva AM, Moss J. Clin Chest Med. 2016;37:389-403. This abstract is funded by: None
Khan et al. (Fri,) studied this question.