Lupus aortitis mimicking an aortic intramural hematoma

Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder with diverse clinical manifestations. While small vessel vasculitis is a common SLE complication, lupus aortitis is an exceedingly rare entity with limited documentation. Here, we report a novel case of lupus aortitis misleadingly appearing on imaging studies as an aortic intramural hematoma in a 68-year-old male. The patient initially exhibited fatigue, dyspnea, and pericardial effusion. Imaging studies suggested an intramural hematoma of the ascending aorta and the aortic arch, prompting urgent surgical exploration. Intraoperative findings revealed a thickened, fibrotic, and heavily calcified aorta (egg-shell aorta) without hematoma. Subsequent histopathological and immunological analyses confirmed lupus aortitis. This case underscores the diagnostic challenges posed by this rare SLE complication, which can mimic other aortic pathologies. Early recognition and individualized treatment are paramount. Further studies are needed to elucidate its pathophysiology and establish standardized management guidelines.