C33-23 Asthma With a Twist: When Eosinophils Take Center Stage

Abstract Introduction Eosinophilic Granulomatosis with Polyangiitis (EGPA), formerly known as Churg-Strauss, is a rare small vessel vasculitis characterized by asthma, eosinophilia and multi-system involvement. An estimated 5,000 people in the United States carry the diagnosis, and most individuals develop a diagnosis of asthma prior to multi-organ involvement. The diagnosis itself is challenging, requiring clinical, laboratory, and histopathological data without a single definitive test. This case report underscores the critical role of prompt, multidisciplinary collaboration and persistent clinical vigilance for EGPA in patients presenting with unexplained eosinophilia and multi-system symptoms. Early recognition and coordinated care are essential for optimal outcomes in this challenging and often elusive condition. Case Description A 52-year-old woman with a history of asthma, GERD, bilateral hilar lymphadenopathy presented to an outside hospital for chest pain. She had an elevated troponin with concern for acute coronary syndrome (ACS) for which she was transferred for higher level of care. Workup for ACS was negative. She had prior hospitalizations with similar symptoms for which she received prednisone bursts with resolution of her symptoms that worsened once completing the burst. The patient had also endobronchial ultrasound-guided biopsies of the hilar lymphadenopathy at the outside hospital which were unrevealing. Clinical exam on admission was significant for wheezing and intermittent neuropathic pain in the right distal leg. Labs were significant for absolute eosinophil count of 7.48X10E+09/L. Infectious workup was grossly negative. Rheumatologic workup was mildly positive at 1:40 for anti-nuclear antibodies (ANA) but otherwise negative, including antineutrophil cytoplasmic antigen (ANCA) titers. Repeat chest imaging showed bulky mediastinal and hilar adenopathy, nodular and consolidative infiltrates with concern for sarcoid for which pulmonary was initially engaged. CT sinuses showed sinonasal inflammation without nasal polyps. Transthoracic echocardiogram showed a mildly reduced ejection fraction with a moderate pericardial effusion. Cardiac MRI demonstrated patchy subendocardial late gadolinium enhancement consistent with eosinophilic myocarditis. The differential diagnosis was narrowed to either hypereosinophilic syndrome (HES) or EGPA. Bone marrow and endomyocardial biopsy performed showed eosinophilia and eosinophilic myocarditis with perivascular pattern of eosinophils, respectively. Given cardiac involvement, treatment for EGPA was initiated with high-dose glucocorticoids as well as intravenous cyclophosphamide and mepolizumab. Discussion EGPA presents significant diagnostic challenges, with frequent delays in recognition and initiation of appropriate therapy. Timely diagnosis is crucial, as cardiac involvement in EGPA greatly increases the risk of morbidity and mortality. This case underscores the vital role of coordinated multidisciplinary management in addressing the complexities of such cases. This abstract is funded by: None