JUST ANOTHER CASE OF CHEST PAIN? EGPA MYOCARDITIS

Abstract

SESSION TITLE: Medical Student/Resident Signs and Symptoms of Chest Disease Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Eosinophilic granulomatosis with polyangitis (EGPA), formerly called Churg-Strauss syndrome, is a rare vasculitis affecting small and medium vessels. While EGPA has multisystem involvement, cardiac involvement accounts for nearly half of the deaths. CASE PRESENTATION: 63-year-old male with a past medical history of asthma presented with acute onset of chest pain and exertional dyspnea. Review of systems was significant for a 3 months history of bilateral feet numbness. Vitals were remarkable for tachycardia (115/min); cardiopulmonary and abdominal examinations were unremarkable. Neurological examination was significant for left foot drop and a sensory deficit in both feet. Labs revealed leukocytosis (32,000/mm3) with 52% eosinophils; troponemia (2.018 ng/ml) & an elevated ESR (80 mm/hr). EKG revealed sinus tachycardia and echocardiography revealed a trace hemodynamically insignificant pericardial effusion. Left heart catheterization was without coronary arteriopathy. Serum IgE level was 1499 IU/mL and ANA, c-ANCA and p-ANCA were negative. CT chest demonstrated a new parenchymal opacification in the right upper lobe and CT sinus showed bilateral ethmoidal and frontal sinus mucosal disease. Bone marrow biopsy excluded a myeloproliferative disorder. Sural nerve biopsy revealed vasculitis with endoneural fibrosis. Per ACR criteria, the patient was diagnosed with EGPA. Treatment included prednisone (1mg/Kg/day) with resultant resolution of leukocytosis, eosinophilia, chest pain and dyspnea, the latter of which were attributed to myocarditis. DISCUSSION: EGPA is the least common of the ANCA-positive vasculitides. However, only 30 to 60 % of EGPA patients are ANCA positive (1), therefore negative MPO-ANCA and PR3-ANCA do not exclude the diagnosis of EGPA. EGPA can be diagnosed with 99.7% specificity if 4 of 6 of the following criteria are met: asthma, greater than 10% eosinophils on leukocyte differential, mononeuropathy including multiplex or polyneuropathy, migratory or transient pulmonary opacities on chest radiograph, paranasal sinus abnormality, and biopsy showing vasculitis (2). Our patient had multiorgan involvement with upper and lower airway, myocardial/pericardial and neural disease. EGPA with cardiac involvement has a significantly higher mortality rate and about 50% of EGPA deaths are those with cardiac involvement (3). The typical cardiac involvement seen in EGPA includes myocarditis, pericarditis, heart failure, valvular insufficiency. CONCLUSIONS: Multiorgan involvement, atypical presentation, ANCA negativity makes diagnosis challenging, making careful history and physical and thorough systems review vital. Cardiac involvement is the leading cause of EGPA deaths and hence, EGPA, although rare, should be considered in the differential diagnosis in a patient presenting with myopericarditis. Prompt diagnosis and treatment help to prevent complications. Reference #1: 1) Cottin V, Bel E, Bottero P, et al. Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): A study of 157 patients by the Groupe d’Etudes et de Recherche sur les Maladies Orphelines Pulmonaires and the European Respiratory Society Taskforce on eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Autoimmun Rev. 2017 Jan;16(1):1–9. Reference #2: 2)Masi AT, Hunder GG, Lie JT, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum. 1990 Aug;33(8):10941–100. Reference #3: 3) Cardiac involvement in eosinophilic granulomatosis with polyangitis (Churg Strauss) (RCD code: I-3A.7a) Szczeklik W, Miszalski-Jamka T. https://jrcd.eu/index.php/crcd/article/download/56/26 J Rare Cardiovasc Dis. 2013;1:91–95. DISCLOSURES: No relevant relationships by Floyd Burke, source=Web Response No relevant relationships by Jovan Gayle, source=Web Response No relevant relationships by Somshukla Ghosh, source=Web Response