Case Presentation: A 50 year old caucasian male with past medical history of asthma and chronic sinusitis presented with a 1-week history of worsening gradual onset exertional dyspnea. He denied orthopnea, paroxysmal nocturnal dyspnea, chest pain, palpitations, syncopal episode, fever, cough with sputum, upper respiratory tract symptoms, sick contacts, recent travel and lower extremity swelling with pain. The remainder of the history was unremarkable. On physical exam, patient was found to be afebrile, tachycardic and tachypneic with mild wheezes in bilateral lung bases. Complete blood count revealed significant eosinophilic leukocytosis with a white count of 19,700 and 28% eosinophils. EKG showed sinus tachycardia. Chest x-ray revealed mild cardiomegaly without any pulmonary infiltrates. Subsequently, an echocardiogram was done which revealed an EF of 40% with moderate to severe pericardial effusion. He underwent pericardiocentesis and his dyspnea improved significantly. Analysis of the pericardial fluid revealed a transudative effusion. However, the etiology for his pericardial effusion remained unclear. Over the next 48 hours, he developed a new non-blanching purpura on the forehead and chest wall. Biopsy of the skin lesions showed eosinophils and leukocytoclastic vasculitis. His ANCA panel returned negative. Further review of his outpatient records revealed an elevated IgE level of 893 kU/L (reference range: 0–100 kU/L). Based on the presence of peripheral blood eosinophilia, evidence of vasculitis with extravascular eosinophils on skin biopsy and a history of asthma with chronic sinusitis, a diagnosis of EGPA was made. Prompt initiation of pulse IV corticosteroids and oral cyclophosphamide led to rapid improvement of his skin lesions over the next 2 days. He also had a repeat echocardiogram during his hospitalization which revealed an improved EF of 55% with no evidence of pericardial effusion.
Discussion: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of necrotizing vasculitis that affects small to medium sized vessels and is characterized by asthma, chronic rhinosinusitis, peripheral eosinophilia and necrotizing granulomas in the affected tissues. Although EGPA affects multiple organ systems, cardiac involvement is noted to be the most serious complication of the disease and it occurs in 50% of affected patients. Cardiac involvement in the form of myocardial infarction and arrythmias accounts for 50% of the deaths in EGPA patients and is more commonly seen in patients who are ANCA negative with a high eosinophil count. The cardiac manifestations are variable and include pericarditis, myocarditis, cardiomyopathy, myocardial infarction, pericardial effusion, heart failure, arrythmias and valvular insufficiency. The differential diagnoses that should be excluded are other forms of vasculitides such as Granulomatosis with Polyangiitis, Microscopic polyangiitis and Hypereosinophilic syndrome.
Conclusions: It is important for internists to be cognizant of the fact that pericardial effusion and other cardiac manifestations can be the first presentation in patients with EGPA and there should be a high degree of clinical suspicion for EGPA in an appropriate clinical setting. Early diagnosis and aggressive treatment with corticosteroids and cyclophosphamide can result in improved cardiac function and survival in these patients.
To cite this abstract:Gopalarathinam R, Vishwanathan S, Indramohan P, madhana Kumar A, Sharma T. Eosinophilic Granulomatosis with Polyangiitis : Not for the Lighthearted. Abstract published at Hospital Medicine 2016, March 6-9, San Diego, Calif. Abstract 524. Journal of Hospital Medicine. 2016; 11 (suppl 1). https://www.shmabstracts.com/abstract/eosinophilic-granulomatosis-with-polyangiitis-not-for-the-lighthearted/. Accessed January 24, 2020.