Case Presentation: A 15 year old female with a history of severe persistent asthma, allergic rhinitis, and eczema presented with a two day history of cough, shortness of breath, and chest pain. She was diagnosed with asthma at age 13 and had eight presumed exacerbations and five diagnoses of pneumonia since that time. She was initially managed as an asthma exacerbation with albuterol and steroids. Review of outside records from one month ago revealed eosinophilia (28%) and chest imaging significant for mosaic lung attenuation, small pulmonary nodules, and thoracic adenopathy. She again demonstrated eosinophilia (41%) as well as an elevated troponin-I (0.118, normal <0.045) and inflammatory markers. An echocardiogram revealed low normal systolic function. Cardiology believed that her chest pain was non-cardiac in origin. Laboratory studies recommended by Allergy/Immunology suggested acute on chronic allergic inflammation. Evaluation for parasites was negative. She clinically improved and was discharged home. Ten days later she was admitted with cough, shortness of breath, and chest pain. She again had eosinophilia (64%). Chest x-ray was concerning for atypical pneumonia. Her troponin was markedly elevated (4.82); electrocardiogram was unremarkable. Cardiac catheterization revealed diastolic dysfunction and biopsy demonstrated vascular reactivity with no inflammatory cells or eosinophils. Cardiac magnetic resonance imaging demonstrated acute myocarditis and slightly worsened systolic function. She had multiple episodes of an accelerated ventricular rhythm for which she was started on metoprolol. Rheumatologic evaluation was significant for negative ANCA, eotaxin-3, and lysozyme. Head imaging demonstrated paranasal sinus mucosal thickening. Further laboratory and imaging evaluation was reassuring against a malignant process. The decision was made to administer high-dose intravenous steroids, after which her eosinophil count decreased to zero. She was diagnosed with eosinophilic granulomatosis with polyangiits (EGPA). She received cyclophosphamide given her cardiac involvement, and was discharged home on a prolonged course of steroids.
Discussion: EGPA is a vasculitis of small and medium sized arteries characterized by asthma, chronic rhinosinusitis, and prominent eosinophilia. It is a debilitating disease with multi-organ system involvement. EGPA often initially masquerades as severe persistent asthma requiring frequent and prolonged courses of steroids, or asthma accompanied by atypical pulmonary findings such as pleural effusions, pulmonary nodules, or thromboembolic disease. Prior to steroids, this disorder was uniformly fatal with 50% mortality within three months of vasculitis onset. Survival is now estimated to be 70-90% at five years with current therapies.
Conclusions: Asthma is one of the most common diseases encountered by pediatric hospitalists. Given the significant morbidity and mortality associated with rare diseases such as EGPA that present similarly, hospitalists must have a high index of suspicion for alternative diagnoses in cases that do not follow the typical disease course.