Case Presentation: This is the case of a 55-year-old female with history of asthma who presented with chief complaint of abdominal pain. The patient endorsed an 8-month history of intermittent stomach discomfort that acutely worsened to severe RUQ pain, nausea, and diarrhea. Social history was notable for recent travel to China and herbal supplement use. Patient’s outpatient workup included an EGD notable for gastritis and negative H pylori. Initial labs revealed leukocytosis of 24,900 with 41% eosinophils and mild transaminitis. CT abdomen showed mesenteric stranding consistent with gastritis. Initial differential was broad including infectious etiology, autoimmune disease, vasculitis, neoplastic progress, or drug reaction. Colonoscopy was performed with biopsy showing eosinophilia and bone marrow biopsy was unremarkable. Autoimmune workup revealed low CD4 and CD8 counts, elevated IgG subsets, and negative ANCA. During the admission patient developed progressive distal extremity numbness and weakness. Patient was started on empiric intravenous immune globulin for possible Guillain-Barré syndrome (GBS) given recent symptoms consistent with gastrointestinal illness but was discontinued when lumbar puncture findings were inconsistent with GBS. Electromyogram was obtained and was consistent with eosinophilic granulomatosis with polyangiitis (EGPA), so the patient was started on pulse dose steroids given her severe acute mononeuritis multiplex. Superficial peroneal nerve biopsy was performed and was consistent with EGPA. Patient was started on Cyclophosphamide on hospital day 30. Patient was discharged on hospital day 34 with plan for continued cyclophosphamide infusions and steroid taper.
Discussion: EGPA is a multiorgan system disease with unknown exact etiology. This patient’s unusual presentation illustrates the ability of EGPA to affect any organ system and the need for high clinical suspicion for diagnosis. EGPA is typically preceded by a prodromal phase of asthma, atopic disease and allergic rhinitis. While the patient did not have any skin, ENT, or pulmonary complaints on admission, her asthma was diagnosed within 2-3 years prior to presentation. The eosinophilic phase of disease involves peripheral eosinophilia and eosinophilic infiltration of multiple organs, typically the pulmonary and gastrointestinal systems. The disease may then progress to life-threatening small and medium vessel vasculitis. The American College of Rheumatology criteria for diagnosis involve the presence of four out of six features of the disease[1]. These features are asthma, over 10% eosinophils on peripheral differential, neuropathy, transient pulmonary opacities, paranasal sinus abnormalities, and blood vessel biopsy showing extravascular eosinophils.
Conclusions: EGPA is a disease with a diverse range of presentations and has potential for severe complications. The lack of ENT involvement as seen in this patient is associated with a worse prognosis.[2] Given that this disease may affect any organ system, hospitalists should have high clinical suspicion for EGPA in patients with significant eosinophilia and history pulmonary or atopic disease.