Case Presentation: A 41-year-old male with a history of recurrent sinusitis, asthma, chronic nephrolithiasis, and open angle glaucoma from steroid use presented to the emergency department with new onset pleuritic chest pain and dyspnea. Over the last 10 years, he had experienced chronic sinusitis that was significantly decreasing his quality of life. The patient previously had extensive multidisciplinary evaluation from Ear Nose and Throat, Rheumatology, Infectious Disease, Pulmonology, and Allergy/Immunology without a definitive diagnosis. During this complicated clinical course, he was treated persistently with antibiotics, had multiple sinus surgeries without clinical improvement, and had a prednisone taper that resulted in steroid induced glaucoma. Rheumatology planned to start methotrexate due to concern for an autoimmune process, however he was lost to follow up. On admission at our hospital a CT pulmonary angiogram demonstrated concern for multilobar pneumonia and atelectasis with mucous plugging. He was afebrile and hemodynamically stable. Laboratory analysis was remarkable for WBC 23.47 k/mcl, but notably there was an absence of peripheral eosinophilia. Azithromycin and ceftriaxone were initiated. Further workup revealed negative respiratory viral panel polymerase chain reaction, negative urinary antigens, and blood cultures without growth. With the lack of clinical improvement, pulmonology was consulted for a bronchoscopy. The bronchoscopy showed copious green mucous plugging the airway, inflamed mucosa, unresolving right middle and lower lobe infiltrates, and a bronchoalveolar lavage (BAL) with 58% eosinophils. The BAL cultures were unremarkable with no growth. Chart review showed prior p-ANCA positivity and low titer MPO findings. Antibiotics were discontinued and consultations were placed to Rheumatology and Allergy/Immunology who collectively diagnosed him with eosinophilic granulomatosis with polyangiitis (EGPA). Now with a confirmed diagnosis, the mainstay of treatment for EGPA with glucocorticoids was avoided in light of his prior steroid induced glaucoma. He was subsequently started on Rituximab and discharged with close outpatient follow-up.
Discussion: Traditionally, EGPA is diagnosed on 6 clinical criteria established by the American College of Rheumatology (ACR) and having 4/6 of the criteria has shown to have a 99.7% specificity for the disease. EGPA also typically manifests in 3 phases of disease. These are the prodromal, the eosinophilic, and the vasculitis phases. However, in clinical practice, as evident in this case the presentation is variable and often requires extensive workup and a multidisciplinary approach to confirm the diagnosis. The extremely high concentrations of eosinophils in the BAL combined in the clinical setting of asthma, paranasal sinus abnormalities, transient pulmonary opacities, and history of recurrent nephrolithiasis were sufficient to confirm the diagnosis.
Conclusions: This case highlights the wide variability of clinical manifestations of EGPA, and that peripheral eosinophilia is not required for the diagnosis. It is also evident that more research is needed on alternative treatment effectiveness due to the long list of adverse effects of the first line therapy, glucocorticoids. Recent research has shown promising alternatives, such as Rituxan. Randomized control trials are needed to further categorize their efficacy, specifically evaluating Rituxan’s use in the ANCA positive subset of patients.