Case Presentation: A 69-year-old male with a history of bladder cancer, adult-onset asthma, and stage IIIa chronic kidney disease, presented to his PCP and was found to have an elevated WBC. Upon further questioning, the patient reported non-productive cough, rhinorrhea, sinus congestion, malaise, myalgia, and arthralgia. He was started on supportive care for a suspected viral syndrome. Two days later, he developed increased pain in his jaw and cheeks with chewing. The worsening symptoms prompted further work-up for possible giant cell arteritis. Initial labs showed elevated C-reactive protein, rheumatoid factor, and CPK, 2+ proteinuria, 3+ hematuria, as well as leukocytosis with 61% eosinophils (reference <1%). Based on these findings, he was scheduled for temporal artery and renal biopsies as part of the work-up for giant cell arteritis.In recovery following the biopsy, the patient reported new-onset weakness and numbness in his right arm and was taken to the ED for stroke work-up. A head and neck CT-A showed no vascular stenosis. An MRI showed multifocal lacunar infarcts and telemetry showed episodes of SVT with heart rates into the 190s for 5 minutes. He was transferred to our hospital for more advanced care and work-up of a suspected vasculitis. Upon admission, vitals were stable, physical exam was unremarkable. Initial lab work suggested leukocytosis with eosinophilia (32%) and AKI. Chest X-ray, echocardiogram and MR-angiography were unremarkable and he was negative for MPO, ANCA, and PR-3. Following pan-negative work-up, his renal biopsy returned consistent with eosinophilic granulomatosis with polyangiitis (EGPA). Rheumatology and nephrology were consulted. He was started on 1g of IV methylprednisolone for 3 days with improvement of symptoms and transitioned to 1mg/kg prednisone following the high-dose steroid pulse. Because of his history of bladder cancer, the patient was treated with rituximab infusions instead of the standard cyclophosphamide.
Discussion: EGPA is an extremely rare disease with a prevalence of 1-3 in 100,000 patients. Diagnosis of EGPA is complicated by its non-specific symptoms. The prototypical EGPA patient presents with asthma, fatigue, myalgia, and sinusitis, which poses a diagnostic challenge. These non-specific symptoms can be easily mistaken as asthma exacerbated by a viral illness, as they were early in this case. The non-specific symptoms, in combination with the rarity of the disease means that many physicians fail to identify EGPA in a timely manner. In the context of negative ANCA testing, this patient’s diagnosis was aided by the fact that he reported jaw pain, which prompted work-up for giant cell arteritis and biopsies; without the work-up for a completely separate vasculitis and a renal biopsy, it is unclear how quickly he may have been diagnosed.
Conclusions: Here we report a case of EGPA vasculitis to raise awareness of a less common cause of stroke. EGPA presents with neurological symptoms in 62% of patients. Therefore, patients with a history of asthma, presenting with stroke in the absence of metabolic risk factors should prompt a clinical suspicion of EGPA.