A 53-year-old woman required ten hospital admissions and three rheumatology assessments over a two-year period before clinicians diagnosed her with eosinophilic granulomatosis with polyangiitis, according to a case report published September 29, 2026, in Cureus by Mary Knowles, Pak Him Timothy Leung, and Suresh Chandran. The patient initially presented with non-specific respiratory symptoms, persistent low-positive proteinase 3 anti-neutrophil cytoplasmic antibody (PR3-ANCA) serology, and an active smoking history of roughly ten cigarettes daily, alongside electronic cigarette use and a prior history of intranasal cocaine use that caused destructive sinonasal disease.
Diagnostic Challenges in Recurrent Hospital Admissions
During the two years preceding her formal diagnosis, the patient received diagnoses for community-acquired pneumonia, lower respiratory tract infections, chronic obstructive pulmonary disease (COPD) exacerbations, cellulitis, and acute-on-chronic pancreatitis. Clinicians documented intermittent purpuric skin rashes during several of those admissions. Three separate rheumatology evaluations occurred due to persistent low-positive PR3-ANCA serology, but doctors withheld immunosuppressive therapy because her renal function remained preserved with a normal urine protein-to-creatinine ratio, and she showed no articular or systemic end-organ involvement.
Emergence of Marked Eosinophilia and Systemic Vasculitis
Retrospective analysis of serial laboratory data revealed persistent peripheral blood eosinophilia over several months, escalating to a hypereosinophilic peak of 15.5 × 10⁹/L roughly seven weeks before her index emergency presentation. When she arrived at the hospital following an episode of transient loss of consciousness, her eosinophil count measured 9.1 × 10⁹/L against a normal reference range of 0.0 to 0.5 × 10⁹/L. Physical examination revealed palpable purpuric lesions across both lower limbs, feet, upper limbs, and the chest, paired with non-pitting ankle oedema, painful oral ulcers, and nasal crusting.
Multidisciplinary Management and Treatment Response
Multidisciplinary review by rheumatology and respiratory teams established a clinical diagnosis of EGPA without requiring histological confirmation via tissue biopsy. Medical staff administered high-dose corticosteroid therapy, which resulted in rapid clinical improvement and normalization of the eosinophil count. Within approximately 48 hours, her peripheral eosinophil count dropped to 0.4 × 10⁹/L, demonstrating a rapid biochemical response. Cutaneous lesions improved, and inflammatory markers declined. Approximately one month after discharge, she suffered a readmission for pulmonary haemorrhage attributed to EGPA, which physicians treated successfully with intravenous methylprednisolone followed by a tapering oral prednisolone regimen.
Common Questions About Eosinophilic Granulomatosis With Polyangiitis
What is eosinophilic granulomatosis with polyangiitis?
Eosinophilic granulomatosis with polyangiitis, formerly designated as Churg-Strauss syndrome, is a rare antineutrophil cytoplasmic antibody-associated vasculitis defined by eosinophilic inflammation, granulomatous disease, and necrotising small- to medium-vessel vasculitis.
Why is diagnosing EGPA frequently delayed?
Diagnosis is often delayed because early clinical manifestations evolve gradually over months or years, mimicking more common respiratory, infectious, or inflammatory conditions like asthma, COPD, and community-acquired pneumonia.
How common is ANCA positivity in EGPA patients?
Antineutrophil cytoplasmic antibodies are detected in only about 30% to 40% of EGPA patients, most frequently directed against myeloperoxidase (MPO-ANCA), though proteinase 3 ANCA can occasionally appear.
What are the primary treatments for EGPA?
Current clinical recommendations position systemic corticosteroids as first-line therapy for remission induction, with additional immunosuppressive or biologic agents reserved for severe, organ-threatening, or relapsing disease manifestations.
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