Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare disease with an incidence of 1-3 cases per 100,000. It results from small to medium vessel vasculitis driven by eosinophil rich inflammation and anti-neutrophil cytoplasmic antibodies. It usually manifests with airway inflammation, followed by tissue damage in the lungs and digestive tract. Gastrointestinal involvement has been seen in roughly 50% of patients, but involvement of the gallbladder is very rare. Our case highlights a unique scenario of cholecystitis leading to an official pathological diagnosis of EGPA.
Case Description/Methods: 17 year old female with a history of asthma presented for two weeks of nausea, vomiting, abdominal pain, decreased urinary output, and 20 pound weight loss. Labs were remarkable for a WBC of 33.3, granulocytes of 10.46, eosinophils of 17.44, ESR 78, CRP 65.6, and a urinalysis showing 20-50 rbcs. Renal ultrasound was done to evaluate for pyelonephritis, but incidentally found slight thickening of the gallbladder wall and biliary sludge. She was started on ceftriaxone, and due to her clinical picture, a cholecystectomy was performed. Despite elevated WBC and eosinophils, she was discharged as symptoms improved. Gallbladder pathology returned and showed necrotizing vasculitis with fibrinoid necrosis and marked eosinophilia concerning for EGPA. New work up noted positivity for ANCA. At follow up, she acutely developed profound weakness in right upper and bilateral lower extremities, so she was re-admitted for EGPA related peripheral vasculitis neuropathy. Despite being started on steroids, she had a long hospitalization including bowel perforation, respiratory failure, seizures and glomerulonephritis. Ultimately, she stabilized on antibiotics, high dose steroids and cytoxan and was discharged to follow up.
Discussion: EGPA involving the gallbladder is extremely uncommon. As blood vessel inflammation and eosinophilic proliferation are the foundation of illness, any organ system can be involved. Lung involvement with an asthma equivalence is usually the initial presentation, but in patients with intestinal involvement, nausea, vomiting, and intestinal perforation can be seen. Biliary involvement can present as a cholecystitis picture. This case provides a degree of educational value for everyone. Early recognition is important as a multi-specialty approach is crucial, and early immunosuppressive therapy can prevent escalation of disease.
