A discussion on a suspected case with EGPA after maintenance hemodialysis for 5 years and related literature analysis: A case report.

Abstract

Rationale: Pathological featuring by necrotizing granulomatous inflammation of peripheral blood and tissues with increased eosinophils infiltrating small and medium vessels, eosinophilic granulomatosis with polyangiitis (EGPA), a family of rare antineutrophil cytoplasmic antibody (ANCA) associated with systemic vasculitis. With low morbidity, diverse clinical manifestations, and difficult early diagnosis, the majority of patients are confirmed after multiple organ damages, thus missing the best treatment time and having a poor prognosis. About 25% to 30% of EGPA cases have been reported to suffer from the renal disease, and there are few studies on EGPA complicated with kidney damage, most of them on ANCA-positive patients. Generally, the initial diagnosis of EGPA on maintenance hemodialysis is even rare. We report a case of a patient with maintenance hemodialysis for 5 years and then was diagnosed with EGPA.

Patient concerns: The female patient, 54-year-old, having maintenance hemodialysis for 5 years consecutively, was hospitalized for the recurring rash in the past 3 years and then exacerbation in the last 2 months. With the previous history of bronchial asthma having attacked frequently recently, it could be observed from peripheral blood that the eosinophils increased, from the cardiac color ultrasound that it was prone to eosinophilic endocarditis, from 5 tests for vasculitis that P-ANCA and MPO-AB were positive.

Diagnoses: The patient's onset is renal dysfunction, with maintenance hemodialysis for 5 years, recurrent lung infections, combined with eye lesions, scattered skin rashes, P-ANCA positive, MPO-AB positive, asthma present, eosinophil absolute value 1.60 × 109/L, total score >6 points, diagnosis considering EGPA.

Interventions: Due to multiple organ damage, the patient received treatment with a combination of steroids and cyclophosphamide.

Outcomes: After 2 days, the patient's rash significantly darkened compared to before, wheezing improved, and eosinophils returned to normal levels.

Lessons: The ANCA test shall be put on the high agenda for patients presenting with kidney failure at first. Meanwhile, the neglected immune monitoring for patients with dialysis tells us that it is of great significance for this kind of patient to have immune monitoring in the early diagnosis of EGPA.