¹Fatima AlKindi, ¹Mariam Al Ali, ¹Afra Al Dhaheri, ¹Khalid Alnaqbi,¹Anwar AlZaabi, ²Abraham George,
²Nihal Al Bashir, ^{2, 3}Ahmad Chaaban, ²Mohammad Al Hakim, ^{2, 3}Yousef Boobes

¹Tawam Hospital, ²Seha Kidney care, Nephrology, ³Department of Internal Medicine, College of Medicine and Health Sciences, United Arab Emirates University

Background(s):  Eosinophilic granulmatosis with polyangiitis (EGPA) is a small vessel vasculitis characterize by asthma, peripheral eosinophilia, and various degrees of skin, renal and neurological manifestations. The serology for P-ANCA or anti-myeloperoxidase is positive in 50% of cases. We describe very rare association of PR3-ANCA in EGPA diagnosed in hemodialysis patient.

Method(s): 63 years old female, known to have long-standing hypertension > 25 years. She had ESRD and started on hemodialysis (HD) via right IJ PermCath on 5/2019. She developed chronic hypereosinophilia with symptoms of dyspnea, rash, fatigue and nausea during HD.

Result(s): In a retrospective European multicentre cohort study including 845 EGPA patients, (28.6%) patients had MPO-ANCA and only 16 (2.2%) were PR3-ANCA positive. PR3-ANCA EGPA patients share clinical features with granulomatosis with polyangiitis (less asthma, peripheral neuropathy and more cutaneous manifestations, pulmonary nodules, lower median eosinophil count). In review of literature, only one case reported cardiac tamponade with PR3- ANCA EGPA vasculitis. Our case had PR3 ANCA EGPA vasculitis in HD patient with HD membrane allergy, and cardiomyopathy with hemorrhagic pericardial effusion.

Conclusion(s): onset of EGPA vasculitis in hemodialysis patient is uncommon and the associated with PR3-ANCA and hemorrhagic pericardial effusion is very rare. Prompt and early diagnosis vasculitis and initiation of steroid and other immunosuppression medications yielded favorable outcomes. Evaluation of hyper-eosinophilia in dialysis patients should include dialysis membrane allergy, workup of vasculitis and exclusion of other causes.