Mr Michael Ooi1, Dr Prianka Puri2
1Princess Alexandra Hospital, Brisbane, Australia, 2Royal Brisbane and Women’s Hospital, Brisbane, Australia
Background: Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis is rare, but associated with significant mortality and morbidity, with 10% of patients requiring chronic dialysis. Treatment involves immunosuppression to preserve renal function and prevent life-threatening complications. Paraneoplastic ANCA has associations with gastrointestinal, pulmonary, and haematological malignancies. Here we describe a case associated with Renal Cell Carcinoma (RCC).
Case Report: We present a 78 year old female with subacute kidney failure (eGFR 22 mL/min/1.73m²), on a background of hypertension and baseline eGFR of 55mL/min/1.73m². She had 2.2 grams proteinuria and microhaematuria, MPO 98 CU and PR3 74 CU. Imaging revealed a 10cm left RCC with local invasion and asymmetric renal differential on MAG3 (left kidney providing 62% function). She underwent nephrectomy, with non-malignant tissue demonstrating segmental glomerulosclerosis, adhesions, and disruption to Bowman’s capsule, representing resolving crescents. Postoperatively her eGFR was 15 ml/min/1.73m², with increasing ANCA titres. The risk of immunosuppression to prevent ANCA progression versus the risk of metastatic malignancy was discussed with the patient. She initially declined immunosuppression but developed extrarenal manifestations and agreed to oral azathioprine and prednisolone. Subsequent disease progression manifested as fevers and worsening arthritis. She was treated for sepsis, and immunosuppression briefly withheld. Within 24 hours her vasculitis rapidly progressed, requiring acute haemodialysis. The patient agreed to immunosuppression after extensive discussions and re-staging excluded RCC recurrence. After nearly 4 months, the patient received rituximab. Extrarenal manifestations resolved and her ANCA titres were suppressed, however she remains dialysis dependent.
Conclusions: This case highlights the risks of paraneoplastic ANCA vasculitis progressing to end stage renal failure. ANCA positivity can be associated with malignancy. Surgically curable malignancy should be pursued aggressively prior to immunosuppression.
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