A 74 year old Caucasian male was diagnosed with PR3 ANCA positive GPA in 2010 when he presented with acute kidney injury and had biopsy proven pauci-immune glomerulonephritis. He achieved disease remission with remission induction therapy with glucocorticoids and Cyclophosphamide. He experienced a renal relapse in 2013 and achieved disease remission with glucocorticoids and rituximab. He presented in March 2016 with abdominal pain and was found to have a right renal mass and a paraspinal mass. Ultrasound guided biopsy of the renal mass revealed clear cell renal cell carcinoma. Four days after the biopsy, he was admitted with fatigue, night sweats and arthralgias. Laboratory data were notable for a rise in serum creatinine, proteinuria and hematuria. His inflammatory markers were elevated and his PR3 ANCA titer had increased by two fold. His B cells showed reconstitution. He underwent biopsy of his paraspinal mass which revealed necrotizing granuloma with no evidence of infection or malignancy. He was treated with glucocorticoids and rituximab. His serum creatinine improved and his hematuria and proteinuria resolved. His inflammatory markers normalized and his PR3 ANCA titer became negative. His repeat CT scan revealed improvement in the size of the paraspinal mass (Figure 1 and 2). He subsequently underwent cryoablation of his right renal mass in October 2016.
In our patient, he was at high risk of disease relapse with doubling of PR3 ANCA titer and B cell reconstitution and he was at high risk of malignancy and therefore, a decision was made to obtain tissue biopsy for both his renal and paraspinal mass. His GPA responded well to rituximab and glucocorticoids and his renal cell carcinoma were treated with cryoablation. It is interesting to speculate that his renal cell carcinoma might have been a trigger for relapse of his GPA.
Key words: Granulomatosis with Polyangiitis, Paraspinal mass, Biopsy, immunosuppression.
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