Key result
A 64-year-old male with a history of granulomatosis with polyangiitis developed clear cell renal cell carcinoma and a subsequent relapse of active renal vasculitis, suggesting a potential link.
Why the study?
Renal cell carcinoma association with granulomatosis with polyangiitis is rare, and whether renal cell carcinoma can trigger relapse remains an open clinical question.
Case Report (n=1)
Answers are grounded in this paper and cite their sources.
This case highlights the rare association between granulomatosis with polyangiitis and renal cell carcinoma, suggesting that RCC may trigger a relapse of GPA or that chronic immunosuppression increases cancer risk.
Granulomatosis with polyangiitis (GPA) is a potentially lethal Anti neutrophil cytoplasmic antibody (ANCA) associated with small-vessel vasculitis (AAV) involving the upper respiratory tract, lungs and renal vasculature.The presence of Renal cell carcinoma (RCC) as a manifestation of GPA is rare.RCC association with GPA has been reported in a few cases.Following the improved survival rates in patients with GPA, there is accumulating evidence to suggest an increased risk of different types of cancers.This is likely because of impaired immunosurveillance, direct oncogenicity of immunosuppressive agents, and perhaps malignant transformation of tissues undergoing chronic immune stimulation.Exposure to cyclophosphamide seems to be one of the main causes.Data suggests a standardized incidence ratio (SIR) of cancer in AAV of 1.6-2.0compared to the general population.The most prominent cancers observed were urothelial cancer, leukemias, and non-melanoma skin cancer.This association might be related to chronic immunosuppression, however data showing the simultaneous occurrence of both suggests a common pathological pathways may be responsible.We report a patient with chronic GPA previously in remission, managed for a renal mass with histopathology showing clear cell RCC.Post-surgical development of active renal vasculitis questioned whether RCC was a trigger for GPA relapse.Awareness of cancer risk in AAV requires increased measures to prevent or screen for cancer and development of carcinogenic therapies.
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Fawad et al. (2021) conducted a case report in Granulomatosis with polyangiitis and renal cell carcinoma (n=1). Immunosuppressive therapy was evaluated on Development of renal cell carcinoma and relapse of GPA. A 64-year-old male with a history of granulomatosis with polyangiitis developed clear cell renal cell carcinoma and a subsequent relapse of active renal vasculitis, suggesting a potential link.
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