Cancer incidence in a population‐based cohort of patients with Wegener's granulomatosis

Abstract

Wegener's granulomatosis is necrotizing granulomatous vasculitis of unknown origin, which untreated has a high mortality within the first year of onset. The introduction of corticosteroids and cyclophosphamide in the treatment has considerably improved survival rates, but past studies have indicated an increased cancer risk, including an increased risk for urinary bladder cancer. No large assessment of the general cancer occurrence in Wegener's granulomatosis has been reported. The aim of our study was to assess the general incidence of cancer in patients with Wegener's granulomatosis and to put this in relation to the risk for bladder cancer. We identified a population-based cohort of 1,065 patients with Wegener's granulomatosis in the Swedish Inpatient Register. Through linkage with the Swedish Cancer Register, we followed the cohort for cancer occurrence for up to 26 years. Standardized incidence ratios (SIR) between observed and expected numbers of cancers were used as a measure of relative risk. There was a 2-fold overall increased risk for cancer in the cohort. The increase was most pronounced for bladder cancer (SIR ‫؍‬ 4.8; 95% CI 2.6 -8.1), squamous cell skin cancer (SIR ‫؍‬ 7.3; 95% CI 4.4 -12), leukemias (SIR ‫؍‬ 5.7; 95% CI 2.3-12) and for malignant lymphomas (SIR ‫؍‬ 4.2; 95% CI 4.2-8.3). The results confirm previous indications of an increased risk for cancer of the urinary bladder but also points to increased risks for cancer at other sites.

Improved outcome in 445 patients with Wegener's granulomatosis in a German vasculitis center over four decades

“…When kidney involvement was stratified according to sex, males presented with kidney involvement more frequently than females (57.8% versus 40.1%; P Ͻ 0.001). The median DEI at diagnosis and over the whole disease course was higher in males (at diagnosis, 9 [range [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17] [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19]; P ϭ 0.001). The median time to diagnosis after the occurrence of first symptoms was 6 months (range 0-250 months) for young males and 11 months (range 0-195 months) for young females (P ϭ 0.47).…”

Retrospective Investigation of Case Studies to Determine the Concurrent Malignancy Rate Associated with Microscopic Polyangiitis

“…Several large-scale studies demonstrated that cases with AAV developed malignancies 1.6-2.4 times as often as the general population [7][8][9][10][11] . Randomized controlled trials (RCTs) and long-term follow-up (LTFU) studies in Western countries showed that malignancies were observed in 12.6% of MPA cases and 10.8% in cases of granulomatosis with polyangiitis (GPA) during the 87-month follow-up period after treatment for vasculitis 12)18-21) .…”

“…Primary vasculitis, polyarteritis nodosa, anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), and IgA vasculitis also frequently coincide with malignancies [2][3][4][5] . In cases of AAV, immunosuppressants such as cyclophosphamide are administered for treatment after diagnosis, and it is reported that malignancies develop more often in such cases than in the general population [6][7][8][9][10][11][12][13] . This may suggest that immunosuppression treatment carries an inherent risk of increased cancer rates in those with AAV.…”

Novel Strategy for the Treatment of Refractory Vasculitis Syndrome

“…The most important adverse effect was the occurrence of solid cancer observed in 6 patients, all belonging to the cyclophosphamide+etanercept group. The incidence of solid cancer has been reported to be 2-6 times higher in patients with Wegener's granulomatosis compared with gender-and age-matched general population, 4,5) and the cyclophosphamide administration has been known to increase in the incidences of bladder cancer and hematologic malignancies. However, the 6 patients consisted of 2 with mucous adenocarcinoma and 1 each with bile duct cancer, kidney cancer, breast cancer, and liposarcoma, which differed from cyclophosphamide-related tumors.…”