(excerpt from) Clinical and Epidemiological Studies of Wegeners Granulomatosis
by Anne Knight
The incidence of Wegener’s Granulomatosis in Sweden increased three-fold between 1975 and 2001. Neither ANCA related increased awareness nor diagnostic drift from other vasculitic dIseases fully explain this dramatic increase over time, but it still remains an open question whether the observed time trends reflect a true increase in incidence.
In clinical practice the significance of C-ANCA/PR3-ANCA in patents who test positive for both these tests but do no present sufficient evidence for a vasculitis diagnosis appears limited. Patients, who are positive for C-ANCA/PR3-ANCA but do not present sufficient evidence for a systemic vasculitis diagnosis at the time of testing, appear unlikely to develop vasculitis in the future.
In absolute terms, the risk for Wegener’s Granulomatosis among close relatives of patents with the disease is low. However, a moderately increased risk among close relatives cannot be excluded. Although a genetic predisposition to Wegener’s Granulomatosis may exist, the clinical occurrence of the disease is likely to be strongly dependent on exogenous triggers.
Patients with Wegener’s granulomatons have an overall doubled nsk of developing cancer and there is a particularly increased risk of cancer of the urinary bladder, cancers of the haematopoietic system including lymphomas and squamous skin cancer.
The markedly increased risk of developing bladder cancer in patients with Wegener’s granulomatons can at least in part be attributed to the use of cyclophosphamide, with a dose-response relationship. ..
My analysis: They don’t know why there is an increase of WG in Sweden. A possibility exists that more doctors are aware of the problem so more doctors are diagnosing before death.
Also, it says that through the disease and treatment WG patient’s are at risk for bladder cancers, lymphomas, and squamous skin cancer. One of our number was just diagnosed with leukemia (from cytoxan possibly).
What else can I say? The medications are less dangerous than the disease, but unfortuately we are still in the experiemental phase.