PARIS—Patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis have substantial knowledge deficits about potential treatment-related adverse effects, according to data from a British survey.

The survey was completed by 378 patients who belong to Vasculitis UK, a 700-member nationwide support group. The survey was developed by a multidisciplinary group involving nephrologists, rheumatologists, neurologists, otorhinolaryngologists, ophthalmologists, and patient representatives. 

Mike Venning, MD, a consultant nephrologist at Manchester Royal Infirmary and the University Hospital of South Manchester, presented the findings at the 49th Congress of the European Renal Association-European Dialysis and Transplant Association. Nina Brown, MD, a vasculitis research fellow at Manchester Royal Infirmary, was the principal investigator.

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Despite advances in therapy, ANCA-associated vasculitis has a five-year mortality rate of 25% and is associated with major morbidities. The major causes of death are no longer active vasculitis but instead include cardiovascular disease, malignancy, and infection, all of which may be related to the use of immunosuppressive therapy. About 60% to 80% of patients with ANCA-associated vasculitis will have kidney involvement, Dr. Venning said.

The study aimed to assess patient reported knowledge of side effects with vasculitis therapy as well as uptake of screening and prophylactic measures to reduce these complications and side effects. Most survey respondents had primary systemic vasculitis.
Results revealed that 96% of patients had reportedly been treated with oral steroids, 75% with cyclophosphamide, and 69% with azathioprine. However, only 27% knew that potential side effects of such treatments included osteopenia/osteoporosis. A similar percentage was aware of other steroid risks including poor healing and bruising. 

While infection accounts for about half of all deaths during induction therapy over 12 months in newly diagnosed patients, only 10.5% of respondents were aware of their increased infection risk.

Several studies have reported about a twofold increased cancer risk but only 16% of patients in this survey were aware of their heightened cancer risk.

The study found variable results on uptake on prophylaxis or screening measures that may have been recommended by patients’ health care providers.
The uptake of strategies to decrease steroid-related complications was generally good.   Overall, 63% of patients had undergone bone scanning, and 59% had received calcium.

Also, 85% of patients reported having annual flu vaccinations, and about 75% had steroid prophylaxis involving gastroprotection.  

Only 40% of patients had undergone vaccination against Pneumococcus or Haemophilus bacteria, which can cause serious pulmonary infections.

Eighteen percent and 13% of patients, respectively, were aware of the need to limit sun exposure and monitor agents like azathioprine or cyclophosphamide due to a potential for increased malignancy risk.

Dr. Venning, who is also the North of England Lead for the UK Vasculitis Registry and UK Vasculitis Rare Disease Working Group, said that the survey sample size was impressive given that vasculitis is a rare disease. However, he emphasized that the survey results probably represent a “best-case scenario.” Patients who participate in surveys are more likely to be motivated than patients who decline to participate. Also, respondents were members of a patient support group, which “by definition” is a more motivated cohort.

He added that he hopes that “vasculitis patients will, wherever cared for, eventually undergo routine comprehensive clinical assessment which will allow clinicians to identify, monitor, and pro-actively manage therapy-related complications and also develop better sources of patient information and self-management education.  “Vasculitis is about 20 years behind diabetes in terms of comprehensive management of outcomes,” he said.  “Diabetes clinics are highly structured and offer screening for eye disease, neuropathy, foot problems, and so on, and we’d like to offer the same comprehensive care to vasculitis patients.”