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ANCA-associated vasculitis and malignancy: current evidence for cause and consequence relationships
Alfred Mahr1, Caroline Heijl, Guillaume Le Guenno
1Department of Internal Medicine, University Hospital Saint-Louis, Paris, France. alfred.mahr@sls.aphp.fr
Best Practice & Research. Clinical Rheumatology
|March 20, 2013
Summary
Patients with anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) face an increased cancer risk, particularly urinary tract, leukaemia, and skin cancers. Cancer may also rarely trigger AAV, necessitating improved cancer screening and less carcinogenic therapies.
Area of Science:
- Rheumatology
- Oncology
- Immunology
Background:
- Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) is an autoimmune disease linked to increased cancer risk.
- Factors contributing to this risk include impaired immunosurveillance, immunosuppressive drug oncogenicity, and chronic immune stimulation.
- Specific AAV types like granulomatosis with polyangiitis (GPA) may carry a higher risk than microscopic polyangiitis (MPA).
Purpose of the Study:
- To review the current understanding of the bidirectional relationship between cancer and AAV.
- To identify specific cancer types associated with AAV.
- To discuss the role of therapeutic agents and potential cancer triggers in AAV.
Main Methods:
- Literature review of studies investigating cancer incidence and risk factors in AAV patients.
- Analysis of cancer types observed in AAV cohorts.
- Evaluation of the impact of immunosuppressive therapies on cancer development.
Main Results:
- A standardized incidence ratio of cancer in AAV ranges from 1.6-2.0 compared to the general population.
- Prominent cancers include urinary tract cancer, leukemia, and non-melanoma skin cancer.
- Cyclophosphamide is identified as a significant contributor to cancer risk due to its carcinogenic properties.
Conclusions:
- Increased cancer surveillance and development of less carcinogenic treatments are crucial for AAV management.
- While cancer can potentially trigger AAV, the attributable fraction appears small and data remain inconsistent.
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