Cancer risk 23% higher in people with AAV, large US study finds
Findings highlight need for more tailored cancer screening in these patients
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People with ANCA-associated vasculitis (AAV) have a significantly higher risk of developing certain blood and solid-organ cancers, compared with those without the rare autoimmune disease, according to a large real-world study in the U.S.
The study examined the medical records of more than 18,000 people with AAV and a similar number of matched individuals without the condition.
People with AAV were 23% more likely to be diagnosed with cancer over five years of follow-up, with the highest risks seen for certain blood cancers and brain cancer.
“These findings highlight the need for more tailored cancer screening and early-detection strategies in patients with AAV, and further research to check the study’s findings,” researchers wrote.
The study, “Hematologic and solid-organ malignancy risk in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis,” was published in Communications Medicine.
Most previous studies on AAV cancer risk have been small
AAV is a group of autoimmune disorders in which the immune system mistakenly attacks small blood vessels, causing inflammation and tissue damage. Symptoms vary depending on which organs are affected, most commonly the kidneys, lungs, or upper airways.
While immunosuppressive therapies have significantly increased the survival of people with AAV, “the long-term consequences of both the disease and its treatments, including possible increased cancer risk,” are increasingly recognized as important contributors to illness and death in these populations, the researchers wrote.
“Patients with chronic autoimmune diseases often experience elevated risks of hematologic [blood] and solid-organ malignancies, likely due to a combination of sustained immune activation, tissue injury, and the [cancer-inducing] potential of immunosuppressive therapies,” the researchers added.
While a previous review study reported elevated risk of certain cancers among AAV patients, most studies to date on cancer risk have been small and conducted at single centers. This limited their ability to characterize specific cancer types or assess their relative risks compared with well-matched healthy controls.
“Consequently, the true magnitude and pattern of malignancy risk in AAV remain incompletely understood, and there are no disease-specific guidelines for cancer surveillance in this population,” the team wrote.
Higher risk largely driven by blood cancers
To address this knowledge gap, a trio of researchers in the U.S. analyzed electronic health records from the TriNetX Research Network, a large global database, covering the period from December 2014 to December 2024.
The team identified records from 18,255 people with AAV and compared them with those of 18,255 individuals without the disease who were generally matched for age, sex, race, and other health conditions.
The main goal was to assess the five-year cumulative rates of blood and solid-organ cancers. Data showed that 1,045 AAV patients and 806 healthy people were diagnosed with cancer over five years of follow-up, representing a 23% higher chance of developing any type of cancer compared with matched controls.
Blood cancers largely drove this significantly higher risk, as people with AAV were slightly more than twice as likely to develop a blood cancer and 21% more likely to develop a solid-organ cancer than those without the condition.
Among blood cancers, the highest risk was observed for myelodysplastic syndromes (by about fourfold), followed by leukemia (by more than 2.5 times) and lymphoma (by twofold).
The researchers hypothesized that long-standing immune dysfunction and activation of certain immune cells in AAV could, over time, increase the likelihood of abnormal blood cell growth and cancer development.
This large [-population] study demonstrates that patients with AAV face significantly increased risks of select hematologic and solid-organ malignancies.
In terms of solid-organ tumors, people with AVV were more than twice as likely to develop brain cancer as matched controls. Other solid tumors with significantly increased risks among AAV patients included bladder cancer (by about twofold), lung cancers (by 78%), and those affecting the lip, oral cavity, and pharynx (by 78%).
Chronic inflammation in organs commonly affected by AAV, including the lungs, may create an environment that makes cancer development more likely, the team noted. In addition, some immunosuppressive treatments historically used in AAV, such as cyclophosphamide, have been linked to certain cancers, including bladder cancer.
To ensure the findings were not simply due to broader health differences or more frequent medical visits in people with AAV, the researchers also examined unrelated conditions, including varicose veins and head injuries. They found no significant differences between the AAV and control groups, suggesting that the higher cancer rates reflect a true increase in risk.
“This large [-population] study demonstrates that patients with AAV face significantly increased risks of select hematologic and solid-organ malignancies,” the researchers wrote. “The magnitude and specificity of these associations underscore the need for heightened clinical vigilance, tailored cancer screening, and mechanistic research to elucidate shared pathways between chronic autoimmunity and [cancer].”
However, they cautioned that the study was observational and based on electronic health records, meaning unmeasured factors — such as healthcare access or socioeconomic differences — could have influenced the results.
“Our findings provide a foundational evidence base for malignancy surveillance strategies and future translational studies aimed at understanding and mitigating cancer-related [illness] in AAV,” the team concluded.
