ANCA-associated vasculitis tied to risk of heart, brain vascular events

Findings underscore importance of early screening, monitoring

Andrea Lobo, PhD avatar

by Andrea Lobo, PhD |

Share this article:

Share article via email
A risk dial has its indicator at the very top of 'HIGH.'

People with ANCA-associated vasculitis (AAV) have an 83% higher risk of developing cardiovascular and cerebrovascular diseases (CCVD) relative to those without vasculitis, according to a review study that also showed the risk was significantly higher in the first two years after an AAV diagnosis.

“These findings underline the importance of early screening and close monitoring for cardio-cerebrovascular complications among AAV population, especially during the early period after diagnosis,” the researchers wrote.

The study, “Cardiovascular and cerebrovascular outcomes in anti-neutrophil cytoplasmic antibody-associated vasculitis: A systematic review with meta-analysis,” was published in Autoimmunity Reviews.

AAV is a group of autoimmune diseases marked by inflammation and damage to small blood vessels. The inflammation is typically caused by ANCAs, or self-reactive antibodies that bind and overly activate neutrophils, a type of immune cell, that launch an attack against the cells that line blood vessels.

The disease can damage several organs, most often the kidneys and lungs, leading to a wide range of symptoms. AAV has also been increasingly associated with a higher long-term risk for cardiovascular and cerebrovascular diseases as a result of more available treatments and patients living longer.

Recommended Reading
A doctor gestures with one hand while talking with a patient who sits on the edge of an examination table.

Asthma drug may trigger rare ANCA-associated vasculitis

AAV and CCVD risk

Cardiovascular conditions involve the heart and blood vessels, while cerebrovascular diseases affect the blood vessels and blood flow in the brain. To quantify their risk in AAV, researchers in China systematically reviewed studies published up to January that describe those risks in AAV patients relative to those without vasculitis, who were used as controls.

Fourteen studies that covered 20,096 AAV patients were included in the meta-analysis. All were retrospective studies published between 2009 and 2023, and most were from Europe. For 16,270 patients with an available AAV type data, granulomatosis with polyangiitis (GPA) was the most frequent form (90.1%), followed by microscopic polyangiitis (MPA; 6.4%), and eosinophilic GPA (EGPA; 3.5%).

During a mean follow-up of 3.4 to 9.5 years, 5,757 cardiovascular and cerebrovascular events were reported. This corresponded to a rate of 15.8 to 59.9 events per 1,000 person-years, reflecting the number of cases reported in 1,000 patients followed during a year.

Types of AAV, CCVD, and risk

People with AAV had a significantly higher risk of developing CCVDs, by 83%, relative to those without vasculitis, pooled data showed. Particularly, AAV patients were 48% more likely to develop coronary artery disease, a condition marked by reduced blood flow in the arteries that deliver blood to the heart, and had a 56% higher risk of stroke, which happens when there’s a loss of blood flow to an area of the brain.

GPA patients had an 87% higher risk for CCVD, while the risk was almost three times higher in MPA patients.

Regarding types of CCVD, people with AAV had a 97% greater risk of a stroke, a 72% higher risk of heart failure, and a 67% increased risk of heart attack, which occurs when when there’s reduced blood flow to the heart’s muscle. There was also a trend toward a higher risk for chest pain and discomfort and for ischemic stroke, or that caused by a blockage of a brain artery, among people with AAV.

CCVD risk was significantly higher within the first two years after an AAV diagnosis than during the whole follow-up period (2.23 vs. 1.48 times), further analyses indicated. Moreover, the type of study setting (nationwide or not) and AAV type, along with study quality, were significantly associated with variations in CCVD outcomes between studies.

“The present meta-analysis provided comprehensive insights into the risk of overall, type-specific CCVD as well as heart failure in AAV population,” the researchers wrote. “We observed people with AAV had a substantial increase in risks of CCVD in general accompanied by varying degrees of most common specific types of CCVD.”

Possible reasons proposed for increased CCVD risk included a higher prevalence of other cardiovascular risk factors among AAV patients, such as diabetes and high blood pressure, chronic inflammation that contributes to fat buildup in the artery walls, and using certain medications, particularly high-dose glucocorticoids.

The findings “emphasize the importance of effective strategies for screening, assessment, and intervention of cardiovascular complications in AAV patients, particularly those in the early stage,” the researchers wrote.