Chronic lung condition doubles infection risk in AAV patients: Study
In GPA, MPA, developing bronchiectasis may also increase risk of death
Written by |
People with ANCA-associated vasculitis (AAV) who develop bronchiectasis — a chronic lung condition marked by permanent widening of certain parts of the airways — may face a higher risk of serious infections and death, according to a new study.
The researchers found that bronchiectasis in individuals with granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) — the two most common types of AAV — was linked to more than double the risk of serious infections and slightly more than three times the risk of death within a year compared with patients without the lung condition.
The increased risks did not clearly depend on the type of initial AVV treatment patients received, the data showed. Such therapies included standard immunosuppressive therapies given to induce and maintain disease remission in people with GPA or MPA.
According to the scientists, the findings suggest that, despite concerns about increased infection risk with immunosuppressant use, these therapies may still benefit patients with bronchiectasis when combined with preventive measures to reduce infections.
“Bronchiectasis was associated with increased risks of serious infections and mortality in patients with MPA and GPA,” the researchers wrote. “However, we did not detect [a] clear interaction [with] initial treatment, suggesting that avoiding specific therapies may not be necessary.”
The study, “Association Between Bronchiectasis and Serious Infections in Microscopic Polyangiitis and Granulomatosis With Polyangiitis From the J- CANVAS,” was published in the International Journal of Rheumatic Diseases.
AAV is a group of autoimmune diseases marked by inflammation and damage to small blood vessels. Most cases are driven by self-reactive antibodies called ANCAs. These conditions can affect nearly any part of the body, but the lungs are commonly involved, causing symptoms such as shortness of breath, chest pain, wheezing, or even coughing up blood.
Bronchiectasis suspected of worsening outcomes in AAV
MPA and GPA have also been associated with bronchiectasis, a condition in which the bronchi — the major air passages of the lungs — become permanently widened. This can impair the lungs’ ability to clear mucus, allowing it to build up and increasing the risk of chronic cough and repeated lung infections.
In AAV, serious infections are major complications that can lead to death, data have shown. Researchers have therefore suspected that bronchiectasis may worsen outcomes in people with AAV. Some infections have also been linked to disease relapse.
Now, a team of scientists in Japan turned a nationwide registry to learn more. The team investigated whether bronchiectasis increased the risk of infections, disease relapse, or death in 844 adults with MPA or GPA who were newly diagnosed or experiencing a severe relapse.
Data were drawn from the Japan Collaborative Registry of ANCA-Associated Vasculitis (J-CANVAS), which collects clinical information from patients enrolled at 29 medical centers across the country. The information used in this study pertained to patient care between 2017 and 2023.
Among the patients whose records were reviewed, 614 had MPA and 230 GPA. A total of 68 (8.1%) had bronchiectasis at the start of the study. The patients were followed for up to 52 weeks, or one year, after starting treatment.
During follow-up, the researchers recorded 122 serious infections among 101 patients, as well as 85 disease relapses among 72, and 48 deaths.
Statistical analyses adjusted for potential influencing factors — such as age, disease severity, and other coexisting health conditions — showed that bronchiectasis was significantly associated with more than double the risk of serious infections and slightly more than three times the risk of death from any cause. No significant association was found between bronchiectasis and the risk of disease relapse.
Risk of death not influenced by type of treatment given
When the researchers examined whether these risks differed by initial treatment strategy, the team found no clear interaction between bronchiectasis and treatment. That result suggests that the increased risks of infection and death were not influenced by the type of therapy, according to the team.
Standard remission-induction therapy remains important in patients with bronchiectasis, accompanied by careful infection-prevention measures.
Rates of Pneumocystis pneumonia, a serious lung infection caused by the fungus Pneumocystis jirovecii, were higher in the bronchiectasis group. Importantly, per the team, data suggested that preventive treatment with the antibiotic combination trimethoprim-sulfamethoxazole (TMP/SMX) was associated with lower infection rates.
These findings suggest that standard remission-induction immunosuppressive medications that can increase infection risk — including rituximab (marketed as Rituxan and available as biosimilars) and cyclophosphamide (sold as Cytoxan and others, with generics available) — may still be appropriate for patients with bronchiectasis when infection-prevention strategies are used.
“Bronchiectasis was associated with higher risks of serious infections and mortality, and we did not observe meaningful effect modifications across initial treatment regimens,” the researchers wrote. “Standard remission-induction therapy remains important in patients with bronchiectasis, accompanied by careful infection-prevention measures, including early TMP/SMX [preventive therapy].”
Overall, according to the team, the study findings “provide guidance for clinical management of patients with bronchiectasis.”
