{"id":452048,"date":"2026-05-21T00:16:07","date_gmt":"2026-05-21T00:16:07","guid":{"rendered":"https:\/\/www.newsbeep.com\/il\/452048\/"},"modified":"2026-05-21T00:16:07","modified_gmt":"2026-05-21T00:16:07","slug":"more-severe-lung-disease-tied-to-worse-outcomes-in-aav-ild","status":"publish","type":"post","link":"https:\/\/www.newsbeep.com\/il\/452048\/","title":{"rendered":"More severe lung disease tied to worse outcomes in AAV-ILD"},"content":{"rendered":"

Among people with ANCA-associated vasculitis<\/a>\u00a0(AAV) and interstitial lung disease (ILD), severe lung scarring and poorer lung function at the time of ILD diagnosis\u00a0are tied to a higher risk of lung disease progression, respiratory failure, and death, a European study shows.<\/p>\n

ILD is a condition marked by inflammation and progressive scarring of lung tissue, and a common lung manifestation of AAV.<\/p>\n

Data also showed that treatment with rituximab<\/a> (sold as Rituxan, with biosimilars available) was associated with the greatest improvements in lung function, especially in patients with more severe lung scarring.<\/p>\n

This study highlights \u201cthe need for early identi\ufb01cation and individualized treatment in ILD associated with AAV,\u201d and \u201cidentified meaningful clinical, radiologic, and prognostic features,\u201d researchers wrote.<\/p>\n

The study, \u201cInterstitial Lung Disease in Antineutrophil Cytoplasmic Antibody\u2013Associated Vasculitis: A European Multicenter Study<\/a>,\u201d was published in Arthritis & Rheumatology.<\/p>\n

Recommended Reading<\/p>\n

\"An<\/p>\n

Cough, shortness of breath most common symptoms at ILD diagnosis<\/p>\n

AAV is a group of diseases typically\u00a0caused<\/a>\u00a0by self-reactive antibodies, called ANCAs, that overly activate neutrophils, a type of immune cell. This results in inflammation and damage to small blood vessels in several organs, most frequently the lungs and kidneys.<\/p>\n

ILD is a serious lung disease that can occur in association with AAV (AAV-ILD), or as an isolated condition with the presence of ANCAs, but no vasculitis, or blood vessel inflammation (ANCA-ILD).<\/p>\n

To learn more about the features and outcomes of AAV-ILD and ANCA-ILD, a team of researchers retrospectively analyzed data from 123 people with AAV-ILD and 39 people with ANCA-ILD who were seen at six centers across four European countries.<\/p>\n

More than half of the participants (57%) were men and they were followed for a median of 4.2 years. AAV patients had a median age of 72 years at diagnosis, and in terms of AAV type<\/a>, most had microscopic polyangiitis (61%).<\/p>\n

In both groups, ANCAs targeting the myeloperoxidase protein were detected in about 85% of patients, while the remaining patients had ANCAs against the proteinase 3 protein. Median levels of both types of ANCAs at diagnosis were significantly higher among those with AAV.<\/p>\n

Among all participants, the most common symptoms<\/a> at ILD diagnosis included cough (85%) and shortness of breath (75%). On chest CT scans, usual interstitial pneumonia (UIP) was the most common radiological pattern (56%), followed by nonspecific interstitial pneumonia (NSIP, 17%). UIP is marked by more permanent lung scarring, or fibrosis, and tends to have a worse prognosis, while NSIP often has more inflammation than scarring.<\/p>\n

ANCA-ILD patients were significantly more likely to have cough (81% vs. 57%), shortness of breath (81% vs. 48%), UIP (69% vs. 51%), and more severe fibrosis than those with AAV-ILD. In contrast, diffuse alveolar hemorrhage, a form of lung bleeding, was present exclusively in the AAV-ILD group (18%).<\/p>\n

ANCA-ILD patients also had poorer lung function, with significantly lower total lung capacity, or the maximum volume of air the lungs can hold after a deep breath (63% vs. 90%).<\/p>\n

Recommended Reading<\/p>\n

\"A<\/p>\n

53% of patients experienced lung fibrosis progression during follow-up<\/p>\n

During follow-up, participants with UIP and those with NSIP showed a significantly greater annual decline in forced vital capacity (FVC) percentage compared with those with other disease patterns. FVC percentage is a lung function measure that indicates how much air a person forcefully exhales after a deep breath relative to the predicted average for someone with the same age, sex, and other factors.<\/p>\n

Overall, 96% of patients received immunosuppressive treatment<\/a>, most commonly glucocorticoids, but rates of use, including glucocorticoids and rituximab, were significantly higher in the AAV-ILD group.<\/p>\n

Treatment with rituximab led to the greatest annual FVC percentage gain during follow-up, particularly in participants with UIP. The therapy was associated with a trend toward improved FVC percentage in both AAV-ILD and ANCA-ILD groups.<\/p>\n

These findings highlight the urgent need for early diagnosis, standardized radiologic evaluation, and a multidisciplinary strategy to optimize care and improve prognosis in this underrecognized, high-risk patient population.<\/p>\n

Over follow-up, 53% of the patients experienced lung fibrosis progression on CT scans (radiologic progression), while 48% died, and 19% developed respiratory failure, which occurred significantly earlier in ANCA-ILD patients.<\/p>\n

\u201cSurvival did not differ between the AAV-ILD and ANCA-ILD groups,\u201d the researchers wrote. The most common cause of death was lung disease progression caused by an infection or ILD worsening.<\/p>\n

Statistical analyses adjusted for potential influencing factors showed that younger age and more severe lung fibrosis at ILD diagnosis were significantly associated with a higher risk of radiologic progression.<\/p>\n

Also, lower FVC% at ILD diagnosis and more severe fibrosis were independent risk factors of respiratory failure, while older age and lower FVC% at ILD diagnosis were significantly associated with a higher risk of death.<\/p>\n

\u201cThese findings highlight the urgent need for early diagnosis, standardized radiologic evaluation, and a multidisciplinary strategy to optimize care and improve prognosis in this underrecognized, high-risk patient population,\u201d the researchers wrote.<\/p>\n","protected":false},"excerpt":{"rendered":"Among people with ANCA-associated vasculitis\u00a0(AAV) and interstitial lung disease (ILD), severe lung scarring and poorer lung function at…\n","protected":false},"author":2,"featured_media":452049,"comment_status":"","ping_status":"","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[10],"tags":[163,85,46,211205,155433],"class_list":["post-452048","post","type-post","status-publish","format-standard","has-post-thumbnail","category-health","tag-health","tag-il","tag-israel","tag-lung-involvement","tag-outcomes"],"_links":{"self":[{"href":"https:\/\/www.newsbeep.com\/il\/wp-json\/wp\/v2\/posts\/452048","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.newsbeep.com\/il\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/www.newsbeep.com\/il\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/www.newsbeep.com\/il\/wp-json\/wp\/v2\/users\/2"}],"replies":[{"embeddable":true,"href":"https:\/\/www.newsbeep.com\/il\/wp-json\/wp\/v2\/comments?post=452048"}],"version-history":[{"count":0,"href":"https:\/\/www.newsbeep.com\/il\/wp-json\/wp\/v2\/posts\/452048\/revisions"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/www.newsbeep.com\/il\/wp-json\/wp\/v2\/media\/452049"}],"wp:attachment":[{"href":"https:\/\/www.newsbeep.com\/il\/wp-json\/wp\/v2\/media?parent=452048"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.newsbeep.com\/il\/wp-json\/wp\/v2\/categories?post=452048"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.newsbeep.com\/il\/wp-json\/wp\/v2\/tags?post=452048"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}