Eye symptoms linked to AAV most common in GPA patients: Study

Eye-related issues affect over one-third of those with GPA, per analysis

Andrea Lobo, PhD avatar

by Andrea Lobo, PhD |

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A human eye is seen in the lens of a giant telescope as a person looks at the stars.

Eye symptoms related to ANCA-associated vasculitis (AAV) are most common among those with granulomatosis with polyangiitis (GPA), affecting more than one-third of these patients, a new study from China showed.

“These insights collectively underscore the importance of consistent ocular [eye-related] assessments for AAV patients, enabling the monitoring of disease progression and complications,” the researchers wrote, noting that eye-related symptoms overall are “a prevalent complication across all forms of AAV.”

The researchers also identified potential risk factors for eye involvement in AAV and used these to create a predictive machine learning model. Machine learning is a form of artificial intelligence that uses algorithms to analyze data, learn from its analyses, and then make a prediction about something.

“The development of a machine learning model to predict … eye involvement represents a promising tool for early identification and management of ocular complications in AAV,” the researchers wrote.

The study, “Ocular manifestations in ANCA‑associated vasculitis: a comprehensive analysis from Chinese medical centers,” was published in Clinical Rheumatology.

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Researchers in China investigate AAV-related eye symptoms

AAV is an autoimmune disease marked by inflammation and damage to small blood vessels. Most cases are associated with the production of self-reactive antibodies, called ANCAs, that ultimately drive the damaging inflammation in blood vessels.

Several organs and systems can be affected in AAV, including the lungs, kidneys, nervous system, and skin, as well as the ears, nose, and throat. Eye symptoms may also occur, mainly among those with GPA, a common AAV type. Indeed, such manifestations have been reported in 26% to 50% of people with GPA.

Now, to increase the knowledge of AAV-related eye symptoms, a team of researchers in China retrospectively analyzed data from 533 adults, half of whom were women. The patients were diagnosed with AAV between January 2016 and November 2023. All were recruited from two Chinese centers: one in the north and another in the south.

The patients’ average ages were 55.6 for men, and 58.5 for women. GPA and microscopic polyangiitis (MPA), another common AAV type, were the most common diagnoses, with each affecting about 40% of patients. Eosinophilic GPA or EGPA, the rarest type, was present in 18.6%. In seven patients (1.3%), the type of AAV was not determined.

ANCAs against the proteinase 3 enzyme, one of the two most common targets of AAV-driving antibodies, were mostly detected in GPA patients (66.7 %), while ANCAs against the other common target, myeloperoxidase, were more common in MPA patients (82%). Most patients with EGPA (68.7%) did not have any ANCAs.

Eye-related manifestations — symptoms affecting either the eyes or eye sockets — occurred in 20.6% of the patients, and were most common in those with GPA (36.7%), followed by those with EGPA (18.2%) and MPA (7.4%).

A total of 77 patients had eye involvement, affecting any of several eye layers, including the light-sensitive retina. Symptoms of retina involvement often lead to impaired vision, while inflammation in other eye layers typically leads to red eye, eye pain, reduced vision, and light sensitivity.

Eye socket-related abnormalities occurred in 38 patients, most often seen as protruding eyes or restricted eye movement.

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Findings suggest need for more eye screenings for patients

Inflammation in non-retinal eye layers and eye socket issues were significantly more common among GPA patients (16.7% each) than in those with other AAV types. Retinal involvement, conversely, was similar across AAV types.

Involvement of the optic nerve, which carries signals between the eye and the brain, was significantly more frequent among GPA and EGPA patients than in MPA patients.

Blood parameters indicated that patients with retinal involvement had a significantly higher percentage of eosinophils, or immune cells that boost inflammation compared with those without eye symptoms. These patients also had a lower CD4/CD8 T-cell ratio, which reflects increased immune activation, and lower counts of immune B-cells, than did their counterparts without such symptoms.

Also, a significantly higher proportion of patients with retinal involvement had elevated levels of C-reactive protein, a marker of inflammation (81.5% vs. 52.3%).

A machine learning model using all of these factors, except B-cell counts, was able to discriminate between patients with and without retinal involvement with an accuracy of up to 84.3%.

To distinguish patients with eye involvement from those without, the team developed another model using these and other factors, including the presence of anti-PR3 ANCAs. That model was able to make such a distinction with an accuracy of up to 70.1%.

“Predictive models developed through machine learning offer promising tools for early intervention and tailored patient care,” the researchers wrote.

This study provides new insights into the ocular manifestations of ANCA-associated vasculitis across different subtypes, revealing a notable prevalence of ocular complications. … The findings could guide more systematic ocular screenings in ANCA-associated vasculitis patients and influence treatment protocols.

Patients were mainly treated with systemic, or whole-body, medications, primarily with the corticosteroid prednisone (83.1%). Many patients also received localized eye treatment, including eyedrops, steroids, or laser surgery to destroy abnormal blood vessels in the retina.

“Managing various forms of ocular inflammation often requires a collaborative approach between rheumatologists and ophthalmologists,” the researchers wrote.

According to the team, their work shows the impact of eye-related symptoms in AAV and its types.

“This study provides new insights into the ocular manifestations of ANCA-associated vasculitis across different subtypes, revealing a notable prevalence of ocular complications,” they wrote. “The findings could guide more systematic ocular screenings in ANCA-associated vasculitis patients and influence treatment protocols.”