Eye symptoms seen in one-third of GPA patients in Italian study

Symptoms can sometimes be present in the eyes before appearing in other areas

Margarida Maia, PhD avatar

by Margarida Maia, PhD |

Share this article:

Share article via email
An illustration shows an eye magnified in a telescope lens.

More than one-third of people with granulomatosis with polyangiitis (GPA), a type of ANCA-associated vasculitis, may experience eye symptoms, a study in Italy has found.

In many cases, eye symptoms were present from the onset of disease, sometimes even preceding more specific symptoms affecting other body parts.

“Patients should be diagnosed as quickly as possible to avoid disease progression to severe or even life-threatening stages. … Increased awareness, early diagnosis, and multi-specialty collaboration are critical in achieving a favorable outcome of GPA,” researchers wrote.

The study, “The eye is a common site of granulomatosis with polyangiitis. A collaborative study,” was published in BMC Ophthalmology.

Recommended Reading
banner image for Allison Ross' column

Life milestones look different when you have vasculitis

 Eye symptoms have been reported in 13% to 60% of GPA patients

GPA is a type of AAV caused by self-reactive antibodies called ANCAs that most often target proteinase 3 (PR3), a protein found at the surface of a type of immune cell called neutrophils.

This abnormal immune attack leads to the formation of granulomas, or immune cell masses, causing inflammation and damage to small blood vessels, mainly those in the lungs, kidneys, and upper respiratory tract. This type of AAV is typically associated with a higher risk of relapse.

Ocular, or eye, manifestations of GPA “have been reported in a limited number of studies and with largely variable frequency,” ranging from 13% to 60% of patients, the researchers wrote.

These eye symptoms can appear at variable times during the course of the disease, including its onset, and can involve every eye structure.

To know more about the features of ocular manifestations in GPA, a team led by researchers in Italy looked back at the clinical data of 63 Caucasian GPA patients (36 women and 27 men), who had been admitted to a single Italian tertiary AAV referral center from May 2005 to December 2019.

Patients’ average age was 53.5 years, ranging from 29 to 77 years, and all underwent comprehensive ophthalmologic examination in addition to clinical assessment at diagnosis and during follow-up.

Eye symptoms were present in 22 patients (34.9%), including 13 women and nine men with a mean age of 55 years (range of 35-73 years). In most of them (68.2%), only one eye was affected.

Patients should be diagnosed as quickly as possible to avoid disease progression to severe or even life-threatening stages.

Most common eye symptom was inflammation of the white part of the eye

The most common eye symptom was inflammation of the white part of the eye, called the sclera (40.9%). This was followed by disease of the eye sockets (27.3%), and inflammation of the tissue between the sclera and the membrane that lines the inside of the eyelid (13.6%).

Nine of the 22 patients (40.9%) had both ocular and extraocular manifestations at disease onset. Eye symptoms appeared after other body symptoms in eight patients (36.3%), and first in four patients (18.2%).

One patient (4.5%) had an isolated mass in the right eye socket and mild non-specific symptoms but did not develop disease-specific extraocular symptoms at any time during the course of disease.

Extraocular manifestations most often involved the ears, nose, and throat (77%), followed by the kidneys (59%), lungs (50%), nervous system (9%), and cardiovascular system (4.5%).

A combination of glucocorticoids plus cyclophosphamide was most commonly used to induce remission in these GPA patients with eye symptoms. Induction treatment lasted from four to seven months and was thereafter replaced by maintenance treatment, which continued for up to three years.

The most common treatment to maintain remission was a combination of glucocorticoids plus rituximab, an antibody that reduces the number of antibody-producing B-cells.

Recommended Reading
A tired woman sleeps sitting up at her desk.

Most AAV patients have fatigue severe enough to be a disorder

Most of the patients achieved long-term remission after treatment

More than three-quarters of patients (77.3%) achieved long-lasting remission of eye symptoms, while 63.6% achieved remission for extraocular manifestations. Seven (31.8%) patients experienced up to three relapses, because they stopped treatment or did not fully adhere to it. None of the relapses affected the eye exclusively.

Overall, 31 of the 44 eyes (70.4%) in the 22 patients were considered to have a good visual outcome at the last follow-up, but the five eyes with poorest visual acuity at first evaluation remained unchanged throughout follow-up.

“Based on the data obtained in this [patient group], we recommend an ophthalmological examination for all GPA patients to allow the early recognition of ocular injury and thus the prevention of the often severe consequences of a late diagnosis,” the researchers wrote.

“We also highlight the recent advances in the therapeutic landscape marked by the introduction of B-cell-depleting monoclonal antibodies and other biologic agents, which have remarkably improved the outcome of GPA patients, including those with relapsing or refractory disease,” they added.