Granulomatosis with polyangiitis (GPA) patients often experience eye involvement, suggesting an ophthalmologist is necessary for proper disease management and better outcomes, according to Egyptian researchers.
Their study, “Relationship of ocular presentation in granulomatosis with polyangiitis to autoantibodies and disease activity,” was published in the journal Zeitschrift für Rheumatologie.
“Ocular involvement must be considered in all GPA patients and referral to an experienced ophthalmologist is mandatory for proper management and improved outcome of such a rare systemic disease,” the researchers wrote.
ANCA-associated vasculitis with GPA is characterized by necrotizing vasculitis — inflammation of blood vessel walls — of small arteries and veins. While the disease most commonly affects the kidneys and respiratory organs, other organs may also be affected.
In a subset of GPA patients, the disease affects the eye. The management of ocular involvement is tailored to the symptoms of each patient.
Because the eye is a very delicate organ, the treatment of many forms of ocular inflammation requires collaboration between a rheumatologist and an ophthalmologist.
To further characterize patients with GPA who have ocular involvement, researchers studied the disease characteristics, autoantibody profile, and disease activity of 46 GPA patients with ocular involvement who visited an ophthalmology clinic in Egypt.
Among the 46 patients, 22 were males and 24 were females; ages ranged from 32-63. The patients had experienced ocular manifestations for up to 16 years, and 32 of them had involvement of both eyes.
The majority of patients studied (40 of 46) experienced scleritis/episcleritis, which refers to inflammation of the white outer coating of the eye, known as the sclera. Also, three patients experienced eye perforation associated with scleritis.
Keratoconjunctivitis — an inflammation of the eye that involves both the cornea and the mucous membrane that covers the front of the eye — was seen in 33 patients, and 12 patients experienced proptosis, which is an abnormal protrusion or displacement of an eye.
The stroma of the eye is a delicate interlacing of fibers which plays an important role in the proper functioning of the eye.
Researchers found that 11 patients had acute infiltrative stromal keratitis (acute inflammation of the corneal stroma), 15 had peripheral ulcerative keratitis (inflammatory disease that results in the destruction of the corneal stroma), and 11 had sclerosing keratitis (inflammation of the cornea as a complication of scleritis).
Also, 11 patients experienced uveitis (inflammation of the middle layer of the eye, called uvea) and seven experienced retinal changes including vasculitis, exudates (fluid that filters from the circulatory system into areas of inflammation), and bleeding.
Most patients also experienced blurred vision, and two had vision loss. Four patients had glaucoma and two patients experienced hypotony (low pressure in the eye).
Interestingly, rheumatoid factor (RF) — a biomarker of rheumatoid arthritis — was found in 56.5% of patients and was significantly associated with uveitis. Disease activity tended to be higher in patients with uveitis.
Also, 45.7% of patients had antinuclear antibodies (ANA), which were significantly associated with keratoconjunctivitis.
“ANA and RF positivity may raise suspicion for [keratoconjunctivitis] or uveitis, respectively,” researchers wrote. “There was a remarkable association between uveitis and disease activity.”