Tailored, progressive treatment urged for ocular sarcoidosis: Study
Glucocorticoids should be first option, followed by immunosuppressants
The treatment of people with ocular sarcoidosis should be tailored based on symptom severity, starting with glucocorticoids. If patients do not respond adequately, immunosuppressants can be added, followed by biologic therapies, if necessary.
These are the conclusions of a study in Spain that retrospectively analyzed data from 65 people with ocular sarcoidosis. Biologic therapies, also known as biologics, are treatments derived from living organisms.
“We believe that a progressive treatment approach is currently the best option to treat patients,” researchers wrote. “Special consideration should be given to high-risk presentations,” they added, noting that the early use of immunosuppressants or biologic therapies may be required.
The study, “Treatment of Ocular Sarcoidosis: Analysis of 65 Patients from a Series of 342 at a University Hospital in Northern Spain,” was published in the journal International Ophthalmology.
Glucocorticoids are the mainstay treatment for ocular sarcoidosis
Sarcoidosis is defined by the formation of granulomas, which are small clusters of inflammatory immune cells that can develop in various tissues throughout the body.
The prevalence of ocular sarcoidosis, when sarcoidosis affects the eyes, varies by country, ranging from 4.5% in Finland to up to 50% in Japan. One of the most common manifestations of ocular sarcoidosis is uveitis, or inflammation of the uvea, the middle layer of the eye’s outer wall that supplies blood to the retina.
Treatments for ocular sarcoidosis mainly include agents with anti-inflammatory and immunosuppressive effects. Glucocorticoids are the mainstay treatment, but they can have serious side effects, and some patients may not respond to them.
In such cases, conventional immunosuppressants are often required, including methotrexate, mycophenolate mofetil, azathioprine, and cyclosporine.
Biologic therapies are typically the next therapeutic step, with the most commonly used being tumor necrosis factor inhibitors (TNFis). Examples of TNFis used in ocular sarcoidosis include infliximab (sold as Remicade, with biosimilars available) and adalimumab (sold as Humira, with biosimilars available). Still, adverse events may also be a concern, according to the authors.
For patients who continue to be unresponsive, Janus kinase inhibitors “are emerging as a promising treatment option,” the researchers wrote.
Most frequent eye-related manifestation was uveitis
In this study, researchers in Spain retrospectively analyzed clinical features, treatment, and clinical outcomes of 65 adults (33 men and 32 women) with ocular sarcoidosis who were seen at a single Spanish university hospital.
Patients’ mean age at diagnosis was 45.6 years. Pulmonary involvement was the most common extraocular manifestation (80%), and ocular sarcoidosis affected both eyes in 26 patients (40%).
The most frequent eye-related manifestation was uveitis (83.1% of patients), followed by lesions in the eye socket (7.7%), retinal vasculitis (6.2%), dry eye (6.2%), and scleritis (1.5%). Retinal vasculitis refers to inflammation of the retina, the light-sensitive layer at the back of the eye, and scleritis is an inflammatory condition affecting the sclera, the white outer layer of the eyeball.
Body-wide treatment, specifically oral glucocorticoids, was given to 52 patients (80%) and topical glucocorticoids to nine patients (13.8%). Four patients (6.2%) did not require any treatment.
Oral glucocorticoids were used, with a maximum dose of 75 mg/day. Into-the-vein pulses of the glucocorticoid methylprednisolone were required for 13 patients (25%).
Conventional immunosuppressants prescribed to nearly half of patients
At the study’s start, or baseline, seven patients had cystoid macular edema, or accumulation of fluid in the central part of the retina, which poses a risk factor for vision loss. Two of the seven patients with cystoid macular edema required glucocorticoids injected directly into the eye due to recurrent episodes.
Only three patients (5.8%) did not require additional conventional immunosuppressive or biologic therapy.
Conventional immunosuppressants were prescribed to 32 patients (49.2%), with methotrexate as the main choice (90.6%). About a third of these patients switched to another immunosuppressant. About half of the patients on immunosuppressants did not require biologic therapies.
Biologics, most frequently adalimumab, were administered to 17 patients (26.2%), most of whom had previously been treated with at least one immunosuppressant. Biologics were more frequently prescribed to patients with severe manifestations, including panuveitis, or inflammation of all layers of the uvea.
Cataracts, or cloudy areas in the eye’s lens, were the most common ocular complication during follow-up.
The use of immunosuppressive agents, including biologics, should be tailored to the type and severity of ocular manifestations, aiming to preserve visual function and minimize long-term complications.
There were no significant differences between the immunosuppressant and biologic groups in terms of ocular findings, treatment initiation, or outcomes, including remission rates and treatment discontinuation.
Visual acuity was also similar in the two treatment groups at diagnosis and at one month, six months, and one year of follow-up. However, patients on biologics, but not those on conventional immunosuppressants, experienced a significant improvement in best-corrected visual acuity, or the best vision with the aid of corrective lenses, after one year.
Adverse events led to treatment discontinuation in 23.5% of patients treated with immunosuppressants and 41.2% of those receiving biologic therapy, with infections as notable concerns in the latter group.
“Based on our findings, we propose a progressive treatment algorithm,” the researchers wrote, in which glucocorticoids are the first-line treatment, immunosuppressants the second line, and biologics, particularly TNFis, the third line.
“The use of immunosuppressive agents, including biologics, should be tailored to the type and severity of ocular manifestations, aiming to preserve visual function and minimize long-term complications,” the researchers concluded.
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