Sarcoidosis-linked Optic Neuropathy Responds to Corticosteroids, Study Reports

A clinical study conducted over 20 years in patients with sarcoidosis, treated at three large teaching hospitals in London, found that those with inflammatory optic neuropathy, or damage to the optic nerve due to inflammation, respond to treatment with corticosteroids.

Improvement was less marked in patients with infiltrative or progressive optic neuropathies, even though the inflammatory disorder responds to therapy.

The study, “Optic neuropathy associated with systemic sarcoidosis,” published in the journal Neurology, involved 52 sarcoidosis patients, ages 20–71, treated at The Royal Free Hospital, St Thomas’ Hospital London, and James Paget University Hospital in Great Yarmouth.

Results also showed that optic neuropathy in sarcoidosis is more common in women than men, and affects people of a wide range of ages. It is also more common among people of African or Caribbean descent.

The study identified two clinical subtypes of the disease: the more common subacute optic neuropathy resembling optic neuritis, or inflammation of the optic nerve, and a more slowly progressive optic neuropathy, identified in 17% of the cases.

In 31% of the patients, the neuropathy affected both eyes, and in 36%, uveitis (or the inflammation of the pigment layer of the eye, called the uvea) occurred at the same time. The condition was painful in 27%, and two patients had optic perineuritis (or inflammation of the protective sheath around the optic nerve).

When researchers examined the patients through magnetic resonance imaging (MRI), they found that the optic nerve was involved in 75% of the cases, and more widespread inflammation in 31%.

Corticosteroid treatment was helpful for patients with inflammatory optic neuropathy, but not for those with mass lesions.

In 25% of the cases, visual symptoms — such as a reduction in central visual acuity and color vision, a reduction in the visual field, or an abnormal pupillary response — reappeared after a period of time, making it necessary to increase the treatment dose.

Sarcoidosis patients with optic neuropathy should be treated with high-dose corticosteroids, followed by slow dose reduction and close monitoring for relapses, the researchers, led by Dr. Desmond P. Kidd, a consultant neurologist at the Royal Free Hospital, concluded.

According to the researchers, “further study is required to identify precisely which treatments are best and over what period of time.”