Clinicians Offer Best Practices to Diagnose and Treat Neurosarcoidosis

Neurosarcoidosis (NS) is challenging to diagnose and treat due to its heterogeneous presentations and the wide-ranging differential diagnoses clinicians must consider when examining a patient for the disease. Therefore, a collaborative and multidisciplinary approach to treatment is ideal for patients and care teams.

Shruti Agnihotri, MD, Associate Professor at the University of Alabama at Birmingham, and Huzaefah Syed, MD, MPH, FACR, Associate Professor and Program Director for Rheumatology at the Virginia Commonwealth University Medical Center, shared their expertise to help improve clinicians’ evaluation, diagnosis, and management of NS during the Saturday, Nov. 16, session Neurosarcoidosis: Sharing the Headache. The session will be available on-demand to all registered ACR Convergence 2024 participants after the meeting through Oct. 10, 2025, by logging into the meeting website.

According to Dr. Agnihotri, NS is often the presenting manifestation of sarcoidosis. Only 34% of patients initially present with systemic disease. Dr. Agnihotri clarified that systemic disease eventually develops in approximately 52% of patients with NS but can manifest after the neurologically presenting features. She also shared that isolated NS, without systemic disease, comprises 10–17% of all NS cases.

“We care about this, of course, because neurologic involvement is associated with increased mortality and a lot of morbidity in these patients,” she said.

Diagnosing NS isn’t always a simple task. Dr. Agnihotri offered a case study in which a patient presented with optic neuropathy, one of several potential manifestations of NS. However, clinicians must also eliminate possible differential diagnoses associated with optic neuropathy, including conditions ranging from, but not limited to, multiple sclerosis, optic gliomas, lymphoma, and non-arteritic ischemic optic neuropathy.

“Sarcoidosis is the great mimicker, so you always have to consider another diagnosis if your patient is just not improving when you think they really should be,” said Dr. Syed.

Dr. Agnihotri explained that varied, though well-defined, clinical neurological syndromes are the key to accurately diagnosing the disease. A diagnosis of NS is probable when a clinical syndrome suggests NS, alternate diagnoses (particularly infection and malignancy) are excluded, and there is histologic evidence of systemic sarcoidosis. When a patient’s clinical presentation suggests NS, but alternate diagnoses are not excluded, and there is no histologic confirmation in any tissue, Dr. Agnihotri labeled this as “possible NS.”

“We always have good confidence in the diagnosis of probable and definite NS, but possible NS or isolated NS always requires extensive workup,” she said.

Clinicians’ approaches to therapy for NS can also vary by patient. According to Dr. Agnihotri, there are no U.S. Food and Drug Administration (FDA)-approved treatments for patients with NS. Given the complex nature of the disease, Dr. Syed advocated for multidisciplinary clinics to treat sarcoidosis, which can require multiple specialists to help manage patients’ conditions.

Dr. Syed’s work at the Virginia Commonwealth University Health Multidisciplinary Sarcoidosis Clinic has allowed her to experience the benefits of the multidisciplinary care model firsthand. In addition to advantages like improved efficiency and increased communication for members of the clinical care team, multidisciplinary clinics allow patients to see multiple clinicians or have various tests done in a single visit that would normally require multiple trips to specialists, making it cost-effective as well as convenient.

“It really reduces a lot of burden for our patients,” Dr. Syed said.

She also expounded on the benefits of working at a multidisciplinary clinic that is centered on treating sarcoidosis.

“Our patients are always at the center of treating sarcoidosis or any disease,” Dr. Syed said. “But when you have a center for any disease, it really highlights and allows you to do other things, like research and teaching.”