Founded in 1962, it offers multidisciplinary care for an inflammatory illness with no known cause or cure.

Chris Thames visited several doctors, including a urologist, a pulmonologist, a dermatologist and a neurologist, trying to find out why the lymph nodes in his groin were swollen.

Biopsies, MRIs and X-rays revealed granulomas in his lymph nodes, skin and lungs, indicating he might have sarcoidosis, a rare systemic inflammatory disease with no known cause or cure. The time from first symptom to diagnosis was 10 years.

“Sarcoidosis is one of the most under-recognized diseases,” says Michelle Sharp, who co-directs the Johns Hopkins Sarcoidosis Program alongside fellow pulmonologist Edward Chen. “Nobody fully understands it yet.”

For a year, Thames and his pulmonologist monitored his breathing, which didn’t get appreciably worse, to see if additional care was needed. But Thames also had episodes of severe pain on just one side of his face and scalp, and an MRI showed a granuloma behind his eye.

The Loudon County, Virginia, resident decided it was time to see the specialists at the Johns Hopkins Sarcoidosis Center.

Johns Hopkins has one of the oldest sarcoidosis centers in the country, founded in 1962 by Carol J. Johns. It’s one of just 33 nationwide designated as a Center of Excellence by the World Association of Sarcoidosis and Other Granulomatous Disorders.

The center brings together specialists from several disciplines, including pulmonology, neurology, cardiology, dermatology and ophthalmology, who work together to provide coordinated multidisciplinary care.

The pulmonary team, with three pulmonologists (Sharp, Chen and Nancy Lin) and two nurse practitioners, gets about 500 patient referrals a year, Sharp says.

Diagnostic Difficulties

Sarcoidosis, first identified in the 1800s, causes immune cells to clump together, potentially interfering with organ function. About 90% of cases occur in the lungs, although any organ can be involved.

Adding to its mysterious nature, it can affect anyone, though it is more prevalent in women and people of either African or Scandinavian descent. It emerges most often in people between the ages of 20 and 50, or older than 60.

Patients may have a dry cough or chest pain when they take a deep breath. They may have neurologic symptoms like blurred vision, pain, weakness or loss of balance. They may have nodules on the skin around their eyes. Or they may have no symptoms at all.

Diagnosis generally requires three things, says Sharp: “You need a good clinical story, although sarcoidosis can have almost any clinical manifestation. You need a biopsy in most cases — but not all cases — showing noncaseating granulomas. And then you have to rule out everything else that can cause a granuloma. It’s a diagnosis of exclusion.”

Treatment Options

The sarcoidosis team monitors patients carefully to track potential disease progression and signs of organ damage. Treatment is usually prednisone or other medicines that suppress the immune system in order to control inflammation.

Half or more of patients with sarcoidosis go into remission without treatment, says Sharp. “They just switch their immune system back to normal and live the rest of their lives.”

Thames, 49, began treatment at Johns Hopkins nearly three years ago. He currently takes medications for inflammation and headaches, and the sarcoidosis team monitors his bone density, which can thin as a result of the steroids.

“I pretty much function normally,” he says, though he does get short of breath during strenuous exercise, and sometimes gets headaches. 

Members of his sarcoidosis team, he says, “all talk to each other and reach out to my other doctors as needed. That’s how they are able to come up with the right medications in the right doses.”