SAN FRANCISCO (FRONTLINE MEDICAL NEWS) Rituximab is, for now, the first-line option for Sjögren’s syndrome patients with systemic symptoms severe enough to require biologic therapy, according to new systemic treatment guidelines from the Sjögren’s Syndrome Foundation.

In general, tumor necrosis factor inhibitors are out because of the risk of lymphoma; Sjögren’s patients are already at risk for the disease.

The foundation has also released guidelines for the management of dry eyes and prevention of cavities in Sjögren’s. Thirteen more guidelines are in the works to tackle neurologic complications, lung disease, lymphoma, and other issues. The ocular guidelines have been published; the systemic and cavity ones will be soon.

“We are really excited about this. These are the first U.S. clinical practice guidelines for Sjögren’s. It will be a game changer,” said Katherine Hammitt, the Sjögren’s Syndrome Foundation’s vice president of medical and scientific affairs.

Topical fluoride should be used in all patients with dry mouth to prevent cavities, along with measures or medications to increase saliva. A comprehensive corneal exam is the first step for dry eyes to determine if they’re due to a lack of tears or abnormal oil secretion by eyelid glands, which causes tears to evaporate too soon. Treatment proceeds according to the findings.

The recommendations are based on a literature review and the consensus of about 100 Sjögren’s experts. The guidelines address what the experts considered to be the most pressing problems.

The foundation launched the efforts after fielding calls from patients reporting that doctors didn’t understand the disease; didn’t take it seriously; or said there was nothing that could be done. On the flip side, physicians were calling in about problem patients.

“So about 5 years ago, we launched an initiative to address the issues.” With current options, “to tell patients that they don’t need treatment or that nothing can be done is, in my view as a rheumatologist, malpractice,” said presenting author Dr. Frederick Vivino, chief of rheumatology at Penn Presbyterian Medical Center, a University of Pennsylvania teaching hospital in Philadelphia. He is also director of the Penn Sjögren’s Syndrome Center.

Rituximab (Rituxan) isn’t approved for Sjögren’s, but “clinicians are using it quite frequently because it’s really the only [biologic] out there” for the disease. “We’ve seen moderate improvement” of organ involvement, vasculitis, neuropathy, and other extraglandular problems, but “it hasn’t knocked our socks off. We need something better than rituximab” for the sickest patients, Ms. Hammitt said at the annual meeting of the American College of Rheumatology. The guidelines recommend hydroxychloroquine as the first step for inflammatory musculoskeletal pain, followed, as needed, by methotrexate, steroids, and other options. Azathioprine is a good option for recalcitrant musculoskeletal pain, as well as organ involvement. Meanwhile, aerobic exercise is important to help with fatigue.

A baseline corneal exam is “the most important recommendation” for eye patients, Dr. Vivino said.

A stepwise treatment algorithm based on the nature and severity of the problem comes next, and can included tear supplementation and stabilization; control of inflammation of the lacrimal glands and ocular surface; systemic therapy with secretagogues; tear preservation measures; and eyelid surgery. Salivary deficiency causes cavities in Sjögren’s. “If the patient has dry mouth, they need to be given topical fluoride. Either encourage them to ask their dentist, or, as I would do, just prescribe it, and they should use it on a regular basis. The group also felt that giving medications to stimulate saliva flow,” like pilocarpine or cevimeline, “would likely help prevent caries, as well,” Dr. Vivino said.

Sugar-free lozenges or chewing gum can also help with saliva flow. The group gave a weak recommendation for chlorhexidine varnishes, gels, and rinses, and a moderate one for nonfluoride remineralizing agents.

The work was supported by the Sjögren’s Syndrome Foundation, with no pharmaceutical industry funding. Some of the authors have financial ties to numerous pharmaceutical companies.