Biologics and Sjögren's syndrome: Seek payer approval for disease manifestations
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The ability to identify the features of Sjögren’s syndrome, and to seek payer approval for specific manifestations of the disease, is of paramount importance for the rheumatologist, according to a presenter at the 2021 Interdisciplinary Autoimmune Summit.
During the virtual meeting, Robert I. Fox, MD, PhD, head of rheumatology at Scripps Memorial Hospital and Research Foundation, said that more than 50% of patients with Sjögren’s syndrome are misdiagnosed.
“Although lupus and Sjögren’s syndrome share many features, it’s easiest to think of lupus as an immune complex disease and Sjögren’s as a lymphocyte aggressive disorder,” Fox explained. “The antibody to SSA is not a criterion for lupus. Over 50% of Sjögren’s syndrome patients are misdiagnosed. They end up in pulmonary and hematology and renal clinics, and ... as soon as they get in that specialty, they’ll be lost as Sjögren’s patients and many of their important issues such as rampant dental decay will not be adequately addressed.”
Three common complaints
When it comes to diagnosis, Fox cautioned attendees to be vigilant when patients present with oral and ocular signs and symptoms, as well as fatigue. Oral complaints include a feeling of dry mouth, recurrent swollen salivary glands and frequent use of oral liquids to aid in swallowing. Cevimeline and pilocarpine are both FDA-approved for the treatment of dry mouth.
As for ocular symptoms, dry eyes are common, and patients can develop corneal abrasions and uveitis, which require swift and proper treatment. Fox also alerted attendees to beware of ocular herpes zoster and herpetic keratitis. FDA-approved options for dry eyes include topical cyclosporin and topical lifitegrast (Xiidra, Novartis).
Extraglandular manifestations also occur in Sjogren’s syndrome, including kidney infiltrates, which are most commonly interstitial nephritis. “If you see a glomerulonephritis in Sjögren’s, think of mixed cryoglobulinemia, think of amyloid,” Fox said.
Neurologic manifestations vary among patients with Sjögren’s syndrome, but neuromyelitis optica and length-independent neuropathy are typical, as well as mononeuritis multiplex. There is also a much higher incidence of lymphoma — mucosa-associated lymphoid tissue or diffuse — among patients with Sjogren’s syndrome, compared with patients with lupus, Fox said.
Differentiating Sjögren’s from systemic lupus erythematosus
Additional differences between Sjögren’s and lupus include skin manifestations, which are typically mixed cryoglobulinemia, subacute lupus and skin dryness, also called xeroderma, in Sjögren’s. “Lung manifestations are also different,” Fox said. “We see more interstitial pneumonitis, particularly lymphocytic interstitial pneumonitis rather than the pleurisy you normally see in lupus.”
One of the most important points Fox stressed regarding extraglandular manifestations is that they do, indeed, respond to biologics, despite lack of an FDA-approved therapy specifically for Sjögren’s syndrome.
“The problem for the rheumatologist is that these biologic agents may not be covered by insurance because they’re not officially approved for Sjögren’s syndrome, but they may be approved for interstitial pneumonitis, for [lymphoid interstitial pneumonia] ... they may be covered for mixed cryos,” Fox said. “There are manifestations, like hemolytic anemia, for which we can use rituximab rather than just putting it down as Sjögren’s syndrome. These biologics can work, but you must remember they work in certain situations and you must not be misled by false statements that there are no biologics for Sjögren’s syndrome.”
Fox challenged the audience to, as rheumatologists, be the “gatekeeper” for patients who present with dry eyes or dry mouth, by contacting specialists like ophthalmologists to help patients secure appointments quickly. He concluded by reiterating the importance of addressing cognitive complaints, whether related to sleep, emotional or idiopathic neurocognitive processes.
“What we see are patients who come in with arthritis or rashes, but what they’re concerned with is losing their jobs because of their cognitive changes. These need to be triaged in an immediate fashion and not simply blown off because they change family dynamics and the loss of income,” he said.
Collapse
Fox RI. Precision Medicine in Sjögren’s Syndrome. Presented at: 2021 Interdisciplinary Autoimmune Summit (IAS). April 15-18, 2021 (virtual meeting).
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