April 16, 2021
3 minutes to read
Source / Disclosures
Fox RI. Precision medicine in Sjögren’s syndrome. Presented at: Autoimmune Interdisciplinary Summit (IAS) 2021. April 15-18, 2021 (virtual meeting).
Disclosures: Fox reports a consulting relationship with Celegene (now BMS), Novartis and Pfizer.
The ability to identify features of Sjögren’s syndrome and seek payer approval for specific manifestations of the disease is of paramount importance to the rheumatologist, according to a presenter at the 2021 Interdisciplinary Autoimmune Summit.
During the virtual meeting, Robert I. Fox, MD, PhD, chief of the rheumatology department at Scripps Memorial Hospital and Research Foundation, said more than 50% of patients with Sjögren syndrome are misdiagnosed.
“Although lupus and Sjögren’s syndrome share many characteristics, it is easier to think of lupus as an immune complex disease and Sjögren as an aggressive lymphocyte disorder,” Fox explained. “The anti-SSA antibody is not a criterion for lupus. More than 50% of patients with Sjögren syndrome are misdiagnosed. They end up in pulmonology, hematology and nephrology clinics, and … as soon as they enter this specialty, they will be lost because Sjögren’s patients and many of their important issues such as endemic tooth decay will not be treated adequately.
Three common complaints
Regarding diagnosis, Fox cautioned participants to be vigilant when patients present with oral and eye signs and symptoms, as well as fatigue. Oral complaints include a feeling of dry mouth, recurrent swollen salivary glands, and frequent use of oral fluids to aid swallowing. Cevimeline and pilocarpine are both FDA approved for the treatment of dry mouth.
Regarding eye symptoms, dry eye is common and patients may develop corneal abrasions and uveitis, which require prompt and proper treatment. Fox also warned participants to beware of ocular shingles and herpetic keratitis. FDA-approved options for dry eyes include topical cyclosporine and topical lifitegrast (Xiidra, Novartis).
Extraglandular manifestations also occur in Sjogren’s syndrome, including renal infiltrates, which are most often interstitial nephritis. “If you see glomerulonephritis in Sjögren’s disease, think mixed cryoglobulinemia, think amyloid,” Fox said.
Neurological manifestations vary among patients with Sjögren syndrome, but neuromyelitis optic and length-independent neuropathy are typical, as well as multiplex mononeuritis. There is also a much higher incidence of lymphoma – mucosal-associated or diffuse lymphoid tissue – in patients with Sjogren’s syndrome, compared to patients with lupus, Fox said.
Differentiate Sjögren from Systemic Lupus Erythematosus
Additional differences between Sjögren’s disease and lupus include skin manifestations, which are usually mixed cryoglobulinemia, subacute lupus, and dry skin, also called xeroderma, in Sjögren’s disease. “The lung manifestations are also different,” Fox said. “We are seeing more interstitial lung disease, especially lymphocytic interstitial lung disease rather than the pleurisy that you normally see in lupus.”
One of the most important points Fox made about extraglandular manifestations is that they actually respond to biologics, despite the lack of an FDA-approved treatment specifically for Sjögren’s syndrome.
“The problem for the rheumatologist is that these biological agents may not be covered by insurance because they are not officially approved for Sjögren’s syndrome, but they may be for interstitial pneumonia, for example. [lymphoid interstitial pneumonia] … They can be covered for cryo-mixed, ”Fox said. “There are manifestations, like hemolytic anemia, where we can use rituximab rather than just think of it as Sjögren’s syndrome. These biologics can work, but you must remember that they do work in certain situations and you should not be misled by false claims that there are no biologics for Sjögren syndrome.
Fox challenged the public, as rheumatologists, to be the “gatekeeper” for patients who present with dry eyes or dry mouth, reaching out to specialists like ophthalmologists to help patients get quick medication. appointment. He concluded by reiterating the importance of dealing with cognitive complaints, whether related to sleep, emotional neurocognitive or idiopathic processes.
“What we’re seeing are patients coming in with arthritis or rashes, but what worries them is losing their jobs because of their cognitive changes. These need to be sorted out immediately and not just eliminated because they change family dynamics and loss of income, ”he said.