If a patient is SS-A negative but still exhibiting symptoms of Sjögren’s disease, Dr. McCoy orders a labial salivary gland biopsy. However, she said it can be challenging to find an ENT provider or dentist who can perform this procedure.
Another challenge can be getting an accurate pathologic interpretation from the biopsy, as less tissue can lead to a higher likelihood of misdiagnosis.2 “You actually need to pay attention to how much tissue there is and then find a good pathologist,” she said. The pathologist will report a focus score, which involves looking for mononuclear cell infiltrates on the gland.
Treatment
For now, treatments remain mostly supportive. “The first patient described in the literature in the 1880s was treated with the same thing we’re using now—or a derivative of it. That’s nuts,” Dr. McCoy said.
A new episode of ACR on Air comes out twice a month. Listen to the full episode at acronair.org.
For dry mouth, sugar-free gum and candies, wetting agents and sialogogues are available. For dry eye, preservative-free wetting agents, as well as punctal plugs, moisture chamber eyewear and environmental modifications—such as staying out of windy areas—are typical. If patients don’t respond well to the initial ocular treatments, they should be referred to an ophthalmologist. Topical drugs, such as cyclosporine ophthalmic emulsion (Restasis) or lifitegrast ophthalmic solution (Xiidra), may be prescribed, Dr. McCoy said.
The use of endotyping, or grouping patients by pathobiologically similar subtypes, has begun to help patients with Sjögren’s disease by identifying which patients have a phenotype that’s more likely to respond to certain therapies than others, Dr. McCoy said.
Endotyping may help identify which patients could benefit from certain drugs, such as hydroxychloroquine, and is likely to usher in more treatments going forward.4
Vanessa Caceres is a writer in Bradenton, Fla.
References
- Baer AN, Hammitt KM. Sjögren’s disease, not syndrome. Arthritis Rheumatol. 2021 Jul;73(7):1347–1348.
- Fisher BA, Jonsson R, Daniels T, et al. Standardisation of labial salivary gland histopathology in clinical trials in primary Sjögren’s syndrome. Ann Rheum Dis. 2017 Jul;76(7):1161–1168.
- Collins A, Lendrem D, Wason J, et al. Revisiting the JOQUER trial: Stratification of primary Sjögren’s syndrome and the clinical and interferon response to hydroxychloroquine. Rhematol Int. 2021 Sep;41(9):1593–1600.
Adapted from https://www.the-rheumatologist.org/article/new-discoveries-in-sjogrens-disease.
