New Discoveries in Sjögren’s Disease Hold Promise

Sjögren’s disease is evolving as physicians and researchers better understand its causes and types.

One recent change has been renaming the condition, from Sjögren’s syndrome to Sjögren’s disease, said Sara McCoy, MD, PhD, a rheumatologist, physician-scientist and the director of the University of Wisconsin Health Sjögren’s Disease Clinic, Madison, in a recent broadcast of ACR on Air, the ACR’s podcast hosted by Jonathan Hausmann, MD, a pediatric and adult rheumatologist in Boston, who is co-chair for the Sjögren’s OMERACT working group and is on the Board of Directors for the Sjögren’s Foundation.¹

Drilling Down to a Diagnosis

Dr. McCoy said it’s not always clear if dryness is caused by Sjögren’s or something else. Questions you can ask patients with dry eye to help with diagnosis include:

Dr. McCoy

• Do you have a recurrent sensation of sand or gravel in the eye?
• Have you had dry eye daily for more than three months?
• Do you have to use tear substitutes more than three times a day?

Ask patients with dry mouth:

• Have you had dry mouth for more than three months?
• Do you have to take a sip of water to swallow a saltine cracker?
• Have you ever had recurrent or persistent swelling of the salivary glands?

Dr. McCoy performs a Schirmer’s test and a salivary flow test at the same time. A Schirmer’s test involves the use of special paper to test tear production in the eye over five minutes. An unstimulated salivary flow test measures the amount of saliva passively produced by a patient within five minutes after first clearing out their mouth. Passive saliva collection is akin to drooling for five minutes. Dr. McCoy has patients do both tests at the same time for efficiency.

It can be harder to diagnose Sjögren’s disease if a patient does not have strong symptoms of dryness and dryness only becomes apparent during testing. “Some of the patients who say they’re dry aren’t necessarily as dry as you might think and vice versa,” she said.

When it comes to antibody testing, Dr. McCoy orders an SS-A test (anti-Ro 52/60 antibody), both to check for a positive result and to find the patient’s actual SS-A level. Although using other antibodies for a Sjögren’s diagnosis, such as an early antigen panel or carbonic anhydrase VI, has been discussed, not enough evidence supports their use for a Sjögren’s diagnosis.

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.

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

1. Baer AN, Hammitt KM. Sjögren’s disease, not syndrome. Arthritis Rheumatol. 2021 Jul;73(7):1347–1348.
2. 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.
3. 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.