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Sjogren Disease
Sjögren’s Disease: Navigating the Unfamiliar

Released: September 20, 2024

Expiration: September 19, 2025

Elizabeth Kirchner
Elizabeth Kirchner, DNP
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Key Takeaways
  • Diagnosis of Sjögren’s disease is often not straightforward and relies on exclusion of other causes of oral/ocular dryness.
  • There are no FDA-approved disease-modifying antirheumatic drugs for the treatment of Sjögren’s, but off-label treatment is often used for patients with arthritis related to their Sjögren’s.

Sjögren’s disease, although as common as rheumatoid arthritis (RA) and more prevalent than systemic lupus (SLE) in the United States, remains relatively unknown to many patients. Unlike those conditions, Sjögren’s lacks the spotlight. When I broach the topic with patients, the first question is often, “What’s that?”

The name itself—derived from Dr. Henrik Sjögren, a Swedish physician who described the disease in the early 1900s—poses challenges, offering no clues about what to expect. Moreover, Sjögren’s can play a dual role: either piggybacking on another autoimmune disease (secondary Sjögren’s) or standing alone (primary Sjögren’s).

Diagnosing Sjögren’s Disease
Diagnosing Sjögren’s is not always straightforward. There is no single diagnostic test for Sjögren’s; it is a clinical diagnosis made based on symptoms and laboratory features and after the exclusion of other causes of oral and/or ocular dryness. The American College of Rheumatology and the European League of Rheumatology proposed a classification system in 2016. Under this system, patients must achieve a score of at least 4 points from the following criteria:

  1. Positive salivary gland biopsy: 3 points
  2. Positive serology tests for anti–Sjögren’s syndrome–related antigen A (SSA) antibodies, also known as anti-Ro antibodies (anti-SSA/Ro positive): 3 points
  3. Objective evidence of dry eye(s) (positive ocular staining score or Schirmer’s test): 1 point
  4. Abnormal saliva flow rate: 1 point

My Diagnostic Pearls
Every once in a while, a patient will present with classic sicca symptoms (dry eyes, dry mouth) with a note from their ophthalmologist saying they have an abnormal ocular staining score, and their anti-SSA antibody test will be positive. This is as close to a “slam dunk” as one usually gets with Sjögren’s, but even so, it isn’t always clear a patient’s Sjögren’s is primary or secondary.

If they already have an established diagnosis of an autoimmune rheumatic disease (most often RA or SLE), of course, then the Sjögren’s would be considered secondary. But if Sjögren’s is their first rheumatic diagnosis, I have to tell patients that I will treat their Sjögren’s but will also be vigilant for signs or symptoms of the primary rheumatic disease which, confusingly, sometimes reveals itself after Sjögren’s.

A Sjögren’s diagnosis is especially tricky when patients have the clinical symptoms (dry mouth and dry eyes confirmed by an ophthalmologist), but their SSA test is negative. A minor salivary gland biopsy can help secure the diagnosis, but those sometimes can be difficult to schedule and are painful procedures. In those cases, I will sometimes go “off script” and check for other clues, such as whether the patient has a positive test result for antinuclear antibody, anti–Sjögren’s syndrome–related antigen B, and/or rheumatoid factor. If so, I might give them a “soft” diagnosis of Sjögren’s. If patients have symptoms of skin burning/prickling, I will often order a skin biopsy, which can also be useful to determine whether there is a loss of epidermal nerve fibers, which can confirm the presence of small fiber neuropathy, a common neurologic complication of Sjögren’s disease.

Discussing Risk Factors
For patients facing this enigmatic diagnosis, fear and confusion intertwine. They often want to know what caused them to develop an autoimmune disease. I tell them that rather than having one specific cause, a combination of gender, family history, infections, and stress likely contributed to their Sjögren’s. It is well-established that females are far more likely to develop Sjögren’s than males. Patients with a first-degree relative with RA, SLE, or Sjögren’s are much more likely to develop Sjögren’s disease than those without. Infections and stress are “triggers” that are well-known to precede the development of many autoimmune diseases, including autoimmune rheumatic disease, as well as Sjögren’s. For the most part, they were just living their lives when bad luck struck.

Patient Burden
Once the diagnosis is confirmed, the uncertainty for patients is sometimes increased, rather than decreased. The spectrum of manifestations of Sjögren’s disease—from dry eyes and mouth to arthritis, neuropathy, or even cancer—adds complexity to their journey.

To manage their symptoms, patients in the United States often require meticulous dental care but dental coverage is far from guaranteed, annual ophthalmology visits to monitor for hydroxychloroquine toxicity (if using it for treatment), and careful monitoring of complete blood counts and serum proteins to ensure that any abnormalities are caught early and to assess disease activity. It is important to educate patients on the benefits of this type of team approach early in their disease course, so they can plan for multiple specialist appointments and discuss any barriers with their school, employer, and/or insurance company.

A quick mention of some of the more serious manifestations of primary Sjögren’s is also important. Approximately one third of patients with primary Sjögren’s will develop Raynaud’s phenomenon, vasculitis, lymphoma, and/or various organ involvements, especially in the kidneys. Renal tubular acidosis is one of the few true rheumatologic emergencies sometimes seen with primary Sjögren’s.

Patients with primary Sjögren’s who report muscle weakness/paralysis, bone (not just joint) pain, and/or nausea, vomiting, and confusion should be evaluated immediately and referred emergently to nephrology, via admission to the hospital if necessary.

Treatment
Finally, treatment options for Sjögren’s are limited. Dry eye symptoms are typically treated with topical agents such as artificial tears (preservative free), cyclosporine, lifitegrast, topical steroids, and/or punctal plugs. Dry mouth symptoms are treated with artificial saliva, muscarinic agonists, and frequent sips of water.

Systemic treatment is typically not used for patients with symptoms limited to dry eyes/dry mouth but may be offered for patients with arthritis, neuropathy, and/or Raynaud’s related to Sjögren’s.

However, systemic treatment options are also limited. There are no randomized controlled trials supporting the long-term use of prednisone for Sjögren’s disease, and there are no disease-modifying antirheumatic drugs currently approved by the FDA for the treatment of Sjögren’s. Symptoms of Raynaud’s phenomenon and/or neuropathy are treated symptomatically, just as they would be outside the context of Sjögren’s. If a patient has arthritis related to Sjögren’s, off-label treatment often will be offered; this is typically either hydroxychloroquine or methotrexate, but azathioprine, leflunomide, or mycophenolate mofetil may also be used in some cases.

Your Thoughts?
What strategies do you use to ensure an accurate diagnosis for patients who may have Sjögren’s? Leave a comment to join the discussion!