Key Strategies for Treating Sjögren Syndrome

Sjögren syndrome (SS) and its chief symptoms of ocular and oral dryness can be debilitating for patients. Unfortunately, it is commonly misdiagnosed and therefore mismanaged. At the Interdisciplinary Autoimmune Summit 2021, Robert I. Fox, MD, PhD, presented on precision medicine in SS. He highlighted a number of key strategies for treating SS, including several pearls for improving the diagnosis and care of the disease.

In your presentation, you discussed the various chief complaints associated with SS. For your non-rheumatologic colleagues, what sorts of questions could they ask a patient that may help lead to a diagnosis of SS?
Physicians should ask questions such as:

• Have you had dry eyes or dry mouth for over 6 wks?
• Do you need to use artificial tears for your eyes?
• Do you need to drink water when you swallow food or sleep with water by the bed at night?
• Do you have painful involvement of joints, muscles or nerves? Has there been a change in your energy or your cognitive ability?

These questions can highlight the major complaints of SS, dry mouth and dry eyes. Key treatments for ocular dryness include using artificial tears and treating blepharitis with options such as cyclosporine ophthalmic emulsion 0.05% and lifitegrast ophthalmic solution 5%.

What clinical and diagnostic features can help make a differential diagnosis between SS and systemic lupus erythematosus (SLE)?
For the clinical features, dry eyes and dry mouth, including decreased tear flow (Schirmer's test) and unstimulated saliva flow, as well as swelling of parotid and cervical nodes are notable. Extraglandular features to watch for include the type of skin rash and neurologic features. Lymph nodes should also be monitored, as there is a higher frequency of lymphoma in SS vs SLE. In terms of laboratory features, pay attention to the SS-A and SS-B antibodies and their different appearance on CXR and chemistry panel (ie, more overlap with lymphocytic processes such an intersitial nephritis or biliary cirrhosis).

While biologic agents have shown some efficacy in treating SS, none are FDA/EULAR approved. How do you recommend your colleagues work around this restriction?
No biologics are approved for SS as a whole disease. However, particular features, such as rash of mixed cryoglobulinemia, thrombocytopenia, arthritis, and vasculitis including neuropathies, all have particular indications that have FDA approval. Use these to your advantage.

Are there any other pearls or takeaways you would like to share with your colleagues regarding SS?

• More than 50% of patients with a positive ANA and dry eyes are incorrectly diagnosed as SLE, thus physicians do not follow their ocular and oral problems nor follow them for their higher chance of developing lymphoma.
• Remember that SS-A is not a criterium for SLE.
• Ocular symptoms of dryness will not respond to eye drops until you treat the blepharitis. Similarly, oral mouth pain will not respond symptomatically to cevimeline or pilocarpine until you treat the underlying erythematous candida first.

Reference
Fox R. Precision medicine in Sjògren’s syndrome. Presented at: Interdisciplinary Autoimmune Summit 2021; April 15-18, 2021; virtual.