What is Sjogren disease?

Sjogren disease (formerly Sjogren syndrome) is an autoimmune condition in which the immune system attacks the body's own moisture-producing glands. The most prominent symptoms are dry eyes and dry mouth, but Sjogren disease can also affect joints, lungs, kidneys, and other organs, and it commonly causes severe fatigue.

The diagnostic story. Sjogren disease often starts gradually and is frequently attributed to other causes (medications, ageing, allergic eye disease, anxiety) for years before correct diagnosis. Doctors typically use a combination of objective tests (Schirmer test for tear production, salivary flow tests, salivary gland imaging or biopsy) and blood tests (looking for specific antibodies called anti-Ro and anti-La) to make the diagnosis.

The biology. The autoimmune attack on glandular tissue is driven by both T cells and B cells. The immune signalling involves several molecules including BAFF (a B-cell survival factor), CD40 and CD40L (signals between T and B cells), and others. Until recently, no medicine had been specifically approved to interrupt the underlying immune biology in Sjogren disease.

The therapy options.

Symptom relief: artificial tears, saliva substitutes, medicines that stimulate the remaining glandular tissue (pilocarpine, cevimeline). Daily mouth care to prevent dental complications.

Off-label systemic therapy: hydroxychloroquine, corticosteroids in flare, and other immunosuppressants used in autoimmune conditions are sometimes used in Sjogren disease but without formal approval and with mixed evidence.

Emerging specific therapy: medicines targeting BAFF, CD40L, and other parts of the underlying biology are in late-stage trials, with the goal of becoming the first specifically-approved systemic therapy for Sjogren disease.

What to expect. With current therapy, most patients can manage the dryness symptoms reasonably well, but fatigue, joint pain, and broader systemic features have been less well addressed. The emerging specific therapy options offer the prospect of meaningfully reducing the underlying immune activity rather than just managing symptoms.