Sjögren's Syndrome

Sjögren's syndrome (SS) is a chronic inflammatory disorder characterized most commonly by dry “gritty” eyes, dry mouth and swollen, painful glands underneath the jaw, but can involve a wide variety of other organs.  While some patients develop this syndrome on its own, it commonly occurs in the setting of another inflammatory disorder such as rheumatoid arthritis.

The majority of patients complain of painful irritated eyes and/or difficulty swallowing dry food such as crackers without liquids.  Eventually they can develop corneal ulcerations, eyelid infections, and difficulty wearing contact lenses; they can also have accelerated dental caries, a decreased sense of taste, and even infections in the mouth.  Some patients also develop vaginal dryness and infections.

These patients can develop symptoms affecting most every organ, from relatively benign non-specific coughs, recurrent sinusitis, and non-deforming joint and muscle pain, to more serious complications affecting the skin as well as internal organs.  Some of the more devastating manifestations, such as brain abnormalities and motor and sensory peripheral neuropathies, often precede the dry eyes and dry mouth, thus delaying a diagnosis; others, including an increased incidence of neoplasms including non-Hodgkin’s lymphoma (about 5 percent of patients during their lifetimes) only appear years after the diagnosis of SS.

Physicians can make the diagnosis clinically from the patient’s history, after excluding non-autoimmune causes of similar symptoms such as prior head/neck irradiation, HIV, hepatitis C, amyloid, sarcoid, and a number of medications.  While some antibody tests of the blood (such as anti-Ro / SSA and anti-La / SSB) can help make the diagnosis, these tests are often negative in patients who have a definitive and classic presentation of the disease. 

Other helpful tests include the Rose Bengal and Schirmer tests to confirm decreased tear production and lacrimal gland function, and less commonly imaging studies to evaluate the salivary glands. If the diagnosis is still in question, salivary gland biopsy can be performed on an outpatient basis.

While rheumatologists take the lead in treating SS, many different specialists contribute to the therapeutic decisions – including pulmonologists, neurologists, ophthalmologists, otolaryngologists and others.  Patients whose symptoms are isolated to dry eyes and dry mouth may respond to symptomatic measures such as frequent tear drops, low-sugar lozenges and good oral hygiene, while others may need oral medications to induce more salivary production (cevimeline and pilocarpine) or minor surgical procedures. 

Patients with more systemic symptoms may need more aggressive immunosuppressive approaches, from the milder agents like hydroxychlororoquine to stronger medications such as methotrexate. Many of the newer injection and infusion-based biologic agents are currently under investigation for the more refractory cases of SS – which only indicates that the future continues to look brighter for this syndrome.