Sjögren's syndrome is also called sialoadenopathy, kerato-conjunctivitis sicca and xerodermatostenosis, as well as some other names. It is an autoimmune disorder of unknown origin. Possible causes include infectious and allergic agents, as well as endocrine problems. It may have a genetic predisposition and run in families. It is associated with collagen disorders.

It occurs mostly in middle aged women, typically after menopause. People notice less or no tearing or salivary secretions, causing dry eyes or mouth. Inflammation of the pharynx, larynx or nasal passages sometimes occurs, as well as a swelling of the parotid gland (salivary gland located below the ear). Polyarthritis is another frequent symptom. Diagnosis is made by noting at least 2 of the above groups of symptoms together. Other accompanying symptoms may include excessive dental cavities, tracheobronchitis, vaginitis, achlorhydria, Raynaud's disease (characterized by blanching and numbness of fingers and toes when exposed to cold or stress), purpura (skin rash caused by capillaries bleeding into the skin), arteritis (inflammation of the arteries), focal myositis (muscle inflammation), neuropathy (problems of the nervous system), loss of hair, and enlarged spleen or liver.

In the salivary, lacrymal and other affected glands, lymphocytic infiltration is noted, as well as the proliferation of myoepithelial cells and atrophy of some acini (tiny secreting glands). Some patients develop chronic interstitial nephritis (kidney inflammation). Even the benign lesions show clonal B cell populations. Some lymphoma researchers believe that this chronic disease can be classified as pre-neoplastic, where benign lesions already show the precancerous clone cells that may then acquire further genetic abnormalities as they continue to proliferate. Similar evolution is thought likely in cases of MALToma.

Diagnosis is confirmed by three tests: the Schirmer's test measures the extent of moisture in the conjunctival sac. Sialography (saliva analysis) and blood work is performed; the following may be noted: leukopenia (low white blood cells), eosinophilia (abnormal increase of eosinophils), thrombocytopenia (low platelets), hyperglobulinemia, cephalin flocculation, thymol turbidity, rheumatoid factor, tissue antibodies, and increased sedimentation rate.

This chronic disease is said to be only partially responsive to treatment. Mainstream medicine recommends steroids, adrenocorticotropic hormones, electrocautery for keratoconjunctivitis, and topical fibronectin. The disease is usually mild for many years, but some patients develop lymphoma, reticular wall sarcoma, or pseudolymphoma in the form of generalized adenopathy. Pseudolymphomas usually follow a benign course. Other cases become complicated by various collagen diseases.

The above description is culled from somewhat dated compendia. There is a great deal of information on this disease on the Web, some of it from the patients' point of view. As with all chronic diseases, there are many accompanying subtle symptoms patients are better at noticing, sharing, and researching. A simple search will bring up many sites and at least one discussion list.

The disease apparently has significant connection with serious autoimmune diseases like lupus, rheumatic arthritis, and scleroderma. It is cometimes called the "sneaky arthritis." Patients also report many other problems like difficulty swallowing, acid reflux and heartburn, joint pains, vaginal dryness, and others. Some researchers feel that a virus related to the EBV is implicated in the origin of this disease. It also appears that arthritic conditions may be linked to a bacterial infection and respond to antibiotic therapy.

A new book on the disease came out recently, and is worth checking out. The New Sjogren's Syndrome Handbook, compiled by the Sjogren's Syndrome Foundation, ed. by Steven Carsons. Oxford U. Press, 1998.

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Gathered by Vera Bradova © 1999-2003
Updated 8-1-2003