Sjögren's Syndrome

What is it?

Arthritis is a complex family of musculoskeletal disorders consisting of more than 100 different diseases or conditions that destroy joints, bones, muscles, cartilage and other connective tissues, hampering or halting physical movement. Sjögren’s syndrome is an autoimmune disease. A healthy immune system normally protects the body from harm, but in a person with an autoimmune disease, the body’s immune system attacks its own tissues and cells. Sjögren’s syndrome affects the salivary and lacrimal glands, which supply moisture to the eyes and mouth. It causes a reduction in the production of saliva and tears, leading to uncomfortable dryness in the mouth and eyes.

Sjögren’s syndrome is classified as either primary or secondary. The secondary form is diagnosed in people who already have another autoimmune disease such as systemic lupus erythmatosus or rheumatoid arthritis. Sjögren’s syndrome cases are fairly equally divided between primary and secondary.

What are the effects?

Primary Sjögren’s syndrome moves more quickly and causes decreased function in the lacrimal and salivary glands. Secondary Sjögren’s syndrome generally causes less severe dryness in the mouth and eyes; however, patients must manage both the effects of their primary disease in addition to secondary Sjögren’s.

Because it is a systemic condition, Sjögren’s syndrome affects the entire body. It can also cause fatigue, dry skin, chronic dry cough and vaginal dryness. Some people with Sjögren’s syndrome will experience joint pain, stiffness and swelling or numbness and tingling in their extremities. Less common symptoms include rashes and inflammation of the lungs, kidneys or liver. Sjögren’s syndrome that manifests more than just moisture-producing glands is called extraglandular.

One of the most serious concerns facing people with Sjögren’s syndrome is an elevated risk of developing lymphoma, cancer involving the lymphatic system. Though only a small percentage of people with Sjögren’s syndrome develop lymphoma, your doctor should pay particular attention if your major salivary gland is enlarged or rapidly changes size.  Lymphoma can involve the salivary glands, lymph nodes, the gastrointestinal tract and the lungs.

How is it diagnosed?

There is no single test to definitively diagnose Sjögren’s syndrome. Because Sjögren’s symptoms mimic those of many other diseases, it can often take years to make a proper determination.

 To accurately diagnose Sjögren’s syndrome, your doctor will review your medical history, complete a physical exam and perform some lab tests. She will evaluate your overall health, your family’s medical history and ask questions about your lifestyle, including alcohol and tobacco use. Side effects of certain medications can mimic the dry eyes and dry mouth symptoms of Sjögren’s syndrome, so be sure to tell your doctor if you’re taking any medications.

What are the treatment options?

Currently, there is no cure for Sjögren’s syndrome; however, effective treatments are available to manage its symptoms. You will likely see a rheumatologist, a doctor specializing in rheumatic diseases, who will coordinate your care, possibly along with an entire team of healthcare providers.

You should see an ophthalmalogist to manage your eye care and an otolaryngologist to care for your mouth and throat. It’s important to understand that no two patients experience Sjögren’s syndrome in the same way; therefore, a personalized and closely monitored treatment plan is essential.

Various over-the-counter eye drops are available to help ease eye dryness. Your doctor might also prescribe lubricating ointments. Joint pain or inflammation can be treated with nonsteroidal anti-inflammatory drugs (NSAIDs). She may administer corticosteroid medications, which mimic natural anti-inflammatory hormones, to contain organ inflammation in the lungs, liver or kidneys.

If your doctor determines that your overactive immune system needs to be controlled further to prevent organ damage, he may prescribe disease-modifying anti-rheumatic drugs (DMARDs) – medications that actually impede disease progress.