WHAT IS SJOGREN'S SYNDROME ?

What is Sjögren's Syndrome?

Sjögren's Syndrome (SS) is a systemic autoimmune disease. An autoimmune disease, as its name suggests is one in which white blood cells that normally protect the body against infection attack different tissues of the body. In Sjögren's Syndrome, the attack is seen against the glands of the mouth and eyes that produce moisture. However, almost any body part can be affected.

What are the symptoms of Sjögren's Syndrome?

Patients with SS have varied symptoms. Dryness of eyes and mouth are described as the most common features. Patients may feel the need to drink water repeatedly, drink water to aid swallowing of food and have a gritty or burning sensation in the eyes. However, in India, symptoms of dryness as mentioned above are relatively less common and many patients are oblivious of dryness. Other symptoms suggestive of dryness could also be the complaints. For example, instead of causing an obvious sensation of dryness, lack of moisture in the mouth can result in decay of teeth at an early age, recurrent fungal infection and burning sensation in mouth. Patients can also develop swelling of moisture producing glands. Dryness of vagina is also seen in patients which can cause painful sexual intercourse.

It is very important to remember that Sjögren's Syndrome is not merely a disease of dryness but a systemic disease. By systemic disease, we mean a disease that can affect almost any organ of the body such as the joints, kidney, skin, nerves, brain, lungs etc. And these symptoms can develop before dryness develops. For instance patients can have pain and swelling of the joints, skin rash, weakness and decreased sensation or pins and needle sensation when nerves are affected, cough and breathing difficulty when lungs are affected and so on. Equally important is the fact that many of these symptoms can precede the onset of dryness even by a decade. Many patients also have fibromyalgia (https://indianrheumatology.org/ira-e-newsletter/issue/2020/january/know-your-disease) which is a condition characterized by one or more of the following: aches and pain all over the body, feeling tired easily and sleep disturbances. In patients with long standing disease, 5-10% develops a complication called lymphoma, which is a cancer of white blood cells.

Whom should I consult?

Patients with Sjögren's Syndrome are evaluated and treated by rheumatologists as is the case with any autoimmune disease. As patients with SS can have involvement of varied organ systems the concerned specialists are also involved in the care. For instance, those with eye and mouth/dental symptoms should consult an ophthalmologist and dentist regularly.

What are the treatments available?

Treatment depends on the presence of dryness and the degree of involvement of other organ systems. For dryness of mouth, salivary substitutes are available in the form of spray. For dryness of eyes, tear supplements can be used. If no response to the above, drugs that can stimulate secretion of saliva and tears for example, pilocarpine can be used.

In case of systemic involvement, drugs which can modify or suppress the immune system are used based on the severity of disease. The drugs used include hydroxychloroquine, methotrexate, leflunomide, azathioprine, mycophenolate, glucocorticoids, rituximab.

Why regular and long term follow up is important?

Regular and long term follow up is important so that complications can be detected well in time and therapy can be tailored to the clinical condition.