Sjögren syndrome

What exactly is Sjogren syndrome (Sicca syndrome, autoimmune exocrinopathy)?

Sjogren syndrome refers to an autoimmune disease that affects especially the salivary and lacrimal glands leaving patients with complaints of dry eyes and a dry mouth. Also other organ systems can be functionally impaired, including the lungs, kidneys and the central nervous system. Additionally, numerous patients experience systemic symptoms such as joint pain, skin rashes or depression.

Women are generally more affected than men, as Sjogren syndrome is thought to develop in relation to the amount of estrogen that circulates within the bloodstream.

As Sjogren syndrome does not belong to those diseases which can be cure, treatment primarily focuses on the management of symptoms.

However, there might be cases when Sjogren syndrome occurs along with another disease. Such patients may experience relief of Sjogren syndrome related symptoms with treatment of the primary disease.

How does Sjogren syndrome develop?

The manifestation of Sjogren syndrome is in many cases associated with an additional chronic condition. This includes rheumatoid arthritis, systemic lupus erythematosus or chronic hepatitis. Some patients develop Sjogren syndrome without an apparent underlying cause, though. Such forms of the disease are referred to as idiopathic – this means it cannot be medically explained.

The symptoms associated with Sjogren syndrome are caused by an infiltration of inflammatory cells into lacrimal and salivary glands, which leads to a crowding-out effect on the healthy glandular tissue. As a result, the glands can no longer produce saliva and tears. Oftentimes it is not quite clear, how these immune cells are programmed to infiltrate these glands. However, it is known that patients who simultaneously suffer from a chronic condition present with an increased number of inflammatory cells circulating their blood. This way, also more of these cells are available to enter salivary and lacrimal glands and cause tissue damage.

What are the symptoms of this autoimmune disease?

As mentioned above, main symptoms of Sjogren syndrome are dry eyes and a dry mouth due to diminished function of respective glands. Such „obstructed“ glands are in addition more susceptible to infections, which is why infections of both lacrimal as well as salivary glands can happen more frequently. Patients often complain about additional symptoms like joint pain, decreased performance, depression and impaired sensation in fingers and toes. In more rare cases, lungs, liver or kidneys can be affected which is also due to inflammatory reactions and associated symptoms.

How is Sjogren syndrome diagnosed?

Several tests are available to diagnose Sjogren syndrome. The first step is usually a blood test through which certain parameters can already point to this disease. In the blood of many patients, special antibodies can be detected which make Sjogren syndrome a probable diagnosis. Also nonspecific inflammatory values are frequently increased.

In addition to testing the patients’ blood, the saliva and tear production of both glands can be assessed. The so-called “Schirmer test” measures the amount of tears liberated and thus assesses the function of the lacrimal glands. For checking the saliva production, the so-called „Saxon test” is initiated which measures how much volume the salivary glands can produce in two minutes. Sjogren syndrome is the probable diagnosis if both of these tests show decreased production.

If at this point no certain diagnosis of Sjogren syndrome can be established, a biopsy of the salivary glands will confirm the strong suspicion. By examining glandular tissue, chronic inflammatory changes can be confirmed.

Which treatment modalities are available?

In most cases, management of idiopathic Sjogren syndrome means treating only the symptoms. The goal is to maintain moist eyes and mouth so that recurrent infections are avoided. For most patients it is sufficient to simply drink a lot, chew gum and regularly apply eye drops. Severe cases can be managed with immunosuppressive drugs. However, the idiopathic type of Sjogren syndrome is unlikely to be cured.

If the Sjogren syndrome is associated with a different primary disease, then of course treatment of the underlying cause often goes along with improvement of the symptoms. Such patients actually have a chance to be cured, in case the underlying condition is treated successfully.

How long does a flare last?

Unlike some other autoimmune diseases, Sjogren syndrome normally does not exhibit flares but develops continuously. Depending on the severity of their condition, many patients initially describe the feeling of having a foreign body stuck in their eye at the beginning of the disease which progresses with time. Also swallowing and eating troubles them and in later stages even talking can become difficult. These symptoms will only improve after establishing the diagnosis and initiation of the symptomatic management.

What doctors or which clinics specialize in the diagnosis and treatment of Sjogren syndrome?

Many patients consult their ENT (ear-nose-throat) or eye doctor at first. Following a primary assessment to establish a diagnosis, patients are oftentimes referred to a rheumatologist for treatment. At this point, any possible underlying disease that is associated with Sjogren syndrome will be checked. Usually Sjogren syndrome is diagnosed and treated in an outpatient clinic, meaning patients do not have to stay in the hospital.

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