Types of  Sjogren's Syndrome


What is the difference between Primary and Secondary Sjogren's Syndrome?:

 1. Primary - localized mainly to the eyes and mouth

People with primary Sjogren's have a greater chance of extra glandular involvement.

Primary Sjogren's syndrome occurs by itself. 

Secondary Sjogren's syndrome occurs when another connective tissue disease is present.

Primary and secondary Sjogren’s can have the same symptoms. 

In primary Sjogren’s Syndrome, approximately 10 to 20 percent of people have neurological manifestations such as numbness and tingling in their hands and fingers and carpal tunnel syndrome. In many people these may be the initial symptoms when presenting to a doctor.

In many people these may be the initial symptoms when presenting to a doctor. This can often mean that the doctor does not associate these symptoms with SS as they are expecting dry eyes and dry mouth symptoms when diagnosing SS. 

This may be a contributing factor in the long time it takes many people to get a correct diagnosis.

 2. Secondary - systemic - occuring in conjuction with other connective tissue diseases such as lupus or rheumatoid arthritis.

primary vs secondary sjogren's syndrome
  • Seronegative means that your blood does not produce the antibodies that show up when tested for the presence of an antibody.

  • Antibodies are indicators of autoimmune disease​​​​​​​. Not everyone with Sjogren’s has antibodies to SSA and SSB.  

    "...these antibodies alone do not account for all cases of Sjogren's syndrome, and the presence of anti-CCP and anti-centromere autoantibodies may account for some cases of primary Sjogren's syndrome in patients seronegative for anti-SSA and anti-SSB." MedPage Today 

Seronegative autoimmune diseases.

A close relationship exists between autoimmunity and autoantibodies; despite this, some patients are persistently negative for disease‐specific autoantibodies. These conditions have been defined as seronegative autoimmune diseases. Although the prevalence of seronegative autoimmune diseases is low, they may represent a practical problem because they are often difficult cases. Read more
Primary vs Secondary sjogren's

Treating Primary vs. Secondary Sjögren’s

Does it really matter? NO - It certainly doesn't matter to the patient or the clinician treating a patient. It doesn't alter treatment, since treatment for these diseases is based largely on the clinical manifestations and symptoms. All patients should be treated on a case-by-case basis. 

Does the label mean your disease is more or less severe? ABSOLUTELY NOT.  If someone has labeled you as having "Secondary Sjögren’s," it does not mean that your Sjögren’s is less severe or secondary in importance to the other condition. It also doesn't mean that symptoms that were labeled as Sjögren’s symptoms previously are now symptoms of the other disease. Autoimmune diseases often overlap, and sometimes it's difficult to tell if a symptom is Sjögren’s or, say, lupus. In fact, Sjögren’s is the most frequent disorder that occurs in conjunction with other autoimmune and rheumatic diseases, so, again, your signs and symptoms must guide the treatment. 

Does the label make a difference as to whether patients are monitored for specific complications or not? NO. Again, your management and treatment should depend on your manifestations of autoimmune disease.

From Ask the SSF staff