Diagonstic Sjögren's Syndrome

Ko Bogen  Dusseldorf                                                                                          Copyright   Geoff Catlin




Diagnostic Sjögrens Syndrome


oringially published by Rheuma-Liga Hessen e. V. Germany



What is "primary", what is "secondary"? When Sjögren syndrome is associated with another autoimmune disease, it is referred to as a "secondary Sjögren syndrome", as opposed to the "primary Sjögren syndrome" as the sole disease.


Neither Sjögren's primary nor secondary Sjögren syndrome currently have established diagnostic criteria.


There are only different proposals for classification.


The following classification proposal has been prepared by European doctors:


1. Eye discomfort.

At least one positive answer to one of questions 1-3 in the questionnaire below.


2. Complaints in the mouth.

At least one positive answer to one of questions 4-6 in the questionnaire below.


3. Eye Surgery.

Positive Schirmer or Rose Begal test.


4. Tissue findings.

At least 1 lymphoid cell focus (> 50 mononuclear cells) per 4 mm² salivary gland tissue.


5. Spinal fluid involvement.

At least one positive result in the following three tests:

- saline scintillation,

- parotid sialography,

- unstimulated salivary flow (<1.5 ml per 15 minutes).


6. Autoantibody detection.

At least one positive finding:

- SS-A / Ro or SS-B / La antibodies,

- Antinuclear antibodies (ANA),

- rheumatoid factor.



Questionnaire for recording eye and mouth dryness in Sjögren's syndrome:


1. Have you been suffering from daily, stressful eye and mouth discomfort for more than three months?


2. Do you often feel a foreign body feeling (sand) in your eyes?


3. Do you use tear replacement solutions more than three times a day?


4. Have you been suffering from daily drowsiness for more than three months?


5. Do you suffer from recurring or persistent swelling of the mesenteric glands as adults?


6. Are you forced to drink something to swallow dry food?



These classification criteria are not used when the following diseases are already present: lymphoma, AIDS, sarcoidosis, graft-versus-host disease.


If these diseases do not exist and no other (rheumatic) diseases are present, it can be assumed that there is a primary Sjögren syndrome with more than 90% probability if four of the six criteria are satisfied (at point 6 only SS- A / Ro antibodies).


If another (rheumatic) disease is known, for example, rheumatoid arthritis (chronic polyarthritis), lupus erythematosus, or scleroderma, it can be assumed with a 90% probability that a secondary Sjögren syndrome is present when the first or Second criterion as well as the sixth criterion and two of criteria 3, 4 and 5 are fulfilled.


(According to Peter, H.-H .; Pichler, W. J .: Clinical Immunology, Munich: Urban and Schwarzenberg, 1996)



Elsewhere, the diagnosis of secondary Sjögren's syndrome can be considered assured if typical symptoms (eg, dryness problems) and SS-A / Ro or SS-B / La antibodies are present. It is then not absolutely necessary to carry out further investigations such as a lip biopsy.


(According to Krüger, K .: The Sjögren Syndrome, in: mobile magazine of the German Rheuma League, 25 (1999), No. 2, pp. 4-9.




These classification criteria describe an advanced disease pattern. They are not suitable for the early diagnosis of the Sjögren syndrome. At present, there are no examination procedures or diagnostic criteria with which a Sjögren syndrome can be reliably diagnosed in the early stages. Therefore, the disease is often only recognized by those affected when the symptoms of dryness have become massively conspicuous. This is often the case only at the age of 50 years or more.

As Sjögren's syndrome is usually chronic-progressive, it is assumed that the disease starts much earlier in most cases, probably between the ages of 20 and 30 years.


Please note:

The Schirmer test is relatively unsuitable for measuring disturbances of tear secretion, since tear secretion may even be reflexively increased, especially at the beginning of diseases in which tear secretion is disturbed (eg Sjögren's syndrome). More meaningful tests are not known to me.


The occurrence of SS-A / Ro and SS-B / La antibodies may precede the occurrence of complaints for years. SS-A / Ro and SS-B / La antibodies may be present when the test for antinuclear antibodies (ANA) is negative. Therefore, in the case of negative ANA, the ANA subtypes such as SS-A / Ro-AK, SS-B / La-AK and other collagen-specific autoantibodies (see http://www.hpseelig.de/aak/aak.php?P = Cell nucleus autoantibodies are as differentiated as possible.


After a lip biopsy, the healing of the mucous membrane in the Sjögren syndrome can be made more difficult.


(From the "Lupus Erythematosus Diary" and the "Lupus Erythematosus Encyclopedia" by D. Maxin, Darmstadt: Verlag für Neue Medizin, 2003/2000)



The diagnosis of Sjögren's syndrome is based on the typical dryness symptoms, as well as blood tests on antinuclear antibodies (ANA), and in particular the subgroup of the SS-A / Ro antibodies and the SS-B / La antibodies. ANA may be negative, but SS-A / Ro-AK and / or SS-B / La-AK may be positive.