Diagnosis of Sjögren Syndrome

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As the various symptoms and effects do not always appear in all patients, Sjögren Syndrome can be a difficult disease to diagnose. The symptoms may have to be assessed individually, by different specialists, rather than being evaluated as a whole disease.

There is no single diagnostic test to determine whether a patient has Sjögren Syndrome. Doctors specialising in the disease evaluate a combination of factors including signs and symptoms, a detailed medical history, a physical exam, certain diagnostic tests, and laboratory tests.

In many cases, patients are visited individually by organ specialists (ophthalmologists, dentists, otolaryngologists, or gynaecologists) due to multiple dryness problems; the main specialists, doctors in internal medicine, primary care physicians, and rheumatologists have a comprehensive view of the patient and can make an accurate diagnosis.

Doctors with knowledge of autoimmune diseases, in relation to primary care professionals, are the specialists who diagnose and monitor patients with this syndrome. Other medical professionals are also involved in the diagnosis, as well as the follow-up and treatment of the specific organ affected by the disease.

Tests for diagnosing Sjögren Syndrome

The tests required to diagnose Sjögren Syndrome are:

Folder with a patient's medical history

Medical history. This is taken by a specialist in systemic autoimmune diseases and includes the history of symptoms, oral and ocular changes, fatigue, anxiety, muscle and joint pain, duration of symptoms, medication, and so on.

Optometric chart

Complete physical exam. This includes examinations of the eyes, mouth, lymph nodes, salivary glands, and other organs.

Blood collection tube

Complete immunological exam. This evaluates the need for disease-specific tests.

The main immunological tests are carried out through a blood analysis to determine:

Antinuclear Anti-Nuclear Antibody or ANA Test

Nuclear Antibodies (ANA). ANAs are a group of antibodies that react against the normal components of the cell nucleus. About 90% of patients with Sjögren Syndrome have a positive ANA test result. However, having these does not confirm the existence of the disease.

Rheumatoid factor test sample tube

Rheumatoid factor (RF). This test usually indicates a rheumatological disease such as rheumatoid arthritis, although it may come back positive in half of Sjögren sufferers; again, it does not confirm the disease, but a positive result increases the likelihood of having Sjögren Syndrome.

Sample tube and antibodies for Ro (or SS-A) and La (or SS-B) test

Ro (or SS-A) and La (or SS-B). These are the marker antibodies for Sjögren Syndrome. Up to 80% of Sjögren sufferers are positive for Ro, and 40% are positive for La. Although people with other autoimmune diseases may have these antibodies (mainly patients with lupus, myositis, or scleroderma), a positive result for Ro is considered very indicative of Sjögren Syndrome, to such an extent that it is currently considered the main immunological marker for this disease.

Sample tube with cryoglobulins

Cryoglobulins. These are abnormal blood proteins involved in immune reactions and are detected in approximately 10% of Sjögren sufferers.

Sample tube with cryoglobulins

Complement factors. Decreased levels of complement factors C3 and C4 are detected in 15% of patients with the disease.

Explorations for studying the functionality of the salivary glands:

Doctor performing an E-vision test

Ophthalmological exam.

Lips with a salivary flow measuring tape

Salivary flow test. This measures the amount of saliva produced over a certain period of time.

Salivary scan test to study the function of salivary glands

Salivary scintigraphy. This is a nuclear medicine test that evaluates the function of the major salivary glands. The results are classified into four levels, from I (normal) to IV (maximum involvement of the glands). You should bear in mind that this test measures how the glands work, but does not classify the disease into grades, this is done using the Schall classification.

Salivary gland biopsy

Biopsy of the minor salivary glands. A salivary gland biopsy (usually of the lower lip) may be necessary to confirm infiltration of inflammatory (lymphocytic) cells from the minor salivary glands. It must be carried out by experts in the technique, since its incorrect execution can leave chronic aftereffects. This test is reserved for the most difficult-to-diagnose cases.

These tests are combined and integrated by an expert in systemic autoimmune diseases, applying international criteria (currently the ACR/EULAR 2016 criteria). Occasionally, people may not meet these criteria but the specialist considers them to have the disease; it is recommended that this diagnosis, contrary to the established criteria, is made by experts in the disease who have a great deal of experience and international recognition.

Substantiated information by:

Manuel Ramos Casals

Published: 28 May 2019
Updated: 28 May 2019

The donations that can be done through this webpage are exclusively for the benefit of Hospital Clínic of Barcelona through Fundació Clínic per a la Recerca Biomèdica and not for BBVA Foundation, entity that collaborates with the project of PortalClínic.


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