Diagnosis of Sjogren's Syndrome

Controversy in the Diagnosis of Sjogren's Syndrome

The specific diagnostic criteria required for Sjogren's syndrome has been a source of controversy in the medical community in the past. The lack of a universally accepted classification system has contributed to the controversy and has made the clinical diagnosis of Sjogren's syndrome problematic. For example, different diagnostic criteria for patients with suspected Sjogren's syndrome have been used in the past by doctors in the United States, Europe, and Japan.

In 2002, a European-American consensus committee approved a set of six diagnostic criteria that is believed to be 95% accurate for the diagnosis of Sjogren's syndrome, namely:

• Ocular symptoms
• Ocular signs
• Oral symptoms
• Salivary gland involvement
• Histopathology
• Laboratory results

Ocular Symptoms

• Daily persistent troublesome dry eyes for at least three months
• Recurrent sensation of sand or gravel in the eyes
• Use of tear substitutes more than three times a day

Ocular Signs

• Positive response for Schirmer eye test without anesthetic (5 ml or less of tears produced in 5 minutes)
• Ocular dye score (Rose-Bengal test or other) of 4 or higher

Oral Symptoms

• Daily feeling of dry mouth for more than three months
• Recurrent or chronic swollen salivary glands as an adult
• Frequent drinking of liquid to help swallow dry food

Abnormal Salivary Gland Involvement - at least one of the following tests must be positive:

• Parotid gland scintigraphy
• Parotid sialography
• Sialometry response (unstimulated) of 1.5 mL or less in 15 minutes

Histopathology

• Focus score of 1 or higher (50 or more lymphocytes per 4 sq. mm of glandular tissue) in a minor salivary gland biopsy

Autoantibodies

• Detection of Ro(SS-A) and/or La (SS-B)

For a definitive diagnosis of Sjogren's syndrome, at least one element in four of these six categories must be present and one of those positive elements must be either a positive histology response (focus score of more than 1) or the presence of SS-A/B autoantibodies. It is suggested that ANA be used to confirm the diagnosis rather than as an independent criterion since up to 30% of healthy individuals may present with elevated ANA.

The European-American Consensus group recommended the that following medical conditions be ruled out before a patient with dry eye and dry mouth can be positively diagnosed with Sjogren's syndrome:

• Hepatitis C
• HIV infection (the virus that causes AIDS)
• Lymphoma
• Prior head and neck radiation
• Sarcoidosis
• Graft-versus-host disease (in bone marrow transplant patients)
• Use of anticholinergic drugs

Diagnostic criteria notwithstanding, the consensus group acknowledged that an experienced clinician who is an expert in the area of clinical observation and diagnosis of Sjogren's syndrome is crucial for reaching a correct diagnosis of this condition.