Table 17.2.

International consensus criteria for Sjögren’s syndrome, systemic lupus erythematosus, and scleroderma

I. Primary SS

A. Ocular symptoms (at least one present)

1. Daily, persistent, troublesome dry eyes for more than 3 months

2. Recurrent sensation of sand or gravel in the eyes

3. Use of a tear substitute for more than three times a day

B. Oral symptoms (at least one present)

1. Daily feeling of dry mouth for at least 3 months

2. Recurrent feeling of swollen salivary glands as an adult

3. Need to drink liquids to aid in washing down dry foods

C. Objective evidence of dry eyes (at least one present)

1. Schirmer’s-I test

2. Rose Bengal

3. Lacrimal gland biopsy with focus score ≥ 1

D. Objective evidence of salivary gland involvement (at least one present)

1. Salivary gland scintigraphy

2. Parotid sialography

3. Unstimulated whole sialometry (≤ 1.5 mL/15 min)

E. Laboratory abnormality (at least one present)

1. Anti-SS-A or anti-SS-B antibody

2. Anti-nuclear antibody (ANA)

3. IgM rheumatoid factor (anti-IgG Fc)

• Diagnosis of primary Sjogren’s syndrome requires four of six criteria, including a positive minor salivary gland biopsy or antibody to SS-A/SS-B.

• Exclusions include previous radiation to the head and neck lymphoma, sarcoidosis, hepatitis C infection, AIDS, graft-versus-host disease, and medications that can cause dryness.

• Diagnosis of secondary SS requires an established connective tissue disease and one sicca symptom plus two objective tests for dry mouth and dry eyes at the time of their clinical entry into study cohort.

• Diagnosis of SS can be made in patients who have no sicca symptoms if objective tests of ocular and oral dryness are fulfilled including either a minor salivary gland biopsy or anti-SS-A/SS-B antibody.

Diagnostic criteria of SLE

Criterion definition:

Malar rash

Rash over the cheeks