Table 2.

Standard international criteria for the diagnosis of autoimmune diseases.

DiseaseInternational Classification Criteria
Sjögren’s syndromeFour of 6 criteria are necessary for diagnosis:

  1. Ocular symptoms

  2. Oral symptoms

  3. Ocular signs — objective evidence of ocular involvement defined as positive result for at least Schirmer’s test or rose Bengal score/other ocular dye score

  4. Histopathology: In minor salivary glands (obtained through normal-appearing mucosa) focal lymphocytic sialoadenitis evaluated by an expert histopathologist

  5. Salivary gland involvement: objective evidence defined by a positive result

  6. Autoantibodies: presence in the serum of the following autoantibodies [antibodies to Ro (SSA) or La (SSB) antigens, or both]

Autoimmune thyroiditisGraves-Basedow’s disease: criteria 1 + 2 or 1 + 3 or 2 + 3

  1. Hyperthyroidism

  2. Exophthalmos

  3. Positive Tsh-receptor antibodies

Hashimoto’s thyroiditis: criteria 1 or 2 + 3

  1. Hypothyroidism

  2. Goiter

  3. Positive antithyroid peroxidase antibodies

Primary biliary cirrhosisTwo of 3 criteria are necessary for diagnosis:

  1. Clinical fatigue, pruritus, or jaundice

  2. Presence of antimitochondrial antibodies, which are specific markers of the disease

  3. Histological evidence of nonsuppurative inflammation of the liver

Systemic lupus erythematosusFour of 11 criteria are necessary for diagnosis:

  1. Malar rash

  2. Discoid rash

  3. Photosensitivity

  4. Oral ulcers: includes oral and nasopharyngeal ulcers, observed by physician

  5. Nonerosive arthritis

  6. Serositis

  7. Renal disorder (proteinuria > 0.5 g/24h)

  8. Neurologic disorder (seizures without other cause or psychosis without other cause)

  9. Hematologic disorders: hemolytic anemia or leukopenia (< 4000/mm3) or lymphopenia (< 1500/mm3) or thrombocytopenia (< 100,000/mm3) in the absence of offending drugs

  10. Immunologic disorder: anti-dsDNA, anti-Sm, and/or anti-phospholipid

  11. Antinuclear antibodies

Rheumatoid arthritisFour criteria are necessary for diagnosis:

  1. Morning stiffness

  2. Arthritis of 3 or more joint areas

  3. Arthritis of hand joints

  4. Symmetric arthritis

  5. Rheumatoid nodules

  6. Serum rheumatoid factor

  7. Radiographic changes

MyositisDefinite polymyositis = all first 4; probable polymyositis = 3 of first 4; possible polymyositis = 2 of 4.
Definite dermatomyositis = rash + 3 other; probable dermatomyositis = rash + 2 other; possible dermatomyositis = rash + 1 other:

  1. Symmetrical weakness, usually progressive, of the limb-girdle muscles

  2. Muscle biopsy evidence of myositis

  3. Elevation of serum levels of muscle-associated enzymes

  4. Electromyographic triad of myopathy

  5. Characteristic rashes of dermatomyositis