Table 1 Comparisons between the 2002 and 2012 criteria for Sjögren’s syndrome (SS)

(I) Ocular symptoms (positive response to at least one of three):

(I) Ocular symptoms:

Daily, persistent, troublesome eyes for more than 3 months

Not included

Recurrent sensation of sand or gravel in the eyes

Use of tear substitutes more than three times per day

(II) Oral symptoms (positive response to at least one of three):

(II) Oral symptoms:

Daily feeling of dry mouth for more than 3 months

Not included

Recurrently or persistently swollen salivary glands as an adult

Frequent drinking of liquids to aid in swallowing food

(III) Ocular signs (positive result for at least one of two tests):

(III) Ocular signs:

Schirmer’s test, performed without anesthesia (≤5 mm in 5 minutes)

Keratoconjunctivitis sicca with ocular staining score ≥3, according to Whitcher et al. [13] (preferential use of fluorescein staining or lissamine green staining, but break-up time; and unanesthetized Schirmer’s test can also be used). It is assumed that individual is not currently using daily eye drops for glaucoma and has not had corneal surgery or cosmetic eyelid surgery in the last 5 years).

Rose Bengal score or other ocular dye score (≤4 according to van Bijsterveld’s scoring system)

(IV) Histopathology in minor salivary gland biopsy:

(IV) Histopathology in minor salivary gland biopsy:

Focal lymphocytic sialoadenitis, with focus score ≥1 (a focus is defined as ≥50 lymphocytes per 4 mm² of glandular tissue adjacent to normal appearing mucous acini)

Focal lymphocytic sialadenitis, with a focus score ≥1 (a focus is defined as ≥50 lymphocytes per 4 mm² of glandular tissue adjacent to normal appearing mucous acini)

(V) Salivary gland involvement (positive result for at least one of three):

(V) Salivary gland involvement:

Unstimulated whole salivary flow (≤1.5 ml/15 minutes)

Not included

Parotid sialography showing the presence of diffuse sialectasis (punctuate, cavitary or destructive pattern), without evidence of obstruction in the major ducts

Salivary scintigraphy showing delayed uptake, reduced concentration and/or delayed excretion of tracer

(VI) Autoantibodies:

(VI) Autoantibodies:

Presence in the serum of antibodies to Ro (SS-A) or La (SS-B) antigens, or both

Positive serum anti-SS-A/Ro and/or anti-SS-B/La or (positive rheumatoid factor and anti-nuclear antibody (ANA) titer ≥1:320)

Classification criteria:

Classification criteria:

Primary SS:

At least two of the three items in order to classify a patient as SS

The presence of any four of the six items, as long as either item IV (histopathology) or item VI (serology) is positive

The presence of any three of the four objective criteria (items III, IV, V and VI)

Secondary SS:

In the presence of another connective tissue disease, the presence of item I or item II, plus any two from items III, IV and V

Exclusion criteria:

Exclusion criteria:

Past head and neck radiation treatment, hepatitis C infection, AIDS, pre-existing lymphoma, sarcoidosis, graft versus host disease, use of anticholinergic drugs (since a time shorter than fourfold the half-life of the drug)

History of head and neck radiation treatment, hepatitis C infection, AIDS, sarcoidosis, amyloidosis, graft versus host disease, IgG4-related disease