Sjogren’s Syndrome

The presence of any four of the following six criteria items provides              sensitivity 97.4%, specificity 89.4%.

(1) Ocular symptoms: a positive response to at least one of the following questions:

  • Have you had daily, persistent, troublesome dry eyes for more than 3 months?
  • Do you have a recurrent sensation of sand or gravel in the eyes?
  • Do you use tear substitutes more than 3 times a day?                                          

(2) Oral symptoms: a positive response to at least one of the following quesstions:       

  • Have you had a daily feeling of dry mouth for more than 3 months?
  • Have you had recurrently or persistently swollen salivary glands as an adult?
  • Do you frequently drink liquids to aid in swallowing dry food?

(3) Ocular signs: objective evidence of ocular involvement defined as a positive result for at least one of the following two tests:

  • Schirmer’s I test, performed without anaesthesia (<5 mm in 5 minutes)
  • Rose bengal score or other ocular dye score (>4 according to van Bijsterveld’s scoring system)

(4) Histopathology

  • In minor salivary glands (obtained through normal-appearing mucosa) focal lymphocytic sialoadenitis, evaluated by an expert histopathologist, with a focus score >1, defined as a number of lymphocytic foci (which are adjacent to normal-appearing mucous acini and contain more than 50 lymphocytes) per 4 mm2 of glandular tissue

(5) Salivary gland involvement: objective evidence of salivary gland involvement defined by a positive result for at least one of the following diagnostic tests:

  • Unstimulated whole salivary flow (<1.5 ml in 15 minutes)
  • Parotid sialography showing the presence of diffuse sialectasias (punctate, cavitary or destructive pattern), without evidence of obstruction in the major ducts
  • Salivary scintigraphy showing delayed uptake, reduced concentration and/or delayed excretion of tracer20

(6) Autoantibodies: presence in the serum of the following autoantibodies:

  • Antibodies to Ro(SSA) or La(SSB) antigens, or both

 

PRIMARY SJOGREN’S                                                                                                      In patients without any potentially associated disease, primary SS may be defined as follows:

  • The presence of any 4 of the 6 items is indicative of primary SS, as long as either item IV (Histopathology) or VI (Serology) is positive
  • The presence of any 3 of the 4 objective criteria items (that is, items III, IV, V, VI)

SECONDARY SJOGREN’S                                                                                                In patients with a potentially associated disease (for instance, another well defined connective tissue disease), the presence of item I or item II plus any 2 from among items III, IV, and V may be considered as indicative of secondary SS

EXCLUSION CRITERIA

  • Past head and neck radiation treatment
  • Hepatitis C infection
  • Acquired immunodeficiency disease (AIDS)
  • Pre-existing lymphoma
  • Sarcoidosis
  • Graft versus host disease
  • Use of anticholinergic drugs (since a time shorter than 4-fold the half life of the drug)

 

Reference:  Vitali et al.  Classification criteria for Sjögren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 2002;61:554-558.