Sjogren's Syndrome

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Revised International Classification Criteria for Sj?gren's Syndrome

I. Ocular symptoms:  Yes to one or more of the following:

1. 
   
   Have you had daily, persistent, troublesome dry eyes for more than 3 months?
2. 
   
   Do you have a recurrent sensation of sand or gravel in the eyes?
3. 
   
   Do you use tear substitutes more than 3 times a day?

II. Oral symptoms: Yes to one or more of the following:

1. 
   
   Have you had a daily feeling of dry mouth for more than 3 months?
2. 
   
   Have you had recurrently or persistently swollen salivary glands as an adult?
3. 
   
   Do you frequently drink liquids to aid in swallowing dry food?

III. Ocular signs- objective evidence of ocular involvement defined as a positive result for at least one of the following two tests:

1. 
   
   Schirmer's I test, performed without anaesthesia (
2. 
   
   Rose bengal score or other ocular dye score (>/=4 according to van Bijsterveld's scoring system)

IV. Histopathology: Focal lymphocytic sialoadenitis in minor salivary glands. Requires 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

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

1. 
   
   Unstimulated whole salivary flow (
2. 
   
   Parotid sialography showing the presence of diffuse sialectasias (punctate, cavitary or destructive pattern), without evidence of obstruction in the major ducts
3. 
   
   Salivary scintigraphy showing delayed uptake, reduced concentration and/or delayed excretion of tracer

VI. Autoantibodies: presence in the serum of the following autoantibodies:

1. 
   
   Antibodies to Ro(SSA) or La(SSB) antigens, or both

 Classification

Primary Sjogren's Syndrome

In patients without any potentially associated disease, primary SS may be defined as follows

1. 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

2. The presence of any 3 of the 4 objective criteria items (that is, items III, IV, V, VI)

3. The classification tree procedure represents a valid alternative method for classification, although it should be more properly used in clinical-epidemiological survey

Secondary Sjogren's Syndrome

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

Must exclude:

• 
  
  Past head and neck radiation treatment
• 
  
  Hepatitis C 
• 
  
  Acquired immunodeficiency disease
• 
  
  Pre-existing lymphoma
• 
  
  Sarcoidosis
• 
  
  Graft versus host disease
• 
  
  Use of anticholinergic drugs 

Posted in: MEDICAL,USMLE