American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR) for Primary Sjogren's Syndrome reported criteria for the diagnosis of primary Sjogren's Syndrome (SS).
Inclusion criteria: ocular or oral dryness (persistent dry eye for > 3 months; recurrent sensation of sand or gravel in the eyes; use tear substitutes > 3 times per day; persistent dry mouth for > 3 months; frequently need to drink liquids when swallowing dry good)
Exclusion criteria:
(1) history of radiation to head and neck
(2) active hepatitis C
(3) AIDS
(4) sarcoidosis
(5) amyloidosis
(6) graft versus host disease (GVHD)
(7) IgG4-related disease
(8) patient taking an anticholinergic medication
Parameters:
(1) labial salivary gland biopsy
(2) serum anti-SSA/Ro
(3) ocular staining score
(4) Schirmer's test in mm in 5 minutes
(5) unstimulated whole saliva flow rate in mL per minute (method of Navazesh and Kumar)
|
Parameter
|
Finding
|
Points
|
|
labial salivary gland biopsy
|
negative
|
0
|
|
|
positive
|
3
|
|
serum anti-SSA/Ro
|
negative
|
0
|
|
|
positive
|
3
|
|
ocular staining score
|
negative in both eyes
|
0
|
|
|
positive in at least one eye
|
1
|
|
Schirmer's test
|
> 5 mm in 5 minutes in both eyes
|
0
|
|
|
<= 5 mm in 5 minutes in at least one eye
|
1
|
|
unstimulated whol saliva flow rate
|
> 0.1 mL per minute
|
0
|
|
|
<= 0.1 mL per minute
|
1
|
where:
• A positive labial salivary gland biopsy shows a lymphocytic sialadenitis with a focus score >= 1 in an area of 4 square mm.
• A positive ocular staining score by the method of van Bijsterveld etl al is >= 4.
• A positive ocular staining score by the method of Whitcher etl al is >= 5.
• Interruption of anticholinergic therapy for a sufficient time permits assessment.
total score =
= SUM(points for all 5 parameters)
Interpretation:
• minimum score: 0
• maximum score: 9
• A score >= 4 indicates primary Sjogren's syndrome.
Performance:
• The sensitivity was 96% and specificity 95%.
• The area under the ROC curve was 0.98.
