045
Test #
Sjögren Syndrome Panel (includes ANA, RF, Ro, La, CENP)
CPT Code(s) #
86038, 86039, 86255, 86431 x2, 86235 x3
If Profile, Includes Tests:
N/A
Disease Name:
Sjögren Syndrome
Type of Study:
Serum Studies
Methodology:
Indirect Immunofluorescence and ELISA
Substrate:
HEp2/ELISA plate
Reference Range:
ANA (Titer)
• Negative <40
Rheumatoid Factor
IgG, IgA, IgM (Units)
• Negative ≤6
• Positive >6
Ro(Units)
• Negative <20
• Weak Positive 20-39
• Moderate Positive 40-80
• Strong Positive >80
La (SS-B) (Units)
• Negative <20
• Weak Positive 20-39
• Moderate Positive 40-80
• Strong Positive >80
CENP (Units)
• Negative <20
• Weak Positive 20-30
• Moderate to Strong Positive >30
Units:
ANA:Titer, RF,Ro, La, CENP:Units/mL
Schedule:
Assay performed once per week. Report availability is within one week from the time of specimen receipt.
Specimen Requirements:
Collect 5-10 ml of blood in a red top or serum separator tube. If possible, separate serum from clot and place into red capped tube provided with Beutner Laboratories collection kits. If separation facilities are not available, the blood can be sent in the tube used for collection.
Sample Stability:
Stable at ambient temperature during shipment. If sample is stored prior to shipment, it is stable refrigerated (2-8ºC) up to five days and frozen (-20ºC or lower) up to one year.
Clinical Relevance:
Sjögren’s syndrome (SjS) is a chronic autoimmune disease that primarily affects exocrine glands (mainly salivary and lacrimal) resulting in severe dryness of the mucosal surfaces.
This disease predominantly affects middle-aged women, but can also be observed in children, men and the elderly. The clinical presentation of SjS is heterogeneous and can vary from sicca symptoms to systemic disease. Classification criteria include both clinical and immunological parameters. Two different forms are observed: primary SjS and secondary SS, which is associated with other CTD.
Antinuclear antibody prevalence is estimated between 41.9 and 64% in this disease. These antibodies give a fine speckled pattern by IIF on Hep-2 cells. However, the presence of ANA is not mandatory for the diagnosis of the disease.
The two main antigens recognized by autoantibodies in SS patients are the SS-A/Ro (with two subunits, one of 52 kDa and one of 60 kDa) and the SS-B/La antigens. The detection of either anti- SS-A/Ro and/or anti- SS-B/La autoantibodies constitutes one of the classification criteria but their presence is not mandatory for diagnosis. Anti- SS-A/Ro autoantibodies are detected in 33–77.1% of primary SS, whereas anti- SS-B/La autoantibodies are present in 23–47.8% of primary SS. Anti- SS-A/Ro antibodies can be observed without anti-SS-B/La antibodies in patients’ sera, conversely anti- SS-B/La alone are rarely observed alone. can also occur in SLE with a prevalence between 36% and 64% and between 8% and 33.6% for anti- SS-A/Ro and anti- SS-B/La, respectively.
Between 75-95% of primary SjS patients are positive for rheumatoid factor. It has been shown to be correlated with prognosis. Specifically, IgA-targeting RF has been most commonly reported, with a prevalence ranging from 25.8% to 86% of primary SjS patients.
Selected References:
1. Didier K, Bolko L, Giusti D, Toquet S, Robbins A, Antonicelli F, Servettaz A. Autoantibodies Associated With Connective Tissue Diseases: What Meaning for Clinicians? Front Immunol. 2018 Mar 26;9:541.
2. Maślińska M, Mańczak M, Kwiatkowska B, Ramsperger V, Shen L, Suresh L. IgA immunoglobulin isotype of rheumatoid factor in primary Sjögren's syndrome. Rheumatol Int. 2021;41(3):643-649.
3. Brito-Zerón P, Baldini C, Bootsma H, Bowman SJ, Jonsson R, Mariette X, Sivils K, Theander E, Tzioufas A, Ramos-Casals M. Sjögren syndrome. Nat Rev Dis Primers. 2016 Jul 7;2:16047.
