Sjögren’s Syndrome (diagnosis (laboratory abnormalities (• the ESR is…
• the ESR is elevated
• anemia and /or leukopenia
• raised immunoglobulin levels
• circulating immune complexes
• many autoantibodies: SS-A(anti-Ro) and SS-B(anti-La)
• positive rheumatoid factor
- triad of cryoglobulinemia, hypocomplementemia, and purpura correlates strongly with severe disease complications and death.
- Mixed cryoglobulinemia and monoclonal rheumatoid factor may identify patients with an increased risk for the development of a B-cell lymphoma.
- SS should be suspected when symptoms of ocular and oral dryness have persisted on a daily basis for three or more months.
- commonly used tests for dry eye:
1 the Schirmer test
2.ocular surface staining: supported by staining scores of ≥4 by the von Bijsterveld method and ≥3 by the SICCA method
- Salivary gland imaging:
-MRI: “honeycomb” or “salt and pepper” pattern is thought to arise from fatty infiltration, fibrosis, ductal dilatation, and lymphoid infiltration of parotid lobules
less often employed are
-computed tomography (CT)
-parotid gland contrast sialography.
- Exclusion criteria:
•Prior head and/or neck irradiation
•Infection with hepatitis C virus (HCV)
•Acquired immunodeficiency syndrome (AIDS)
•use of anticholinergic medications
•IgG4 –related disease: findings: IgG4 plasmacytic infiltration, extensive fibrosis, and obliterative phlebitis, Elevated serum IgG4 levels and eosinophilia are present.
- ACR classification criteria for SS:
at least two of the following three findings:
●Positive serum anti-Ro/SSA and/or anti-La/SSB antibody testing OR a positive rheumatoid factor and an antinuclear antibody titer ≥1:320
●Ocular staining score ≥3 (assuming the individual is not concurrently using daily eye drops)
●Presence of focal lymphocytic sialadenitis with a focus score ≥1 focus/4 mm2 in labial salivary gland biopsy samples
Signs and Symptoms
- persistent symptoms of dry eyes and/or mouth.
- parotid gland enlargement.
- unexplained increase in dental caries
- abnormal results of specific serologic tests (eg, anti- Ro/SSA and/or anti-La/SSB antibodies, rheumatoid factor, and hyperglobulinemia)
- secondary to lacrimal gland dysfunction:
• dry, irritated eyes with foreign-body sensation
• gritty sensation
• burning sensation
• painful eyes
• corneal abrasions
• erythematous eyes
- secondary to salivary gland dysfunction:
• dry mouth
• oral sores
• dental caries
• lingual and labial fissures
• dysphagia, difficulties in swallowing dry food
• taste and smell may be diminished
- secondary to other exocrine dysfunction:
• Dryness may also develop in the skin and in mucous membranes of the nose, throat, larynx, bronchi and vagina.
• Dryness of the respiratory tract may cause cough.
• Pancreatitis and pancreatic malabsorption
- Extraglandular manifestations:
• arthralgia or polyarthritis (nonerosive and nondeforming)
• Raynaud’s phenomenon
• Renal tubular defects
• Interstitial lung disease (most common pulmonary abnormality)
• lymph nodes enlargment and bone marrow involvement (lymphoma)
• skin rashes
• prolonged fatigue
- or dry mouth:
Salagen (pilocarpine hydrochloride)
NeutraSal (artificial salivas)
good dental care
maintenance of good hydration by taking regular
sips of water and drinking sugar-free liquids;
avoidance of oral irritants (eg, coffee, alcohol, nicotine)
avoidance of acidic drinks (carbonated beverages, juices).
- for dry eye:
artificial tears or surgical punctal occlusion for dry eyes
Restasis (cyclosporine ophthalmic emulsion)
Lacrisert (hydroxypropyl cellulose ophthalmic insert)
- vaginal dryness: topical lubricants
low doses of prednisolone
- viruses: EBV, human herpes virus-6, HIV, HCV.
- molecular mimicry
- genes: DRw52 and DR3
- lymhocytic infiltration of glandular and nonglandular organs.
- CD4+ helper T cells, B cells.
- chronic autoimmune, inflammatory disorder of exocrine gland dysfunction and other systemic features.
- characterized by sicca symptoms - dry eyes (xerophthalmia) and dry mouth (xerostomia),
due to failure of salivary and mucosal glands, often preceded by salivary gland swelling.
- may occur as a secondary disorder (secondary Sjógren syndrome) in patients with systemic lupus or, rheumatoid arthritis, systemic sclerosis and primary biliary cirrhosis.
- common in women in their 50s and 60s
- increased risk of non-Hodgkin’s lymphoma