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Sjogren's syndrome (SS)                  
SX |  DX | RX     
is a chronic immunemediated inflammatory disorder characterized by lymphocytic infiltration of the lacrimal and salivary glands associated with the clinical features of keratoconjunctivitis sicca (xerophthalmia -dryness of eyes) and xerostomia (dryness of mouth).
SS exists in both a primary and secondary form (associated with other collagen disease).

Sicca Syndrome - dry mouth & dry eyes.

2 -5% of people aged 60 and above have primary SS.





Extraglandular manifestations of SS are more common in patients with primary than secondary SS, especially those with antibodies to Ro(SS-A) and La(SS-B);



Ophthalmologic (Xerophthalmia)

Oral (Xerostomia)


Differential Diagnosis of Sicca Syndrome (dry mouth & eyes)



The treatment of dry eyes  & mouth is largely symptomatic and includes

Patients with extraglandular manifestations such as rashes and arthralgias are usually treated with systemic corticosteroids or hydroxychloroquine (5-7 mg/kg per day) may prove helpful.

Patients with secondary SS should receive appropriate therapy for their associated connective tissue disease. Patients with lymphoma should be treated in consultation with an oncologist.

More refractory symptoms of arthritis may respond to low-dose weekly methotrexate (7.5-15 mg/week) in the presence of folic acid supplementation. Leflunomide, another disease-modifying antirheumatic drug, was recently approved for use in rheumatoid arthritis. This drug may have use in Sjögren syndrome as it was very effective in animal models of autoimmune disease including Sjögren syndrome.Also, inhibitors of TNF may prove useful in modulating the immune response.

In patients with life-threatening vasculitis, high-dose corticosteroids and parenteral cyclophosphamide may be required.


The ophthalmologic and oral manifestations of SS are generally nonprogressive. Patients with primary SS are at increased risk of developing lymphoproliferative disorders, including non-Hodgkin's lymphoma; in one study, the relative risk was estimated to be greater than 40. Patients with splenomegaly, bilateral parotid enlargement, and a history of radiation treatment to shrink these enlarged glands were at especially high risk. The lymphomas are B cell-derived.

Young women with primary SS, especially those with antibodies to Ro(SS-A), should be counseled about the increased risk of delivering a child with neonatal SLE and congenital complete heart block; such women when pregnant should be followed closely by an obstetrician expert in high-risk pregnancies.

Bennett: Cecil Textbook of Medicine, 20th ed., 1996
Frederick Vivino - The Female Patient August 1999, 24:65