Last edited 02/2021 and last reviewed 02/2021
Primary Sjogren's syndrome is the association of
- keratoconjunctivitis sicca and/or xerostomia, with
- rheumatoid arthritis or other connective tissue disorder
Sicca refers to dryness most often involving the eyes and mouth due to inflammation and resultant pathology of the lacrimal and salivary glands.
Salivary gland biopsy shows sialadenitis. Involvement of other secretory glands is common causing dyspareunia, dry skin, dysphagia, otitis media and pulmonary infection.
Up to one-half of affected individuals also develop extra-glandular involvement implying the occurrence of signs and symptoms in organs distinct from the salivary and lacrimal glands including the joints, skin, lungs, gastrointestinal (GI) tract, nervous system, and kidney
Other possible features include dental caries, attacks of monilial stomatitis, neuropathy, hepatosplenomegaly and renal involvement.
Associations include renal tubular acidosis, adverse drug reactions and lymphoma.
Secondary Sjogren syndrome frequently occurs in conjunction with other autoimmune disorders including rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE)
- study evidence suggests that secondary Sjogren's syndrome in rheumatoid arthritis occurs in 17-31% of patients (1,2); although it has been stated that secondary Sjogren's syndrome affects 50% of patients with rheumatoid arthritis (3)
Aetiology of Sjogren syndrome is unknown
- a genetic predisposition involving the major histocompatibility (MHC) locus is seen
- association with HLA-B8, HLA-DR3, HLA-DRW52
- Andonopoulos AP, Drosos AA, Skopouli FN, Acritidis NC, Moutsopoulos HM. Secondary Sjogren’s syndrome in rheumatoid arthritis. J Rheumatol. 1987;14:1098-1103
- Carmona L, Gonzalez-Alvaro I, Balsa A, Angel Belmonte M, Tena X, Sanmarti R. Rheumatoid arthritis in Spain: occurrence of extra-articular manifestations and estimates of disease severity. Ann Rheum Dis. 2003;62:897-900. doi: 10.1136/ard.62.9.897
- Arthritis Research Campaign (2000). Rheumatic Disease In Practice, September 2000, 3.
management of Sjogren's syndrome