Rheumatology, Vol 38, 245-253, Copyright © 1999 by British Society for Rheumatology
BK Davidson, CA Kelly and ID Griffiths
OBJECTIVE: Although primary Sjogren's syndrome is often a benign condition,
characterized by lymphocytic infiltration of salivary and lacrimal glands,
some patients develop systemic features. We have previously found that
anti-Ro antibodies identified patients with more systemic disease, with
increased incidence of parotid swelling, lymphadenopathy and lymphoma.
METHODS: We have followed up a cohort of 100 patients over 10 yr, to
establish whether the phenotypic expression of disease changed, and whether
the different autoantibody patterns expressed at presentation could be used
to predict outcome. RESULTS: While seronegative patients (ANA, RF, Ro and
La negative) remained polysymptomatic, they did not develop systemic
complications or serological changes. Thirty-nine per cent of ANA- or
RF-positive patients who were negative for Ro and La were given revised
diagnoses over the follow-up period, including rheumatoid arthritis,
systemic lupus erythematosus, mixed connective tissue disease and
scleroderma. Parotid swelling and lymphadenopathy were more common in
Ro/La-positive patients, where the relative risk of developing
non-Hodgkin's lymphoma was 49.7. CONCLUSION: Both HLA B8 and DR3 were
present in 79% of Ro/La- positive patients, but were found together in only
4% of seronegative patients, supporting the view that these clinical
subgroups of primary Sjogren's syndrome are both serologically and
immunogenetically distinct. Patients who are initially autoantibody
(including Ro and La) negative do not evolve into 'systemic' Sjogren's
syndrome or other connective tissue diseases.
ORIGINAL PAPERS
Primary Sjogren's syndrome in the North East of England: a long-term follow-up study
Musculoskeletal Unit, Freeman Hospital, Newcastle upon Tyne, UK.
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