Sjogren's syndrome associated with SLE: clinical and laboratory profiles

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Arthritis Rheum. 2004 Mar;50(3):882-91.

Sjogren's syndrome associated with systemic lupus erythematosus:

clinical and laboratory profiles and comparison with primary Sjogren's

syndrome.

Manoussakis MN, Georgopoulou C, Zintzaras E, Spyropoulou M, Stavropoulou

A, Skopouli FN, Moutsopoulos HM.

Department of Pathophysiology, School of Medicine, National University

of Athens, Athens, Greece. menman@...

OBJECTIVE: To address the clinical, serologic, pathologic, and

immunogenetic features of sicca syndrome that occurs in systemic lupus

erythematosus (SLE), as well as its similarities to, and differences

from, sicca syndrome that occurs in primary Sjogren's syndrome (SS).

METHODS: A cohort of 283 consecutive unselected SLE patients was

evaluated for the presence of associated SS using the American-European

classification criteria. Clinical and laboratory parameters in SLE

patients with SS (SLE-SS) were compared with those in SLE patients

without SS (SLE-no SS) and with a group of 86 unselected patients with

primary SS. RESULTS: SS was identified in 26 SLE patients (9.2%); the SS

preceded the development of lupus in 18 of them (69.2%). Compared with

the SLE-no SS group, patients with SLE-SS were significantly older, had

a higher frequency of Raynaud's phenomenon, anti-Ro/SSA, anti-La/SSB,

and rheumatoid factor, but had a significantly lower frequency of renal

involvement, lymphadenopathy, and thrombocytopenia. Compared with the

primary SS group, SLE-SS patients displayed a clinically similar sicca

syndrome, but were significantly younger and had an increased frequency

of perivascular infiltrates in the salivary glands associated with

anticardiolipin antibodies in the serum. SLE-SS patients had a high

frequency of the DRB1*0301 allele. This HLA profile distinguished the

SLE-SS group from the SLE-no SS group, who had an increased frequency of

DRB1*1501 and DQB1*0602 alleles, but was similar to the HLA profile of

the primary SS group, who had an increased frequency of DRB1*0301.

CONCLUSION: SLE-SS appears to constitute a subgroup of patients with

distinct clinical, serologic, pathologic, and immunogenetic features, in

whom SS is expressed as an overlapping entity and is largely similar to

primary SS.

PMID: 15022331

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