[Primary Sjögren's syndrome: clinical and immunological characteristics of 114 patients].
BACKGROUND
To analyze the clinical and immunological characteristics of a series of 114 patients with primary Sjögren's syndrome (PSS), and to evaluate the different diagnostic criteria and the association to lymphoproliferative disorders.


METHODS
We included 114 patients (108 female and 6 male) with a diagnosis of PSS. All patients fulfilled the 1993 European Community criteria for the diagnosis of PSS and 76 patients fulfilled the San Diego Criteria.


RESULTS
Mean age was 51 years with a mean follow-up of 7.3 years. The commonest clinical manifestation at onset (70%) was xerostomia/xerophtalmia (sicca syndrome). Extra glandular involvement was articular in 42% of cases, neurologic (35%), respiratory (21%) and hepatic (13%). Eleven patients (9%) developed vasculitis, and three (2%) developed a lympho-proliferative disorder. No statistically significant differences regarding symptoms at onset, frequency of glandular or extra glandular manifestations and severity of disease were observed between the two diagnostic criteria groups. HCV infection was associated with vasculitis (p < 0.001; OR: 20.6; CI 95%, 3.2-129) and lymphoproliferative disorders (p < 0.001).


CONCLUSIONS
The clinical evolution of PSS does not vary when using different diagnostic criteria (San Diego and European Community criteria). A subset of patients with vasculitis and lymphoproliferative diseases is found to have an associated HCV infection.
Selva O'Callaghan
A
A
Medicina Interna, Hospital General Vall d'Hebron, Barcelona, Spain.
Bosch Gil
J A
JA
Solans Laque
R
R
Segura García
A
A
Armadans Gil
L
L
Mijares Boeckh-Behrens
T
T
Vilardell Tarrés
M
M
D000293:Adolescent; D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012859:Sjogren's Syndrome / Q000150:complications / Q000175:diagnosis* / Q000276:immunology*