RT journal article
T1 Sjögren syndrome or sjögren disease? The histological and immunological bias caused by the 2002 criteria
A1 Ramos Casals, Manuel
A1 Brito Zerón, Pilar
A1 Perez De LiS, Marta
A1 Jimenez, Iratxe
A1 Blanco, Maria Jose
A1 Bove, Albert
A1 Soto Cardenas, María José
A1 Akasbi, Miriam
A1 Diaz, Candido
A1 Sentís, Juan
A1 Siso, Antoni
A2 Cirugía
A2 Medicina
K1 Sjogren's syndrome
K1 Anti-Ro/SS-A antibodies
K1 Salivary gland biopsy
AB The current 2002 classification criteria do not cover the broad clinical and immunological heterogeneity of primary Sjögren syndrome (SS), since five of the six criteria focus exclusively on glandular involvement and the remaining criterion is the mandatory presence of anti-Ro/La antibodies. The aim of this study was to analyze the clinical features of patients with a well-established diagnosis of primary SS who do not fulfill the 2002 classification criteria. Five hundred seven patients diagnosed with primary SS (1993 criteria) were consecutively included and followed up. Two hundred twenty-one (44%) patients did not fulfill the 2002 criteria. These patients were older at diagnosis (p < 0.001) and had a lower frequency of parotid enlargement (p = 0.002), fever (p = 0.041), arthritis (p = 0.041), vasculitis (p = 0.050), peripheral neuropathy (p = 0.002), cranial nerve involvement (p = 0.015), raised erythrocyte sedimentation rate ( ESR) levels (p < 0.001), anemia (p < 0.001), leukopenia (p = 0.037), hypergammaglobulinemia (p < 0.001), positive rheumatoid factor ( RF; p = 0.002), and cryoglobulinemia (p = 0.049) in comparison with those fulfilling 2002 criteria. However, there were no significant differences in the prevalence of sicca features, diagnostic tests, overall systemic involvement, antinuclear antibodies , complement levels, development of B-cell lymphoma, or survival. Patients with anti-Ro antibodies had the highest frequencies of systemic features, hematological abnormalities, and altered immunological markers. In conclusion, patients fulfilling the 2002 criteria, who have either a specific histological diagnosis (lymphocytic infiltration) or highly specific autoantibodies (Ro/La), might well be considered to have Sjögren "disease." In contrast, etiopathogenic mechanisms other than lymphocytic-mediated epithelial damage could be involved in patients with negative Ro and negative biopsy, in whom the term Sjögren "syndrome" seems more adequate.
PB Humana Press
SN 1080-0549
YR 2009
FD 2009-07
LK http://hdl.handle.net/10498/39214
UL http://hdl.handle.net/10498/39214
LA eng
DS Repositorio Institucional de la Universidad de Cádiz
RD 07-nov-2026