TY - JOUR
T1 - The role of ectopic germinal centers in the immunopathology of primary Sjögren's syndrome: a systematic review
AU - Risselada, Anna P.
AU - Looije, Marjolein F.
AU - Kruize, Aike A.
AU - Bijlsma, Johannes W. J.
AU - van Roon, Joel A. G.
PY - 2013
Y1 - 2013
N2 - To determine whether the presence of germinal centers (GCs) in salivary glands of patients with primary Sjögren's syndrome (pSS) is related to the severity of disease course and distinct immunopathology features. A systematic search was performed in September 2011 for terms and synonyms of Sjögren's syndrome and germinal centers. A total of 80 articles were retrieved, of which 16 were included for (meta-) analysis. GC morphology was present in a mean ± SD 25.1 ± 5.0% of pSS patients. Mean lymphocyte focus scores were 1.25 points higher in patients with GCs as compared to those without GCs. Saliva production was reduced in patients with GCs, although this did not reach statistical significance. Percentages of patients positive for rheumatoid factor, anti-Sjögren's syndrome A (SSA), and anti-Sjögren's syndrome B (SSB) antibodies were significantly higher in patients with GCs (mean increase, 15%, 18%, and 18%, respectively). Additionally, patients with GCs were characterized by enhanced levels of local and systemic proinflammatory mediators. Importantly, these patients have a higher risk of lymphoma development (14% versus 1%). Patients with GCs are characterized by more severe disease, although the small number of studies and their design hamper generalizability of results. The precise mechanisms that contribute to the development and persistence of germinal centers in pSS are largely unknown. This and the strongly increased risk of lymphoma development warrant intensive studies for the role of germinal centers in the immunopathology of pSS
AB - To determine whether the presence of germinal centers (GCs) in salivary glands of patients with primary Sjögren's syndrome (pSS) is related to the severity of disease course and distinct immunopathology features. A systematic search was performed in September 2011 for terms and synonyms of Sjögren's syndrome and germinal centers. A total of 80 articles were retrieved, of which 16 were included for (meta-) analysis. GC morphology was present in a mean ± SD 25.1 ± 5.0% of pSS patients. Mean lymphocyte focus scores were 1.25 points higher in patients with GCs as compared to those without GCs. Saliva production was reduced in patients with GCs, although this did not reach statistical significance. Percentages of patients positive for rheumatoid factor, anti-Sjögren's syndrome A (SSA), and anti-Sjögren's syndrome B (SSB) antibodies were significantly higher in patients with GCs (mean increase, 15%, 18%, and 18%, respectively). Additionally, patients with GCs were characterized by enhanced levels of local and systemic proinflammatory mediators. Importantly, these patients have a higher risk of lymphoma development (14% versus 1%). Patients with GCs are characterized by more severe disease, although the small number of studies and their design hamper generalizability of results. The precise mechanisms that contribute to the development and persistence of germinal centers in pSS are largely unknown. This and the strongly increased risk of lymphoma development warrant intensive studies for the role of germinal centers in the immunopathology of pSS
U2 - https://doi.org/10.1016/j.semarthrit.2012.07.003
DO - https://doi.org/10.1016/j.semarthrit.2012.07.003
M3 - Review article
C2 - 22995442
SN - 0049-0172
VL - 42
SP - 368
EP - 376
JO - Seminars in Arthritis and Rheumatism
JF - Seminars in Arthritis and Rheumatism
IS - 4
ER -