Monoclonal gammopathy related to Sjogren syndrome: A key marker of disease prognosis and outcomes

被引:58
作者
Brito-Zeron, Pilar
Retamozo, Soledad
Gandia, Myriam [3 ]
Akasbi, Miriam [4 ]
Perez-De-Lis, Marta [5 ]
Diaz-Lagares, Candido
Bosch, Xavier [2 ]
Bove, Albert
Perez-Alvarez, Roberto [5 ]
Soto-Cardenas, Maria-Jose [3 ]
Siso, Antoni [6 ]
Ramos-Casals, Manuel [1 ]
机构
[1] Hosp Clin Barcelona, Serv Malalties Autoimmunes,ICMiD,IDIBAPS, Sjogren Syndrome Res Grp AGAUR,Dept Autoimmune Di, Lab Autoimmune Dis Josep Font,Inst Invest Biomed, E-08036 Barcelona, Spain
[2] Hosp Clin Barcelona, Dept Internal Med, ICMiD, IDIBAPS, E-08036 Barcelona, Spain
[3] Univ Cadiz, Dept Med, Hosp Puerto Mar, Cadiz, Spain
[4] Hosp Infanta Leonor, Dept Internal Med, Madrid, Spain
[5] Hosp Meixoeiro, Dept Internal Med, Vigo, Spain
[6] GESCLIN, Primary Care Res Grp, Ctr Assistencia Primaria ABS Les Corts AS, IDIBAPS, Barcelona, Spain
关键词
Primary Sjogren syndrome; Monoclonal gammopathy; Lymphoma; Cryoglobulinemia; Mortality; HEPATITIS-C VIRUS; UNDETERMINED SIGNIFICANCE; MULTIPLE-MYELOMA; ADULT-POPULATION; LONG-TERM; PREVALENCE; INFECTION; PROTEINS; IMMUNOGLOBULINS; CLASSIFICATION;
D O I
10.1016/j.jaut.2012.01.010
中图分类号
R392 [医学免疫学]; Q939.91 [免疫学];
学科分类号
100102 ;
摘要
Objective: To analyze the monoclonal expression of SS through the detection of serum monoclonal immunoglobulins (mIgs) in a large series of patients with Sjogren syndrome (SS), focusing on the etiology, characterization and evolution of the monoclonal band and the association with SS clinical expression and outcomes. Methods: Serum immunoelectrophoresis (IE) was performed to 408 consecutive patients who were evaluated by our unit between 1992 and 2011: 221 patients who fulfilled the 2002 American-European criteria for primary SS, 122 primary SS patients who fulfilled exclusively the 1993 European criteria and 65 patients with SS-associated hepatitis C virus infection. IE was performed at diagnosis and every year during the follow-up. Results: Of the 221 patients with primary SS, 48 (22%) had monoclonal gammopathy. In the control groups, the prevalence was 16% in patients with SS who fulfilled the 1993 criteria (p > 0.05) and 52% in SS-HCV patients (p < 0.001). Monoclonal bands were characterized in 47/48 patients with primary SS: IgG (n = 21), IgM (n = 16), IgA (n = 5) and free light chains (n = 5); the light chain was K in 28 patients and lambda in 19 (kappa:lambda ratio 1.5). Primary SS patients with monoclonal gammopathy had a higher prevalence of parotidomegaly (38% vs 20%, p = 0.021), vasculitis (21% vs 6%, p = 0.003), neurological involvement (42% vs 23%, p = 0.016), higher mean values of circulating gammaglobulins (23.4 vs 20.6%, p = 0.026), ESR (56.6 vs 37.6 mm/h, p = 0.003), a higher prevalence of RF (69% vs 50%, p = 0.022), low C3 levels (24% vs 11%, p = 0.028), low C4 levels (24% vs 7%, p = 0.003), low CH50 activity (28% vs 11%, p = 0.008) and cryoglobulins (23% vs 8%, p = 0.012) compared with those without monoclonal gammopathy. Of the 48 patients with primary SS and monoclonal gammopathy, 8 developed hematologic neoplasia after a mean follow-up of 10 years, a higher prevalence than observed in patients without monoclonal gammopathy (17% vs 5%, p = 0.009). Survival rates according to the presence or absence of monoclonal gammopathy were 83% and 97%, respectively (log rank 0.004). Conclusion: Monoclonal gammopathy was detected in 22% of patients with primary SS fulfilling the 2002 criteria, with mIgG kappa being the most frequent type of band detected. In HCV-associated SS patients, the prevalence was higher (52%) with IgM kappa being the most prevalent band detected. Monoclonal gammopathy was associated with a higher prevalence of parotid enlargement, extraglandular features, hypergammaglobulinemia, cryoglobulinemia and related markers (rheumatoid factor, hypocomplementemia), and with a poor prognosis (development of neoplasia and death). (C) 2012 Elsevier Ltd. All rights reserved.
引用
收藏
页码:43 / 48
页数:6
相关论文
共 44 条
[21]   DEMONSTRATION AND IDENTIFICATION OF MONOCLONAL PROTEINS IN THE URINE OF PATIENTS WITH SJOGRENS SYNDROME [J].
MOUTSOPOULOS, HM ;
COSTELLO, R ;
DROSOS, AA ;
MAVRIDIS, AK ;
PAPADOPOULOS, NM .
ANNALS OF THE RHEUMATIC DISEASES, 1985, 44 (02) :109-112
[22]   SJOGRENS-SYNDROME - AUTOIMMUNE EPITHELITIS [J].
MOUTSOPOULOS, HM .
CLINICAL IMMUNOLOGY AND IMMUNOPATHOLOGY, 1994, 72 (02) :162-165
[23]   ASSOCIATION OF SERUM IGM-CHI MONOCLONICITY IN PATIENTS WITH SJOGRENS-SYNDROME WITH AN INCREASED PROPORTION OF CHI-POSITIVE PLASMA-CELLS INFILTRATING THE LABIAL MINOR SALIVARY-GLANDS [J].
MOUTSOPOULOS, HM ;
TZIOUFAS, AG ;
BAI, MK ;
PAPADOPOULOS, NM ;
PAPADIMITRIOU, CS .
ANNALS OF THE RHEUMATIC DISEASES, 1990, 49 (11) :929-931
[24]   Primary Sjogren's syndrome: new clinical and therapeutic concepts [J].
Ramos-Casals, M ;
Tzioufas, AG ;
Font, J .
ANNALS OF THE RHEUMATIC DISEASES, 2005, 64 (03) :347-354
[25]   Hepatitis C virus, Sjogren's syndrome and B-cell lymphoma: linking infection, autoimmunity and cancer [J].
Ramos-Casals, M ;
De Vita, S ;
Tzioufas, AG .
AUTOIMMUNITY REVIEWS, 2005, 4 (01) :8-15
[26]  
Ramos-Casals M, 2011, LANCET 0823
[27]  
Ramos-Casals M, 2007, CURRENT DIAGNOSIS TR, P237
[28]   Primary Sjogren syndrome in Spain -: Clinical and immunologic expression in 1010 patients [J].
Ramos-Casals, Manuel ;
Solans, Roser ;
Rosas, Jose ;
Teresa Camps, Maria ;
Gil, Antonio ;
del Pino-Montes, Javier ;
Calvo-Alen, Jaime ;
Jimenez-Alonso, Juan ;
Mico, Maria-Luisa ;
Beltran, Juan ;
Belenguer, Rafael ;
Pallares, Lucio .
MEDICINE, 2008, 87 (04) :210-219
[29]   MONOCLONAL GAMMOPATHIES IN THE ADULT-POPULATION OF FINISTERE, FRANCE [J].
SALEUN, JP ;
VICARIOT, M ;
DEROFF, P ;
MORIN, JF .
JOURNAL OF CLINICAL PATHOLOGY, 1982, 35 (01) :63-68
[30]   Accurate Detection of Changes in Disease Activity in Primary Sjogren's Syndrome by the European League Against Rheumatism Sjogren's Syndrome Disease Activity Index [J].
Seror, Raphaele ;
Mariette, Xavier ;
Bowman, Simon ;
Baron, Gabriel ;
Gottenberg, Jacques Eric ;
Boostma, Hendrika ;
Theander, Elke ;
Tzioufas, Athanasios ;
Vitali, Claudio ;
Ravaud, Philippe .
ARTHRITIS CARE & RESEARCH, 2010, 62 (04) :551-558