Monoclonal gammopathy related to Sj枚gren syndrome: A key marker of disease prognosis and outcomes

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• 作者：Pilar Brito-Zeró ; n^a ; ¹ ; Soledad Retamozo^a ; ¹ ; Myriam Gandí ; a^c ; Miriam Akasbi^d ; Marta Pé ; rez-De-Lis^e ; Candido Diaz-Lagares^a ; Xavier Bosch^b ; Albert Bové ; ^a ; Roberto Pé ; rez-Alvarez^e ; Marí ; a-José ; Soto-Cá ; rdenas^c ; Antoni Sisó ; ^f ; Manuel Ramos-Casals^a ; ^{mramos@clinic.ub.es}
• 关键词：Primary Sjö ; gren syndrome ; Monoclonal gammopathy ; Lymphoma ; Cryoglobulinemia ; Mortality
• 刊名：Journal of Autoimmunity
• 出版年：2012
• 期刊代码：101_08968411
• 类别：imm
• 出版时间：August, 2012
• 卷：39
• 期：1-2
• 页码：43-48
• 文件大小：230 K

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 Sj枚gren 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聽>聽0.05) and 52%in SS-HCV patients (p聽<聽0.001). Monoclonal bands were characterized in 47/48 patients with primary SS: IgG (n聽=聽21), IgM (n聽=聽16), IgA (n聽=聽5) and free light chains (n聽=聽5); the light chain was 魏 in 28 patients and 位 in 19 (魏:位 ratio 1.5). Primary SS patients with monoclonal gammopathy had a higher prevalence of parotidomegaly (38%vs 20%, p聽=聽0.021), vasculitis (21%vs 6%, p聽=聽0.003), neurological involvement (42%vs 23%, p聽=聽0.016), higher mean values of circulating gammaglobulins (23.4 vs 20.6%, p聽=聽0.026), ESR (56.6 vs 37.6聽mm/h, p聽=聽0.003), a higher prevalence of RF (69%vs 50%, p聽=聽0.022), low C3 levels (24%vs 11%, p聽=聽0.028), low C4 levels (24%vs 7%, p聽=聽0.003), low CH50 activity (28%vs 11%, p聽=聽0.008) and cryoglobulins (23%vs 8%, p聽=聽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聽=聽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魏 being the most frequent type of band detected. In HCV-associated SS patients, the prevalence was higher (52%) with IgM魏 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).