| Objective:Primary Sj?gren's syndrome?p SS?is a common systemic autoimmune disease that mainly affects the exocrine glands and usually presents as persistent dry keratoconjunctivitis and xerostomia due to functional impairment of the salivary and lachrymal glands.Additionally,systemic manifestations may occur such as interstitial lung disease,renal disease,neuropathy and hematological abnormalities,which could increase disability rate and risk of death.The pathogenesis is still unclear,however,a growing number of evidence indicates that excessive activation of B cells plays an important role in the pathogenesis of p SS,hypergammaglobulinaemia and a variety of autoantibodies will appear in p SS patients due to B cell activation.p SS with hypergammaglobulinaemia had high prevalence in the clinic.Many studies have explored the relationship between autoantibodies and systemic involvement and prognosis in patients with p SS.However,there are few systematic researches on the clinical characteristics and treatment of p SS patients with hyperimmunoglobulinemia.This study retrospectively analyzed the systemic involvement characteristics of p SS patients with hyperimmunoglobulinemia,and compared the clinical differences in the treatment of patients with hyperimmunoglobulinemia and those without,to provide an evidence on whether p SS patients need aggressive treatment.Methods:The clinical data of p SS patients who were hospitalized in the department of rheumatology in Affiliated Hospital of Inner Mongolia Medical University from January 1,2009 to December 31,2019 were collected,and 389patients who met the inclusion and exclusion criteria were divided into the group with hyperimmunoglobulinemia?p SS-HG group?and the group without hyperimmunoglobulinemia?p SS-non-HG group?according to the serum immunoglobulin lg G>20g/L.Comparing the clinical characteristics,including general clinical data,clinical manifestations,laboratory tests,and disease activity between two groups,and also the differences in drug treatment between two groups.Results:?1?Of the 389 patients with p SS,108?27.8%?presented hyperimmunoglobulinemia,102?94.4%?were women,with an average age of 54.1±12.3 years.The median course was 36 months.?2?In univariate analysis,p SS-HG patients were younger?54.1±12.3 vs 60.3±12.4,P=0.001?and had a higher frequency of extraglandular manifestations compared with p SS-non-HG patients?94.4%vs 70.1%,P=0.000?,as did higher ESSDAI score and Clin ESSDAI score[8?6.0,12.0?vs 3?1.0,6.0?,8?4.0,11.5?vs 3?0.0,6.0?,respectively;P=0.000].In terms of specific ESSDAI domains,p SS-HG patients had a higher frequency of systemic activity in the constitutional?8.3%vs 2.1%,P=0.004?,parotid enlargement?6.5%vs 1.4%,P=0.007?,lymphadenopathy?18.5%vs 3.9%,P=0.000?,arthritis?14.8%vs 6.8%,P=0.013?),Cutaneous vasculitis?16.7%vs 3.6%,P=0.000?,anemia?24.1%vs 8.9%,P=0.000?and leukocytopenia?25.0%vs 13.9%,P=0.009?.With respect to laboratory parameters,p SS-HG patients had a higher frequency of high ESR level?89.8%vs50.6%,P=0.000?,high Ig A level?38.9%vs 17.4%,P=0.000?and positive autoantibodies[anti-SSA?97.2%vs 83.6%,P=0.000?),anti-SSB?67.6%vs 39.1%,P=0.000?,anti-ANA?95.4%vs 69.0%,P=0.000?,and RF-Ig M?79.1%vs 33.1%,P=0.000?].Predictor factors associated with hyperimmunoglobulinemia were positive anti-ANA[OR 9.189?95%CI3.316,25.462?,P=0.000],high ESR level[OR 6.675?95%CI 3.161,14.095?,P=0.000],arthritis[OR 3.219?95%CI 1.216,8.520?,P=0.019],lymphadenopathy[OR 3.045?95%CI1.106,8.387?,P=0.031],high Ig A level[OR 2.504?95%CI 1.336,4.691?,P=0.004],positive Anti-SSB[OR 2.030?95%CI 1.133,3.638?,P=0.017],higher Clin ESSDAI score[OR 1.074?95%CI 1.016,1.135?,P=0.012],and age[OR 0.957?95%CI 0.936,0.979?,P=0.000]in multivariate analysis.?3?Compared with the p SS-non-HG patients,there were more patients receiving glucocorticoid?prednisone?30 mg/d??55.6%vs 34.9%,P=0.000?,Cyclophosphamide?28.7%vs 6.8%,P=000?and iguratimod?8.3%vs 2.1%,P=0.008?at baseline in the p SS-HG group.Conclusion:p SS with hyperimmunoglobulinemia is common in the clinic,usually with extensive systemic involvement and immunological abnormalities.,especially when p SS patients had younger age,arthritis,lymphadenopathy,high ESR level,positive anti-ANA antibodies and anti-SSB antibodies.It should be carefully take to assess extraglandular injury and disease activity.p SS patient with hyperimmunoglobulinemia have been given more aggressive immunosuppressive therapy in clinical practice,but further prospective controlled studies are needed. |
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