ABSTRACT
Objective To investigate the clinical characteristics,immunological features,treatment and follow-ups of Sj(o)gren's syndrome-associated monoclonal gammopathy (SS-MG).Methods A retro-spective,case-control study was conducted for 18 cases diagnosed with SS-MG and 36 age-and sex-matched non-MG-SS patients from Janurary 2010 to Janurary 2017 in Peking University People's Hospital.The clinical and laboratory features,treatment and follow-ups were recorded and compared.Comparisons between groups were made using t test for normally distributed numerical data,Mann-Whitney U test for non-normally distributed numerical data,and Pearson Chi-square,continuity correction or Fisher's exact tests for categorical data.Results SS patients,when complicated with MG,had significantly increased level of TP [(78± 11) g/L,(71±10) g/L,t=-2.382,P=0.021] and erythrocyte sedimentation rate (ESR) [52.5(45.3) mm/1 h,33.0(42.5) mm/1 h,Z=-2.179,P=0.029],higher prevalence of urine NAG positivity [75％(9/12),28％(7/25),x2=7.298,P=0.007],hypoglobulinemia [33％(6/18),3(1/36),x2=7.407,P=0.006] and thrombotic events [17％(3/18),0％(0/36),P=0.033],and less previous exposure to glucocorticoid [22％(4/18),64％(23/36),x2=8.333,P=0.004],compared to the control group.Primary SS patients complicated with MG had significantly higher ESSDAI [26.0(25.0),12.0 (9.0),Z=-2.724,P=0.006] and Clin EULAR Sj(o)gren's syndrome disease activity index (ESSDAI) [24.0(25.0),10.5 (10.0),Z=-2.523,P=0.011].Among the 18 patients,2 were diagnosed with multiple myeloma,1 was diagnosed with non-Hodgkin lymphoma,and the left were diagnosed as MG of undetermined significance (MGUS).Ten patients were followed up,among whom 2 patients with MGUS experienced increased levels of M protein,newly developed genetic abnormalities,and renal involvement.Conclusion SS patients may be complicated with MG.MGUS is the most common form.However,malignant hematologic disorders are revealed as well.In SS patients with high serum TP and ESR,hypoglobulinemia,tubulointerstitial kidney involvement and unexplained thrombotic events,especially in those with high disease activity,so MG should be an alert for further work-ups,monitoring and treatment.
ABSTRACT
Objective To investigate the role of stem cell factor (SCF) and mast cells (MC) in the pathogenesis and progression of dermal lesions caused by chronic renal failure.Methods Thirty-six Wistar rats were randomly divided into model group (adenine lavage at a dose of 150 mg·kg-1·d-1) and control group (physiological saline lavage at equal volume).Six rats from each group were sacrificed respectively at week 4,8 and 12.The intensity of MC infiltration was examined by toluidine blue staining. The expression of SCF was detected by immunohistochemistry and real-time fluorescence quantitative PCR. Results Compared with control group,the intensity of MC and the expression of SCF were significantly higher in dermal tissue of model group (P＜0.O1,respectively),and they were increased with time.In the model group,the number of MC infiltration was positively correlated with both the protein expression of SCF (r=0.81,P＜O.01) and the level of SCF mRNA (r=0.65,P＜0.01). Conclusion The increased SCF and MC may participate in the pathogenesis and progression of dermal lesions caused by chronic renal failure.