| [Objective] In recent years,the concept of monoclonal gammopathy of renal significance(MGRS)has been paid more and more attention.MGRS-related renal lesions represents a class of kidney disease caused by monoclonal immunoglobulin which secreted by non-malignant B cells or plasma cell clones.This study is a retrospective study,which summarizes the clinical,pathological,testing and treatment characteristics of MGRS-related renal lesions in our center,and explores a new method for diagnosing MGRS-related renal lesions.[Methods] Patients diagnosed with MGRS-related renal lesions or monoclonal gammopathy of undetermined significance(MGUS)with other types of renal lesions,from January 2014 to December 2018,are included in the study.Inclusion criteria: patients with both monoclonal gammopathy and renal lesion;exclusion criteria: patients without renal biopsy or confirmed malignant hematologic disease.Methods: Collect clinical and pathological data of patients,including urine protein,liver and kidney function,serum protein electrophoresis(SPEP),serum/urine immunofixation electrophoresis(IFE),serum free light chain(sFLC),renal pathology,treatment regimen,remission,etc.Use the the guidelines for the diagnosis and treatment of systemic light chain amyloidosis to evaluate the efficiency of treatment.Try to find a new method to diagnose MGRS-related renal lesions by non-invasive examination.[Results] 142 patients were enrolled in the study,72 in the MGRS group and 70 in the MGUS group.MGRS group: male: female = 1.22:1,mean age 57.5 years old,clinical features include nephrotic syndrome(61.1%),low ?(25%),hypotension(5.6%).Serum IFE : 50% patients shows IgG ? and urine IFE shows ? type: ? type = 3:1.The result of renal pathology was mainly light chain amyloidosis(79.2%)and light chain deposition disease(LCDD)(13.9%),and different pathology shows characteristic performance which can be observed by light microscopy,immunofluorescence and electron microscopy.For example,in light chain amyloidosis,apple green birefringence can be observed under polarized light microscopy in Congo red stained specimens and under the electron microscope,the disordered,randomly arranged unbranched fine fibers can be seen in the glomerular basement membrane,vessels,interstitial,and/or tubule basement membrane.While for LCDD,immunofluorescence is important for diagnosis,showing light chain(?majority)in the glomerular mesangial area(nodules),renal tubular basement membrane,diffuse linear deposition of vessel wall.Combined sFLC and/or low ? can be used to diagnose MGRS.The ROC curve shows that ?/? is used to diagnose type? MGRS with a critical value of 2.67 and an AUC of 0.785.The combined predictive factor = ?/?+(0.009 /0.140)? assisted diagnosis of type ? MGRS,with a critical value of 3.96 and an AUC of 0.742.Compared with traditional chemotherapy,the bortezomib based regimen has higher haematological and renal remission rates(Bortezomib group 19 patients vs.Non-bortezomib group 10 patients,hematologic remission 73.7% vs.40%,renal remission 36.8% vs.0%).Bortezomib based regimen showes advantage in decreasing urine protein,protecting renal function,reducing mortality and improving prognosis.[Conclusion] The incidence of monoclonal gammopathy increases with age.If renal lesion is involved,the possibility of MGRS should be taken into account.Renal pathology plays an important role in the diagnosis of such diseases,and sFLC and other tests can assist diagnosis.Among all the MGRS-related renal lesions,light chain amyloidosis accounted for more than 2/3,followed by LCDD,and the remaining pathological types(such as heavy chain deposition disease(HCDD),light chain renal tubular disease(LCPT),immunotactoid glomerulonephritis,cryoglobulinaemic glomerulonephritis,C3 glomerulopathy with monoclonal gammopathy,etc.)were rare.Bortezomib is effective in the treatment of MGRS-related renal lesions,and improves both the hematologic remission rate and renal remission rate. |
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