Clinical And Pathological Analysis Of 75 Patients With Renal Amyloidosis

Objective:Renal amyloidosis is the second leading cause of secondary kidney disease.The etiology of the disease is complex,the early manifestation of kidney damage is not specific,and the clinical detection rate is low.If the patient cannot intervene early,the prognosis is poor.Renal biopsy is the “gold standard” for diagnosis.It includes characteristic amyloid deposits in the kidney under light microscope,electron microscope,pathological morphology changes,and pathological classification.The prognosis assessment has hints.In 2010,some scholars proposed related glomerular lesion patterns,pathological scoring and grading systems,which need to be further clinically verified.1.To analyze the clinical and pathological data of 75 patients with renal amyloidosis,summarize the clinical and pathological characteristics of renal amyloidosis;2.To explore the relationship between the clinical and renal pathological manifestations of the disease using a histopathological classification,scoring and grading system by Sait Sen.Methods:1.Case selection: select 75 cases of renal amyloidosis from January 1993 to November 2019 in our center,collecting clinical and pathological data.2.Clinical data:(1)Normal informationIncluding gender,age(average age,number of cases> 50 years),time from symptom onset to diagnosis,clinical symptoms(edema,foamy urine,hypertension,fatigue,weight loss,etc.),clinical signs(abdominal effusion,chest cavity)Fluid accumulation,enlarged tongue,hepatomegaly,splenomegaly,etc.);(2)Laboratory examination,Clinical stage,Biochemical indicators include urinary protein quantification,serum albumin,blood creatinine,hemoglobin,urea nitrogen,etc;clinical staging according to biochemical indicators,clinical staging standards:(1)proteinuria period: urinary protein quantification<3.5g/24h;(2)nephrotic syndrome period : Quantitative urine protein?3.5g/24 h,serum albumin <30g / L;(3)renal insufficiency: blood creatinine>133?mol/ L.(3)Whether to combine other diseases.Whether it is associated with lymphatic hematopoietic system diseases(multiple myeloma,M proteinemia,etc.),primary glomerular diseases(IgA nephropathy,membranous nephropathy,membranoproliferative glomerulonephritis,etc.)3.Pathologicaldata:(1)light microscopic morphological manifestationsRenal tissue specimens were stained with HE,PASM,PAS and MASSON,and under the light microscope the characteristics of renal pathological morphology(degree of mesangial hyperplasia,basement membrane thickening,etc.).(2)Immunomarker characteristicsDirect immunofluorescence method was used to detect immunoglobulin IgG,IgA,IgM,complement C3,C1 q,fibrinogen Fib,fluorescence intensity and deposition form were observed under a fluorescence microscope,immunofluorescence staining intensity was evaluated by semi-quantitative method,from negative to strongest,Counted as 0 to3+.(3)Electron microscopic manifestationsObserve the ultrastructural morphology of the glomeruli under the electron microscope(whether the mesangial area is widened or the basement membrane is thickened,etc.)Subcutaneous,renal tubules,renal interstitium),whether other deposits(electronic compacts,microtubules,crystals,etc.)are visible.(4)Characteristics of deposition of amyloid in kidneyThe kidney tissue samples were stained with Congo red,and the deposition sites of amyloid in the kidney(glomeruli,renal interstitium,renal blood vessels,etc.),main deposition patterns were observed under a light microscope,and the presence of apple green was observed under a polarized light microscope.(5)TypingImmunohistochemical method was used to detect the expression of anti-immunoglobulin light chain ? and ? antibodies and anti-serum amyloid A(SAA)antibody in 75 cases;non-AA and AL cases were detected by immunohistochemical method to detect anti-leukocyte chemokine 2(Lect2)expression;observe the positive expression of amyloid deposition in the kidney.(6)Pathological classification,scoring and classificationAccording to the light microscope,the deposition pattern and amount of amyloid in glomeruli were used to classify the disease patterns;the quantitative and semi-quantitative evaluation of 75 patients with glomerular,renal vascular,and renal tubular interstitial amyloid deposition,Renal interstitial fibrosis with renal tubular atrophy and renal interstitial inflammation degree and grade.Results:1.Clinical features:(1)General situation: From January 1993 to November 2019,our center received a total of 9382 renal biopsy specimens,of which 75 cases were diagnosed with renal amyloidosis,accounting for 0.7% of renal biopsies in the same period.75 patients were more common in men(male: female = 1.6: 1),the median age was 56.96±8.30 years old,and 81.3% of patients were over 40 years old.The clinical symptom is mainly edema,and the most common signs are peritoneal effusion.The average time from symptoms to visit is 16.7 months.(2)Biochemical indicators and clinical staging: 75 patients had proteinuria,98.6%had hypoproteinemia,28% had reduced hemoglobin concentration,the most commonclinical stage was nephrotic syndrome(60%),followed by renal insufficiency(24%),12 cases of proteinuria(16%).(3)Combined with other diseases: 6 cases with lymphatic hematopoietic disease: 4cases of multiple myeloma(accounting for 5.3% of renal amyloidosis,common in males,all treated with edema as the first symptom,renal injury had proteinuria,3 cases of nephrotic syndrome Level,and there was no increase in serum creatinine;the pathological types were all AL-? type,and the light microscope morphology was lighter),1 case of M proteinemia and Castleman disease of lymph nodes(each accounted for renal amyloidosis)1.3%);5 patients with primary glomerular disease: 3 cases of IgA nephropathy,1 case of membranoproliferative glomerulonephritis and 1 case of membranous nephropathy.2.Pathological features:(1)light microscopic morphological manifestations: Under light microscope,the pathological morphology of the glomerulus is different,and six morphological changes can be seen.The mesangial matrix of the glomerulus is mild to moderately proliferative(30 cases,40%),and the glomerular mild lesions(18 cases,24%),diffuse thickening of the glomerular basement membrane(12 cases,16%),mesangial matrix hyperplasia with thickening of the basement membrane(9 cases,12%),severe mesangial matrix Hyperplasia(5 cases,7%),glomerular ischemic shrinkage(1 case,1.3%).(2)Immunofluorescence characteristics: Immunofluorescence examination of patients with simple renal amyloidosis has no specific immune complex deposition characteristics.Five patients with other kidney diseases showed that immunofluorescence showed corresponding specific deposition and the fluorescence intensity reached 2+ and above.(3)Electron microscopic performance: the glomerular mesangial area and the basement membrane can be seen with 8-12 nm diameter,unbranched fibers disordered arrangement,including 5 cases with other kidney diseases,and the deposition of electron dense substance in the corresponding parts.(4)Characteristics of the deposition of amyloid in the kidney: Amyloid is mainly deposited in the glomeruli,blood vessels and interstitium.Among them,62 cases mainly composed of glomerular deposition(82.7%),12 cases mainly composed of renal blood vessels(16%),and 1 case mainly composed of renal interstitial(1.3%).(5)Classification: The most common type is AL type(64 cases,84%),of which the ratio of AL-? type and AL-? type is 1.4: 1;2 cases of AA type(accounting for 2.7%);ALect2 type 1 case(1.3%).3.Relationship between clinical and pathological:(1)Pathological classification has no correlation with urine albumin quantification and clinical stage(P> 0.05).(2)Pathological classification has correlation with urine protein quantification,blood creatinine level and clinical stage(P <0.01).(3)Glomerular starch There is a correlation between GA,Ifib,Iinf and urine albumin quantification,blood creatinine level,and clinical stage(P <0.01).Conclusion:1.In this study,the incidence of renal amyloidosis was 0.7%,and it was more common in men,more than 50 years old;the clinical manifestations were mainly edema and peritoneal effusion;all had proteinuria,and most were nephrotic syndrome.2.The light microscope morphology of renal amyloidosis is mainly characterized by mild to moderate mesangial hyperplasia;the pathological types are mainly AL type(64 cases,84%),AA type(2 cases,2.7%)and ALECT2.(2 cases,2.7%)followed;glomeruli are the most common deposits of amyloid,followed by renal blood vessels.3.The degree of amyloid deposition and the related pathological changes of renal interstitial tubules in renal amyloidosis are positively correlated with clinical blood creatinine and urine albumin quantification;the use of glomerular lesion model to assess the degree of disease has limitations,and the pathological grade can be better Lesiondegree;pathological grade and pathological score index are closely related to clinical stage.