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Objective:To evaluate the efficacy and safety of lenalidomide plus dexamethasone (LD) in patients with proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID).Methods:The clinicopathological data of PGNMID patients who were treated with LD protocol from January 2010 to October 2019 were retrospectively analyzed.Results:All of 6 patients received LD treatment for≥3 months after renal biopsy in Jinling Hospital. During the follow-up period of 6 to 19 months, 3 patients achieved renal remission, and the renal remission rate was 50%(3/6). Light microscopy showed membranoproliferative glomerulonephritis and immunofluorescence showed single kappa type IgG3 was deposited in the mesangial region and the vascular loop. Before taking LD scheme, the median urinary protein were 7.76(1.27, 14.57) g/24 h, the median serum creatinine was 118.5(70.7, 289.1) μmol/L, and the median albumin was 34.5(22.4, 37.5) g/L. The concentration of serum free kappa and lambda light chain was increased in 5 patients, but the serum free light chain ratio was normal. Hypocomplementemia was detected in two cases. Six patients underwent bone marrow flow cytometry, and 2 patients had elevated monoclonal plasma cells, accounting for 0.7% and 0.5%, respectively. Immunofixation electrophoresis suggested that 1 patient had positive serum M protein for kappa type IgG3. At the last follow-up, median urine protein was 3.33(0.33, 11.23) g/24 h, median serum creatinine was 108.7(80.4, 160.9) μmol/L, and median albumin was 35.9(24.5, 45.6) g/L. The concentration of serum free light chain in 4 patients from 5 patients with elevated serum free light chain was lower than that before taking the drug. Decreased level of serum complement in two cases returned to normal after treatment. The M spike did not turn negative during the follow-up in one patient. Adverse events included anemia, neutropenia, limb numbness and upper respiratory tract infection.Conclusion:This study reports for the first time that LD protocol may be effective in treating PGNMID, but more attention should be paid to the hematological adverse events of lenalidomide.
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Objective To summarize the pathological survey of time-zero renal biopsy (T0-RBx ) . Methods The material qualities and pathological features were analyzed retrospectively for T 0-RBx (n=176) between March 2008 and May 2016 .According to the source of donor kidney ,T0-RBx specimens were divided into living donors (LD) group (n=137) and Deceased donation (DD) group (n=39) .Furthermore , the DD group was divided into cerebral hemorrhage group (n= 10) and brain trauma group (n= 29) according to the causes of death .The inter-group differences of pathological characteristics and the effects of abnormal pathological lesions on allograft function were observed .Results All T0-RBx specimens contained cortical kidney tissue .The average microscopic length of renal tissue was (0 .39 ± 0 .23) cm and the median glomerular number 11 . The abnormal pathological lesions included glomerulosclerosis (GS ,30 .7 ％ ) , segmental glomerulosclerosis (1 .1 ％ ) ,mesangial increase (MI ,19 .3 ％ ) ,tubular atrophy (TA ,35 .2 ％ ) , acute tubular necrosis (ATN ,9 .1 ％ ) ,vacuolar degeneration of tubular epithelium (27 .3 ％ ) ,losses in tubule epithelial brush border (97 .7 ％ ) , protein cast (25 ％ ) , interstitial fibrosis (IF ,34 .1 ％ ) , inflammation (I ,42 .6 ％ ) ,arteriolar hyalinosis (AH) (26 .1 ％ ) and vascular fibrous intimal thickening (CV ,23 .3 ％ ) .Among them ,23 .9 ％ ,1 .1 ％ ,0 .55 ％ and 0 .55 ％ cases were diagnosed as IgA nephropathy ,immune complex associated with glomerular disease and focal segmental glomerulosclerosis diabetic nephropathy respectively .And the reminders were of ischemic injury .The incidence rates of TA ,IF and I were lower in DD group than those in LD group ( P< 0 .05 ) . However , ATN and vacuolar degeneration of tubular epithelium were higher (P<0 .001) .The incidence of GS was significantly higher in cerebral hemorrhage group than that in brain trauma group (P<0 .01) .No statistical difference existed in other lesions or disease constitution among the groups (P>0 .05) .Further analysis showed GS was related with allograft function at 6/12 months post-transplantation in both LD and DD groups (P<0 .05) .IF and AH were also related to short-term renal function of recipients post-transplantation in LD and DD groups (P>0 .05) .Conclusions T0-RBx may detect the abnormal lesions of donor kidney .Some differences exist in types and degree of abnormal lesions among different donor kidneys .LD group has a higher risk for chronic histological injury such as TA and IF while DD group is more susceptible to acute renal tubular interstitial injury .Thus it is valuable for predicting allograft function post-transplantation .Material quality is essential for ensuring the reliability of T 0-RBx .
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Objective Membranous nephropathy ( MN) is rarely complicated by crescents.This study was to observe the clinicopathologic characteristics of MN with crescents. Methods This retrospective study included 53 cases of biopsy-proven idiopathic MN with crescents in the absence of immunologic and clinical etiologic factors and another 100 MN patients without histological crescents as controls.The clinicopathologic features, treatment response, and out-comes were analyzed and compared between the two groups of pa-tients. Results Significantly higher percentages of hypertension and decreased eGFR were observed in the MN +crescents group than in the control (47.2%vs 19.0%, P<0.05;28.3%vs 40.%, P<0.05).Circulating autoantibodies against the M-type phospholipase A2 receptor (PLA2R) were found in 66.7%(30/45) of the patients.The glomeruli exhibited a median of 4.6%(1.8%-35.3%) involvement of crescents.Compared with the controls, the MN +crescents group showed remarkably higher rates of segmental glomer-ulosclerosis lesions (16.0%vs 49.1%, P<0.05), capillary loops necrosis (0.0%vs 11.3%, P<0.05), interstitial fibrosis/tubu-lar atrophy (IFTA) (54.0%vs 86.8%, P<0.05) and afferent arterial lesions (65.0%vs 92.5%, P<0.05).No significant differ-ences were found in the outcomes of the two groups of patients. Conclusion MN with crescents is rare, and secondary MN and cres-centic glomerulonephritis should be considered.Crescentic MN usually presents with hypertension and renal dysfunction clinically and high rates of severe segmental and global glomerulosclerosis, capillary loops necrosis, and IFTA histologically.The condition has a fa-vorable short-term prognosis.
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Objective To report 1 case with renal immunoglobulin light and heavy chain deposited disease (IgG-κ light chain and γ1 heavy chain).Methods The clinical manifestations,serum immunofixation electrophoresis,light and heavy chain abnormalities of blood and urine,bone marrow biopsy and renal biopsy data laboratory data were recorded and analyzed.Results A 63-year-old woman presented with massive proteinuria,microscopic hematuria,hypertension,anemia and serum IgG-κ light chain.Bone marrow aspirate revealed 4％ plasma cells.Renal biopsy revealed nodular glomerulopathy with congo red staining-negative.Immunofluorescence showed that κ light chain and IgG1 (γ1 heavy chain) were deposited along the glomerular basement membranes (GBM) and tubular basement membranes (TBM).Electron-microscopy revealed electrondense deposits that delineate the outer aspect of TBM and endothelial aspect of GBM.Conclusion The diagnosis of renal immunoglobulin light and heavy chain deposited disease (IgG-κ light chain and γ1 heavy chain) should be addressed combine with clinical and is pathology,especially with immune pathological examination.
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Objective To investigate the clinical-pathological features, treatment and prognosis of thrombotic thrombocytopenic purpura in patients with lupus nephritis (LN). Methods A retrospective ana-lysis was carried out based on the clinical-pathological data for the treatment and prognosis of eight patients with LN related TIP. All patients had thrombocytopenia and hemolytic anemia, neurological symptoms and renal dysfunction. Six patients had fever. Results All 8 patients had sudden-onset of rapid progressive glomeurlonephritis (RPGN), one patient with continuous gross hematuria, the pathological features of these patients revealed TMA lesions. Immune suppressive therapy was initiated and blood purification therapy were applied in seven patients. Three cases had plasmapheresis and (or) immunoabsorption. One case was lost during follow-up, the other seven patients were followed with period at one year. One patient died, three patients went into peritoneal dialysis in which one of them was changed to hemodialysis finally. The other three patients had stable renal function. Conclusion The LN patients with TTP had severe clinical-patho-logical changes, rapid progressive and poor outcome, so we should diagnose and treat these patients as early as possible.
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Objective:To analyze and compare the clinicopathological characteristics in low weight proteinuria(LWP) and nephrotic proteinuria(NP) multiple myeloma(MM) patients with renal involvement.Methods:From October 1991 to October 2005,46 patients with MM were diagnosed in the Research Institute of Nephrology of Jinling Hospital(Nanjing,China).Renal biopsies were done in 41 of them.The patients were devided into two groups,LWP and NP groups.Their clinical and pathological features were investigated and compared. Results:The epidemiological features and type of MM in LWP and NP groups were similar.The patients in LWP group had higher incidence of D-S Ⅲ stage and heavier anaemia compared to NP group.Compared to patients in NP group,patients in LWP group had higher incidence of renal insufficiency and lower urine osmotic pressure.Part of patients checked urine N-acetyl-?-glucosaminidase,RBP and there were no difference between two groups.Cast nephropathy was the most frequent pathologic type in LWP group,while light chain deposition disease and glomerular amyloidosis were the most common pathologic type in NP group.Conclusion:According to this study,we get the conclusion that proteinuria analysis may be a significant test to evaluate the clinicopathological characteristics of MM patients with renal involvement.
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<p><b>OBJECTIVE</b>To investigate retrospectively the incidence, distribution of primary disease and clinicopathologic characteristics of diffuse crescentic glomerulonephritis (DCGN) in Chinese patients.</p><p><b>METHODS</b>One hundred and seventy-two consecutive patients diagnosed as having DCGN out of 9828 cases of non-transplanting renal biopsies over sixteen years, were studied. DCGN is categorized into three types according to immunopathologic characteristics. The incidence of this disease, its primary diseases, clinical characteristics and serum antineutrophil cytoplasmic antibodies (ANCAs) were analyzed.</p><p><b>RESULTS</b>The distribution of patients among the three classifications was 8.7% type I, 68.6% type II and 22.7% type III. Clinically, the majority of patients (69.8%) presented rapidly progressive glomerulonephritis (RPGN), but 30.2% manifested a chronic nephritic syndrome or chronic renal failure. In terms of related conditions, 93% were anemic, 61.6% had hypertension, 50.6% oliguria, 45.3% nephrotic syndrome, 43% uremic syndrome and 39.5% displayed gross hematuria. Those patients who were positive in serum for ANCAs had predominantly type III DCGN. Two cases with anti-GBM-antibody crescentic glomerulonephritis and three with lupus nephritis were also positive for ANCAs in serum.</p><p><b>CONCLUSION</b>DCGN is not rare in Chinese patients. A majority of patients in our study presented with RPGN, but 30.2% manifested a chronic renal failure. Lupus patients with DCGN that were positive for ANCAs had more severe vasculitic lesions.</p>
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Adolescent , Adult , Aged , Child , Female , Humans , Male , Middle Aged , China , Epidemiology , Glomerulonephritis , Classification , EpidemiologyABSTRACT
<p><b>OBJECTIVE</b>To analysis the pathological demography in Chinese patients undergoing renal biopsy from our nephrology center.</p><p><b>METHODS</b>Between January 1979 and October 2000 in Jinling Hospital, Nanjing, China, 10,002 attempts of percutaneous renal were performed in patients with renal disease from 33 provinces of China. The pathological classifications were made according to the WHO criteria of 1982 for renal pathology or the modified WHO criteria of 1995 by a panel of pathologists and nephrologists during routine clinical-pathological rounds. The pathological demography between those specimens collected from 1979 - 1989 and those from 1990 - 1999 was compared.</p><p><b>RESULTS</b>The mean age of the 10,002 subjects undergoing renal biopsy was 31.4 +/- 13.0 years (ranging from 1 to 78 years), with a male to female ratio of 1.3:1; for the 592 renal transplant recipients, the mean age was 37.5 +/- 9.1 years (ranging from 16 to 66 years), with a male to female ratio of 2.36:1. Primary glomerular diseases (PGD) accounted for 71% of the total patients undergoing renal biopsies, secondary glomerular nephritis (SGN) 23%, tubular-interstitial diseases 3.2%, unclassified renal diseases 1.3%, hereditary and congenital renal diseases 1.0%, end stage renal diseases 0.96%, and recently realized or rare renal diseases 0.15%. IgA nephropathy (IgAN) was the most frequent pathological pattern (40%) of PGD, followed by mesangial proliferative lesion (MsPL) (30%), membranous nephropathy (MN) (10%), and focal segmental glomerulosclerosis (FSGS) (6%). Lupus nephritis (LN) was the most pathology common seen (74%) in SGN. During the 22 years of the study period, there was a steady increase in patients with SGN discovered during pathological evaluation of renal disorders. A rise in prevalence was found in IgA nephropathy, MN (both P < 0.001), crescentic glomerulonephritis (P < 0.0001), anti-GBM disease, and hemolytic-uremic syndrome/thrombotic thrombocytopenic purpura related renal damages (both P < 0.001). There was a decrease in endocapillary proliferative glomerulonephritis (P < 0.001) and IgM nephropathy (IgMN) (P < 0.01) from 1990 - 1999 as compared to 1979 - 1989. Infrequent renal pathological entities were also diagnosed in this group, including Niemann Pick disease, Fabry's disease, POEMS syndrome, and lipoprotein glomerulonephropathy.</p><p><b>CONCLUSIONS</b>This is the largest series of renal biopsy data in China, and therefore may reflect the demographic picture of renal diseases in this country. Changes in prevalence of renal pathological entities were reflected in this group of patients over the last 22 years. In primary glomerular diseases, IgA nephropathy is still the most frequently observed pathological pattern. In SGN, LN appeared the most often. Increased prevalence was found in anti-GBM nephritis and HUS/TTP.</p>
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Adolescent , Adult , Aged , Child , Child, Preschool , Female , Humans , Infant , Male , Middle Aged , Japan , Epidemiology , Kidney Diseases , Epidemiology , Pathology , PrevalenceABSTRACT
Objective To compare the effect,relapse rate and outcomes between mycophenolate mofetil(MMF)and pulse intravenous cyclophosphamide(CTX)in the induction therapy of pauci-immune crescentic glomerulonephritis(PICGN)in Chinese.Methods A total of 44 patients who had PICGN[16 male,28 female,age(46.8?13.7)y],of whom 25 patients were ANCA positive,were enrolled in this study.All patients had renal involvement with ≥50% crescent formation prior to the study and received either MMF treatment(MMF group,n=22)or intermittent CTX pulse therapy(CTX group,n=22).The patients in both groups also received methylprednisolone(MP)pulse therapy followed by oral prednisone.General conditions,clinicopathological findings,remission rate,relapse rate,and outcomes were compared.All the patients were followed up until June 2005,with an average follow-up of 8～60(Med 27)months in the MMF group,and 6～72(Med 29)months in the CTX group.Results No significant difference was found between MMF group and CTX group in general conditions,base parameters of clinical and pathological findings.The remission rate at the 12th month in MMF and CTX group was 90.9% and 72.7% respectively.The complete remission rate in MMF group(59.1%)was significantly higher than that of the CTX group(27.3%)(P