Diffuse Endocapillary Proliferation Research Articles

#2672 Cryoglobulin-Induced membranoproliferative glomerulonephritis in a HEV-infected kidney transplant recipient: outcomes post ribavirin failure

Abstract Background and Aims Hepatitis E is a zoonosis caused by hepatitis E virus (HEV), which was first discovered 40 years ago. It is the most prevalent hepatitis virus worldwide. Most hepatitis E cases are self-limiting acute hepatitis, but the virus has been recognized to cause chronic hepatitis. Following the first case report of chronic hepatitis E (CHE) in a transplant recipient, CHE has recently been identified as associated with chronic liver damage induced in immunocompromised patients such as transplant recipients. CHE can induce extra-hepatic manifestations, such as immune-mediated thrombocytopenia, neurological symptoms, and kidney injuries. Few cases of glomerulonephritis (GN) and cryoglobulinemia in transplant patients have been related with CHE, with a good progression thanks to viral control with ribavirin introduction. There have been no reported cases in the literature of good progress in renal function in renal transplant patients despite failure of treatment with Ribavirin. Method We report here what is to our knowledge, the first documented case of HEV-induced de novo cryoglobulinemic membranoproliferative glomerulonephritis in a renal transplant patient with a ten-year history of CHE infection unsuccessfully treated with ribavirin. Results A 69-year-old man underwent a kidney transplant for diabetes type 1. 7 years after transplantation, he was diagnosed with a chronic genotype 3.1H-HEV infection. He was treated by Ribavirin, but it did not allow to eradicate HEV but a biochemical response. At 18 years post transplant, he presented with asthenia, abdominal pains and increased liver enzyme levels: liver tests were disrupted with cytolysis and cholestasis. He presented also a nephritic syndrome with high blood pressure, oedemas, increased serum creatinine level, proteinuria with hypoalbuminemia and de novo microscopic hematuria. Biological analysis revealed presence of type 2 cryoglobulinemia with monoclonal IgM Kappa (2285 mg/L), and polyclonal immunoglobulins association. Rheumatoid factor was increased (573 UI/ml) and C4 level was reduced (0.04 g/L). The concentration of HEV RNA in the serum was 750 000 UI/mL. Viral serologies were performed for HBV, HCV, HAV, HIV, EBV, CMV. Hematologic diseases were ruled out by analyzing a bone marrow biopsy specimen. Histologic analysis of a kidney biopsy specimen revealed diffuse endocapillary proliferation, diffuse mesangial proliferation and focal glomerular basal membrane duplication. One arteriolar section showed a focal lesion of vasculitis (Fig. 1). There was no argument for allograft rejection. Immunofluorescence microscopy revealed mesangial and capillary wall deposits of IgM, IgG, C3 and C1q, polytypic for k and l light chains. We suspected cryoglobulinemic membranoproliferative glomerulonephritis induced by VHE. The initial treatment plan involved 10 sessions of plasmapheresis and steroid pulses (500 mg/d for 3 days), alongside a course of rituximab (1 g/week for 4 weeks). However, the patient experienced intolerance to rituximab, prompting a switch to Obinutuzumab. Subsequently, the anti-HEV therapy was modified, and the patient was placed on a course of Sofosbuvir. Consequently, three months after treatment kidney function kidney improved with return to baseline and cryoglobulinemia became undetectable. However, proteinuria remained impaired and VHE RNA was still present. Conclusion Cryoglobulinemia is rarely associated with glomerular disease in HEV infection, and cases currently reported in the literature showed a favorable outcome with ribavirin. This case is the first to display a chronic HEV infection with cryoglobulin-induced glomerulonephritis with global improvement despite unsuccessful treatment with ribavirin. Although the difficulty of managing immunity in the treatment of cryoglobulinemia by immunodepleting treatment, with the risk of increased viral replication in the absence of an effective antiviral, it seems interesting to offer a first-line treatment of cryoglobulinemia that appears to improve renal function despite continued viral replication. These results need to be confirmed in further studies.

C3 glomerulonephritis in post transplanted patient with MGRS (A case report)

C3 glomerulopathy includes several rare forms of glomerulonephritis with underlying defects in the alternative pathway of complement cascade. It is characterized by predominant C3 deposition in glomeruli due to abnormal activation of the alternative pathway of the complement system. C3 GN has been reported to be associated with several systemic diseases. Methods We MEDICUS / No. 4, 2021 13 will describe a case presenting C3 GN in a patient with monoclonal gammopathy of renal significance (MGRS) Results A 61 years old man patient presented with gross hematuria,anemia ,renal disfunction (creatinemia 2,4 mg/dl), proteinuria 814 mg/24 h. The patient was transplanted two years ago and he was taking corticosteroids ,MMF,tacrolimus and entecavir for hepatitis B .Serum protein electrophoresis ; hypogammaglobulinemia with a small homogeneous spike-like peak. Serum Kappa free light chains 32,4 mg/dl ,serum Lambda free light chains 9.3 mg/dl.Ratio 3,4 . Autoimmune tests ANA, ANCA, Anti ds DNA C3 and C4 were negative.Urine Kappa Light Chains 26,4 mg/24 h,urine Lambda Light Chains 6,6 mg/24h.Urine Kappa/Lambda ratio 4. After consultation with haematolgist results of bone narrow biopsy came for monoclonal gammopathy , and FISH conclusion is presence of t(11,14)(q13,q32)which originated from IgH / CCND1 retraction and 1q21 acquisition. Renal biopsy is C3 glomerulopathy with mesengial and diffuse endocapillary proliferation under light microscope and diffuse deposition of C3 and no immunoglobulin under immunofluorescence microscope. Conclusions Monoclonal gammopathy of renal significance MGRS is a term to describe a group of haematological disorders associated with kidney disease that fail to meet the standard definitions for MM or lymphoma. In such cases, the renal impairment is often linked to the underlying hematological disorder. The intention was to make a clear distinction between MGUS, a benign asymptomatic condition, and MGRS, which may be associated with significant morbidity and mortality.

Comparison of clinicopathological features and prognosis between IgA nephropathy and purpura nephritis in adults with diffuse endocapillary proliferation: a single-center cohort study.

Clinical manifestations and histological lesions of IgA nephropathy and Henoch-Schönlein purpura nephritis (HSPN) are different, but related, and are also correlated with the renal outcomes. This study aimed to compare the features of immunoglobulin A nephropathy (IgAN) and HSPN in adult patients with diffuse endocapillary proliferation (DEP) lesions aiming to clarify the differences and relationships in the clinicopathological findings and outcome. Twelve patients with DEP-IgAN and 10 patients with DEP-HSPN were enrolled. Twenty four patients with IgAN (NDEP-IgAN) and matched 20 patients with HSPN (NDEP-HSPN) were enrolled at the same ratio (1:2). The clinicopathological features, clinical efficacy, and renal outcomes were analyzed in the four groups. DEP patients with IgAN or HSPN had worse clinical manifestations (more severe proteinuria, lower serum ALB, higher incidence of gross hematuria). The proteinuria in the DEP-HSPN group was more severe than in the DEP-IgAN group. There was no significant difference in the serum creatinine among four groups. The incidence of endothelial swelling was significantly higher in the DEP-HSPN group than in the NDEP-HSPN group and DEP-IgAN group. The S1 score of Oxford classification was more common in the DEP-IgAN group than in the DEP-HSPN. None in the DEP-IgAN group reached endpoint events during the follow-up period, while the renal outcomes were significantly poorer in the DEP-HSPN group than in the DEP-IgAN and NDEP-HSPN groups. No significant difference was observed in the cumulative renal survival among four groups (χ 2 =7.264, P=0.064), but patients in the DEP-HSPN group had markedly lower renal cumulative survival rate as compared to the NDEP-HSPN group (χ 2 =4.875, P=0.027). The DEP is significantly associated with more severe proteinuria and hematuria regardless the IgAN and HSPN. Among DEP patients, patients with HSPN have poor therapeutic efficacy and renal outcomes, even under active immunosuppressive therapy, as compared to those with IgAN.

SUN-440 RENAL DISEASE PRESENTATION IN MIXED CRYOGLOBULINEMIA TYPE 2 A CASE REPORT AND A LITERATURE REVIEW

Mixed cryoglobulinemia (MC) results of cryoprecipitable immunocomplexes. In type II MC, a combination of polyclonal and monoclonal immunoglobulin is found. Renal involvement (RI) is one of the most serious complications in MC and may affect the overall prognosis. Case presentation: A diagnosis of essential mixed cryoglobulinemia (EMC) was established in a 36-year-old female patient. Eight years earlier, shedevelopedpurpuraof the lower extremities, arthralgia, peripheral neuropathy andpulmonary renal syndrome. A skin biopsy revealed a leukocytic vasculitis.A mixed cryoglobulin and hypocomplementemia were found. Urinary protein excretion was at 12 g per 24h. Initial treatment included pulse methylprednisolone followed by prednisone (1 mg/kg daily). Despite the clinical improvement in the renal disease, palpable purpuric lesions with arthralgia recurred periodically. So methotrexate was introduced but was rapidly interrupted owing to liver toxicity. On June 2019, she was admitted for pneumonia. An outbreak of her disease with dermal, peripheral nerve and renal involvement was objectified. Urinary protein excretion was at 3 g per 24h. A mixed cryoglobulin with monoclonal IgM Kappa and polyclonal immunoglobulin persisted. A Renal biopsy (RB) was done in July 2019, revealed diffuse endocapillary proliferation with granular IgM, C3 deposits. Plasmapheresis with twice exchanges per weak was conducted. Five days after the initiation of therapy, her purpura disappeared and serum complement values returned to normal. Review of the literature: The results of a long-term prospective, randomized controlled trial of rituximab (RTX) monotherapy in patients with cryoglobulinemia vasculitis (CV). RTX was found to be superior to standard immunosuppressive therapy for severe CV over the long term. A multicenter retrospective study included 14 patients with EMC who were successfully treated with lenalidomide. A study, included 21 patients with severe type II EMC unresponsive to immunosuppressive regimens, showed that IFN-a, as a single agent, is able to determine a response rate as high as 77%. A case report demonstrated that 2-Chlorodeoxyadenosine (2CdA) induced a dramatic and prolonged clinical response for a patient with progressive, symptomatic cryoglobulinemia, in whom corticosteroid and alkylating therapy failed. Some studies suggest that plasma exchange alone might be indicated in the treatment of a rapidly progressive glomerulonephritis complicating MC. We presented this case of EMC with renal involvement. The clinical presentation is characterized by a normal kidney function without nephrotic syndrome with membranoproliferative glomerulonephritis (MPGN) at the renal biopsy. This case reveals the importance of RB for the diagnosis of MPGN even in front of an atypical clinical picture.

Membranoproliferative glomerulonephritis related to a streptococcal infection in a girl with IgA deficiency: a case report

BackgroundIgA deficiency associated with glomerulonephritis is rare. In particular, there is no prior report regarding the association between IgA deficiency and membranoproliferative glomerulonephritis (MPGN) in children. Herein, we describe the case of a 5-year-old girl with selective IgA deficiency and MPGN.Case presentationThe patient presented with persisting urinary abnormality and hypocomplementemia following a group A treptococcal infection. Renal biopsy revealed the presence of diffuse mesangial hypercellularity, endocapillary proliferation, and focal thickening of the walls of the glomerular capillaries using light microscopy, with IgG and moderate C3 deposits observed using immunofluorescence. Electron microscopy images revealed nodular deposits in the subendothelial areas, with hump-shaped subepithelial deposits. The pathological diagnosis was confirmed as MPGN. Treatment using oral prednisolone (PSL), mizoribine (MZR), and angiotensin-converting enzyme inhibitors reduced the proteinuria. The PSL dose was gradually tapered, with the low dose of PSL and MZR continued for 4 years. Histological findings were improved on repeated renal biopsy, and PSL and MZR administration was discontinued.ConclusionsWe report a rare case of MPGN related to a streptococcal infection in a child. The clinical presentation included selective IgAD, with several pathological findings and a clinical course typical of glomerulopathy. The patient was successfully treated using multidrug therapy.

Clinical and pathological analysis of IgA nephropathy with acute kidney injury

To investigate the incidence, etiology, clinico- pathological characteristics and prognosis in primary IgA nephropathy (IgAN) children with acute kidney injury (AKI). Retrospective analysis of the clinical and pathological manifestations and follow-up results of 19 Chlidren, who were associated with AKI in 196 cases of children with IgA nephropathy treated in our department from January, 1996 to Jun, 2012 was performed. (1) The 19 cases associated with AKI accounted for 9.7% of all 196 Chlidren with IgAN. Within the 19 cases, there were gross hematuria in 17 cases, massive proteinuria in 16 cases, hypoalbuminemia in 10 cases, edema in 10 cases and hypertension in one case. The peak serum creatinine was from 94.5 μmol/ L to 282 μmol/L. (2) Histological changes: with the formation of crescent in 10 cases, diffuse endocapillary proliferation in 5 cases, 15 cases had renal tubular injury, 10 cases had red blood cell and protein cast, 1 case with acute interstitial nephritis. (3) The cause of IgA nephropathy with AKI: 13 patients had severe glomerular damage, including crescentic glomerulonephritis and diffuse endocapillary proliferation; 1 case was complicated with acute interstitial nephritis after being treated with antibiotics, 2 patients had decreased glomerular filtration rate because of taking benazepril or oral indomethacin, 1 case with renal tubular injury induced by gross hematuria, and the other two cases the reason was not clear. (4) Multivariate Logistic regression analysis showed that massive proteinuria was independent risk factor of IgAN in children with AKI (OR=27.370, 95% confidence interval was 3.151-237.740, P<0.01). (5) None of the patients were on dialysis, steroid therapy was used in 13 cases (including 7 cases of methylprednisolone pulse therapy), 6 cases were treated with combined cyclophosphamide treatment. Except 1 cases no significant improvement, the renal functiones of all patients recovered or improved within 1-2 months after treatment. Follow-up period was from 1 month to 7 years, 3 cases had renal function improved, but 2 cases were lost to follow-up for 3 years and then entered the chronic renal failure, 1 case had renal function loss after 32 months and repeated renal biopsy showed glomerular sclerosis of 32% during the follow-up period. In children with IgAN, AKI accounted for about 10%, except glomerular severe lesion, the onset of AKI is also relevant to clinical medication and repeated gross hematuria, and the heavy proteinuria is an independent risk factor. Based on clinical observation, the short-term prognosis of IgAN children with AKI is optimistic.

Clinical features and outcomes of diffuse endocapillary proliferation Henoch-Schönlein purpura nephritis in children

OBJECTIVE:To investigate the outcomes of childhood diffuse endocapillary proliferation Henoch-Schönlein purpura nephritis (DEP-HSPN) in response to early diagnosis and prompt treatment.METHODS:Eleven cases of DEP-HSPN in children were investigated in comparison to HSPN without diffuse endocapillary proliferation (non-DEP-HSPN).RESULTS:DEP-HSPN had a higher prevalence of nephrotic syndrome but a lower prevalence of hematuria compared to non-DEP-HSPN. IgA, IgG and IgM antibody deposition was found in DEP-HSPN by histopathological examination. Proteinuria cleared in all 11 cases through treatment with steroids and/or immunosuppressive drugs. However, half of the DEP-HSPN patients continuously had hematuria after treatment.CONCLUSION:The early diagnosis and prompt initiation of immunosuppressive treatment based on renal biopsy are important for achieving favorable outcomes.

Sjögren Syndrome and Cryoglobulinemic Glomerulonephritis

We report the case of a 53-year-old woman with Sjögren syndrome and cryoglobulinemia. The patient presented with nephrotic syndrome, hematuria, and reduced estimated glomerular filtration rate. The kidney biopsy revealed diffuse endocapillary proliferation and leukocyte exudation with focal intraluminal hyaline thrombi, prominent tubulointerstitial inflammation, and vasculitis. Diffuse granular mesangial and segmental to global capillary wall staining was observed on immunofluorescence with antisera to C3 and immunoglobulin M (IgM), with less intense staining indicative of IgG and κ and λ light chains. A biopsy diagnosis of Sjögren syndrome-related cryoglobulinemic membranoproliferative glomerulonephritis and vasculitis was rendered. Subsequent investigations revealed the presence of circulating type II cryoglobulins with cryocrit of 9%. Although rare, Sjögren syndrome is the most common cause of non-hepatitis C virus-related mixed cryoglobulinemia. We discuss the possible pathogenic mechanisms involved in the development of mixed cryoglobulinemia and its evolution to lymphoma, as best described in the setting of hepatitis C virus infection. Although the specific antigen involved is unknown, it is likely that the mixed cryoglobulinemia in Sjögren syndrome is triggered by the long-term B-cell stimulation, resulting in clonal proliferation of B cells. Additional chromosomal aberrations and cytokine milieu alterations, as seen in hepatitis C virus infection, may result in prolonged B-cell survival and progression to non-Hodgkin lymphoma.

Clinicopathological features and prognosis of membranoproliferative-like Henoch-Schönlein purpura nephritis in children.

The aim of this retrospective study was to define the clinical manifestations, pathological features and prognosis of children with membranoproliferative-like Henoch-Schönlein purpura nephritis (HSPN), representing International Study of Kidney Disease in Children (ISKDC) grade VI. Among 245 patients with HSPN treated in our hospital between 2008 and 2010, nine patients (3.7%) were diagnosed with HSPN of ISKDC grade VI (males = 5, females = 4, age: 9.5 ± 2.03 years, mean ± SD). The clinical features, laboratory and pathological findings, treatment and outcome of the 9 patients were retrospectively analyzed. Of the 9 patients, 7 (78%) presented with hematuria and nephrotic syndrome, and were treated with steroids (oral prednisone or intravenous methylprednisolone pulse therapy) and immunosuppressants (oral tripterygium glycosides or intravenous cyclophosphamide pulse therapy). One (11%) patient had hematuria and nephrotic range proteinuria (> 50 mg/kg per 24 hours) and was treated with oral prednisone and tripterygium glycosides. Another (11%) patient presented with hematuria and moderate proteinuria (25-50 mg/kg per 24 hours) and was treated with oral tripterygium glycoside only. Histopathological examination showed diffuse glomerular mesangial and endocapillary proliferation, mesangial interposition, double-contour formation, podocyte hypertrophy, shedding, and cytoplasmic absorption droplets. The percentages of glomeruli with small cellular crescents varied from 4%-25% in 6 of 9 patients. Follow-up for 2 to 4 years showed excellent recovery in all patients. The main clinical feature of ISKDC grade VI HSPN in children is a nephrotic syndrome with hematuria. The excellent prognosis of the disease was probably related to early diagnosis and treatment with steroids and/or immunosuppressants, and mild degree of glomerulosclerosis and tubulointerstitial damage.

Mitomycin‐C‐Induced Thrombotic Thrombocytopenic Purpura

ABSTRACTBackgroundAlthough mitomycin‐C has been supplanted by other chemotherapeutic drugs, it is still the drug of choice for anus squamous cell carcinoma. Mitomycin‐C has rarely been reported to cause thrombotic thrombocytopenic purpura with or without haemolytic uraemic syndrome (TTP/HUS).AimTo report a case of probable mitomycin‐C‐induced TTP with associated acute renal failure and severe hypertension.Clinical detailsA 60‐year‐old Caucasian female was diagnosed with anus squamous cell carcinoma and started on mitomycin‐C, fluorouracil and targeted radiation therapy. After completing this regimen, the patient developed acute kidney injury, thrombocytopenia and hypertension. Kidney biopsy revealed diffuse endocapillary cell proliferation and one glomerulus contained multiple hyaline thrombi with basement membrane double contours. Focal subendothelial and intramural fragmented erythrocytes were present; all of which suggested TTP. The patient was started on high‐dose corticosteroids followed by plasmapheresis.OutcomesThe patient's blood pressure, and platelets returned to baseline levels, while her renal function improved partially.ConclusionTTP is a rare but serious adverse effect associated with mitomycin‐C. Discontinuing mitomycin‐C does not resolve TTP; a variety of treatments are required, such as corticosteroids, plasmapheresis, chemotherapeutic drugs and rituximab.

Acute poststreptococcal glomerulonephritis with acute interstitial nephritis related to streptococcal pyrogenic exotoxin B.

A 20-year-old man presented with symptoms of edema. Three weeks prior to admission, he experienced an upper respiratory tract infection. On admission, blood pressure was 177/118 mmHg. Initial laboratory investigations revealed serum creatinine, 170 µmol/L; complement C3, 130 mg/L and antistreptolysin O titer, 538 IU/mL (reference, <239 IU/mL). Urinalysis showed nephrotic range proteinuria and hematuria. A percutaneous kidney biopsy was performed. Light microscopy contained 20 glomeruli, 4 of which were globally sclerotic and 1 of which showed cellular crescent formation. Diffuse endocapillary proliferative glomerulonephritis was identified (Figure 1A). Forty percent of the interstitium was infiltrated by inflammatory cells (Figure 1B). Immunofluorescent staining revealed complement C3, mainly on capillary walls. Fig. 1. (A and B) Findings of the kidney biopsy. Hematoxylin and eosin-stained section showing diffuse endocapillary proliferation with massive infiltration of neutrophils (A) and inflammatory cells infiltration in the cortical interstitium (B). (C–F ... Acute poststreptococcal glomerulonephritis (APSGN) with acute interstitial nephritis (AIN) was diagnosed. We administered methylprednisolone pulse therapy (1 g/day) for 3 days, followed by prednisolone at 80 mg/day. The serum creatinine level declined to 74 µmol/L and proteinuria decreased to <1 g/day. Transient type 4 renal tubular acidosis was observed during steroid treatment. AIN is found in the infectious diseases of children, particularly in streptococcal infection [1]. We investigated the localization of two streptococcal antigens, streptococcal pyrogenic exotoxin B (SPEB) and nephritis-associated plasmin receptor (NAPlr), using an anti-SPEB antibody (Abcam, Cambridge, MA) and a fluorescein isothiocyanate-conjugated anti-NAPlr antibody [1F10] (Abcam). Although NAPlr was not detected, SPEB was positive in the interstitium as well as glomeruli (Figure 1C–F). Chang et al. [2] reported a case of streptococcal infection-related AIN, in which SPEB was found in both tubular epithelial cells and interstitium. In the present case, negative results were obtained for SPEB in tubular epithelial cells. Similarly, SPEB and its precursors have been detected only in glomeruli and interstitium in the rat kidney [3]. SPEB has been reported in association with the pathogenesis of APSGN. The mechanisms of leukocyte infiltration could be mediated by chemotactic effects and the migration inhibitor factor-like activities of SPEB [3]. SPEB also induces proliferation of human mononuclear leukocytes [4]. SPEB may be one of the causes of streptococcal infection-related AIN.

A case of membranoproliferative glomerulonephritis associated with metastatic colon cancer

A case of membranoproliferative glomerulonephritis associated with metastatic colon cancer

Hepatitis B virus-related post-infectious glomerulonephritis: a case report

Hepatitis B virus infection is an uncommon cause of acute glomerulonephritis. We present a case of acute glomerulonephritis revealing a chronic viral hepatitis B. A 45 year-old man was admitted in the nephrology department of Hassan II university hospital (Fez, Morocco) for nephritic syndrome with advanced acute kidney injury. The investigations have revealed viral hepatitis B with a positive HBV-DNA and Others viral serology tests were negative. Renal biopsy showed a diffuse and global endocapillary proliferation without extra-capillary proliferation; and global deposits of C3 and Ig G On immunofluorescence. There wasn't any other infectious cause. We thus retained the diagnosis of hepatitis B virus-associated acute glomerulonephritis. He was given entecavir and corticosteroids. Three months later, the evolution was marked by the normalization of renal function, negativity of proteinuria and HBV DNA became undetectable. There was no relapse of glomerulonephritis and HBV viral load was still negative after one year follow-up.

Peculiar renal endarteritis in a patient with acute endocapillary proliferative glomerulonephritis

Acute glomerulonephritis (AGN) is one of the most common renal diseases. They are often associated with infections and can result in diffuse proliferative glomerulonephritis (GN). This case report reviews an interesting case in which renal endarteritis coexisted in AGN with diffuse endocapillary proliferation. The discussion highlights important pathological findings and clinical aspects in acute endocapillary proliferative GN with renal endarteritis. Coexisting endarteritis should be in the differential diagnosis of AGN in patients with persistent clinical courses.

Rapidly progressive cryoglobulinemic glomerulonephritis

The clinical course of cryoglobulinemic glomerulonephritis (CGGN) is usually slowly progressive, and only a minority of these patients progress to end-stage renal failure. This report describes an atypical case of a patient with CGGN who demonstrated a rapidly progressive and irreversible renal deterioration. After presenting with symptoms of an upper respiratory infection, the patient exhibited oliguric acute renal failure, which was followed by systemic efflorescence accompanied by a fever. The laboratory analyses of his serum revealed the patient to be cryoglobulin-positive with markedly decreased serum C4 levels and an increased anti-streptolysin O titer. A serological test for the hepatitis C virus was negative. A renal biopsy showed that the patient had diffuse endocapillary and extracapillary proliferation together with marked endoluminal thrombi and subendothelial deposits in glomeruli. An electron microscopic examination demonstrated the presence of electron-dense subendothelial, subepithelial and huge intraluminal deposits with a specific annular and cylindrical structure. These features were consistent with active and severe CGGN. Despite aggressive treatment with corticosteroid pulses and a plasma exchange, a second renal biopsy demonstrated further advanced renal injury and revealed no signs of recovery.

Clinicopathologic Features and Outcomes in Endocapillary Proliferative IgA Nephropathy

Objective: Clinical manifestations and histological lesions of IgA nephropathy are variable and correlate with the renal outcome of the patient. The aim of the present study is to clarify the clinicopathological features and the prognosis of patients with IgA nephropathy presenting with dominant diffuse endocapillary proliferative lesions. Methods: The morphological and clinical features of 126 patients with and without diffuse endocapillary proliferation were compared. 29 out of 126 are IgA nephritis patients with diffuse endocapillary proliferation. The others are patients without diffuse endocapillary proliferation. The prognosis of the patients in the 2 groups was analyzed and the effects of diffuse endocapillary proliferation on renal progression in patients with different renal function were also analyzed. Results: Patients with diffuse endocapillary proliferation show significantly higher proteinuria (p < 0.0001) and crescent percent (p < 0.05). There is no significant difference of renal survival in patients with or without diffuse endocapillary proliferation. Further analysis indicated that patients with diffuse endocapillary proliferation had better renal survival than those without it (log rank, p = 0.0561) if their renal function was normal, whereas renal survival of patients with diffuse endocapillary proliferation was worse than of those without it if their renal function was abnormal (log rank, p = 0.0763). Conclusion: IgA nephropathy with diffuse endocapillary proliferation shows a better prognosis in the patients with normal renal function, but it is a relative risk factor for end-stage renal disease in patients with abnormal renal function.

The significance of atypical morphology in the changes of spectrum of postinfectious glomerulonephritis

The characteristics of post infectious glomerulonephritis (PIGN) now differ from what were described decades ago. After encountering several patients of PIGN with atypical morphology, we conducted this retrospective study to determine the significance in the changes of clinicopathological spectrum of the disease. Between July 2000 and February 2009, 21 cases of PIGN were identified at a medical center in Taiwan. The patients' records were reviewed with respect to clinical presentation, microbiology, serology, morphology of renal biopsy, and clinical course. The mean age was 60.4 years. All patients developed acute renal failure and the majority (66.7%) required dialysis support. Hypocomplementemia was present in 61.9% of patients. The most frequently identified infectious agent was Staphylococcus (57.1%). Histological characteristics showed two distinct patterns of PIGN. One was diffuse endocapillary proliferation typical of PIGN (61.9%) and the other was atypical pattern of focal mesangial proliferation (38.1%). In comparison, glomerular neutrophil infiltration was more commonly present in typical pattern (p = 0.018). Glomerular IgA dominant or co-dominant deposition was more frequently seen in atypical pattern (p = 0.032). However, there were no statistically significant differences in the clinical presentation and outcome between the two groups. Our data also showed that the percentage of patients with atypical morphology PIGN significantly increased over time. Atypical pattern of focal mesangial proliferative glomerulonephritis may represent a resolution stage of PIGN. The nature of subclinical infection with a more protracted course may contribute to the increasing recognition of this resolving PIGN at the time of renal biopsy. Another possible explanation is that the atypical morphology may be a peculiar pattern of poststaphylococcal glomerulonephritis which was increasingly identified in PIGN over the past 10 years.

Acute poststaphylococcal glomerulonephritis superimposed on diabetic glomerulosclerosis

Acute poststaphylococcal glomerulonephritis superimposed on diabetic glomerulosclerosis

A Proliferative Glomerulonephritis Secondary to a Monoclonal IgA

A distinct entity mimicking immune-complex-mediated glomerulonephritis characterized by a proliferative glomerulonephritis with monoclonal immunoglobulin G (IgG) deposits recently was described. We now report a case of a 35-year-old woman who presented with sudden onset of edema, proteinuria, hematuria, and hypertension. Renal biopsy showed diffuse endocapillary proliferation, mesangial cellularity, and amorphous material in the mesangium. Immunofluorescence examination showed mesangial and capillary wall staining for IgA (2+), C3 (2+), fibrinogen (2+), and lambda (2+). Congo red stain was negative. Electron microscopy showed mesangial and subendothelial deposits with a paracrystalline lattice-like substructure forming parallel linear arrays. Extensive laboratory evaluation showed a small population of monoclonal plasma cells with lambda restriction. The present case suggests that monoclonal IgA deposits also can cause proliferative glomerulonephritis. However, the presence of paracrystalline deposits in association with monoclonal IgA deposits has not been described previously.

Lupus nephritis and the anti-phospholipid antibody syndrome in pregnancy

Lupus nephritis and the anti-phospholipid antibody syndrome in pregnancy