A case of membranous glomerulonephritis in renal allograft

A 39-year-old man who had been diagnosed with immunoglobulin A (IgA) nephropathy underwent renal transplant from his father. The operation was performed under cyclosporine, prednisolone and mizoribine treatment. Renal function was stable following transplantation, but proteinuria ranged between 1 g/day and 3 g/day. Protocol biopsy 1 year after transplantation revealed membranous glomerulonephritis, with IgG and C3 deposits under immunofluorescence, and subepithelial deposits detected on electron microscopy. The patient was treated by limiting protein intake, controlling blood pressure and administering candesartan. Proteinuria decreased from 5.6 g/day to 1 g/day, but a graft biopsy was performed 2 years after transplantation because of a slightly increasing creatinine level. There was no sign of rejection, and IgG and C3 deposits observed under immunofluorescence had decreased. After the graft biopsy, the creatinine level was stable and proteinuria decreased to 0.7 g/day. In conclusion, de novo nephropathy such as membranous glomerulonephritis should also be considered a possible cause of proteinuria following renal transplantation.     

Primary focal sclerosing glomerulonephritis: A clinicopathological analysis

SUMMARY: The clinical and laboratory features, renal biopsy findings, and outcome of 68 patients with primary focal sclerosing glomerulonephritis were studied. the cumulative probability of not progressing to end-stage renal failure (ESRF) was 0.92 at 5 years and 0.73 at 10 years after presentation, and was significantly worse in patients with hypertension or severe renal impairment (serum creatinine >0.24 mmol/L) at presentation. Proteinuria of up to 1gm/day was associated with an excellent prognosis, whereas proteinuria of 1–3 gm/day and >3 gm/day had similar and poorer survivals. an adverse outcome was associated with, at presentation, age less than 30 years, hypertension, a family history of glomerulonephritis, cigarette smoking, impaired renal function, and heavy proteinuria. Renal biopsy findings which correlated with progressive renal failure included a higher percentage of glomeruli with global or segmental sclerosis, and the degrees of tubular atrophy, interstitial fibrosis, interstitial inflammation and arterial thickening. During follow-up the degrees of renal impairment and proteinuria, persistence or development of hypertension, transient decreases of renal function of >10%, and the total number of red cells and casts on centrifuged urine microscopy were all predictive of progressive renal disease. Multivariate analysis demonstrated that the indices with adverse effects on outcome induced all of the above except tubulointerstitial and vascular changes on renal biopsy. It is concluded that the prognosis may be better than has been suggested in the literature. It is possible to predict which patients are likely to have an adverse outcome, and this should assist with therapeutic decisions likely to retard progression of disease.     

A case of hypocomplementemic mesangial proliferative glomerulonephritis progressing to focal membranoproliferation without aggravation of urinalysis]

We reported a 14-year-old boy with hypocomplementemic mesangial proliferative glomerulonephritis progressing to focal membranoproliferation without aggravation of urinalysis. He was pointed out as having asymptomatic hematuria by a school urinalysis screening, and revealed mild hematuria and proteinuria (not nephrotic). The laboratory data showed severe hypocomplementemia and high titers of antistreptolysin O (ASO) and antistreptokinase (ASK). A renal biopsy specimen obtained 2 months after the onset showed diffuse mesangial proliferation, and did not display any characteristic changes in membranoproliferative glomerulonephritis by either light or electron microscopy. After eight months of observation, a second renal biopsy was performed in order to examine morphological changes, because severe hypocomplementemia, mild hematuria and proteinuria had persisted. The second biopsy specimen showed C3 deposition with a mesangiocapillary pattern detected by immunofluorescence microscopy and diffuse mesangial proliferation by light microscopy. In some glomeruli, electron microscopy showed focal mesangial interposition, which was considered to be a histological feature of focal membranoproliferative glomerulonephritis. Intravenous methylprednisolone pulse therapy and subsequent glucocorticoid administration (60 mg of prednisolone every other day) was performed. The serum complement level was elevated just after the methylprednisolone pulse therapy, but fell to the previous value within 2 months. The hypocomplementemia, even if proteinuria and hematuria were mild, indicated the existence of glomerular change in this case.     

Primary focal sclerosing glomerulonephritis: A clinicopathological analysis

The clinical and laboratory features, renal biopsy findings, and outcome of 68 patients with primary focal sclerosing glomerulonephritis were studied. The cumulative probability of not progressing to end-stage renal failure (ESRF) was 0.92 at 5 years and 0.73 at 10 years after presentation, and was significantly worse in patients with hypertension or severe renal impairment (serum creatinine >0.24 mmol/L) at presentation. Proteinuria of up to 1gm/day was associated with an excellent prognosis, whereas proteinuria of 1–3 gm/day and >3 gm/day had similar and poorer survivals. An adverse outcome was associated with, at presentation, age less than 30 years, hypertension, a family history of glomerulonephritis, cigarette smoking, impaired renal function, and heavy proteinuria. Renal biopsy findings which correlated with progressive renal failure included a higher percentage of glomeruli with global or segmental sclerosis, and the degrees of tubular atrophy, interstitial fibrosis, interstitial inflammation and arterial thickening. During follow-up the degrees of renal impairment and proteinuria, persistence or development of hypertension, transient decreases of renal function of >10%, and the total number of red cells and casts on centrifuged urine microscopy were all predictive of progressive renal disease. Multivariate analysis demonstrated that the indices with adverse effects on outcome included all of the above except tubulointerstitial and vascular changes on renal biopsy. It is concluded that the prognosis may be better than has been suggested in the literature. It is possible to predict which patients are likely to have an adverse outcome, and this should assist with therapeutic decisions likely to retard progression of disease.     

Comparison of higher dose of losartan treatment with losartan plus carvedilol and losartan plus ramipril in patients with glomerulonephritis and proteinuria

BACKGROUND: Proteinuria may cause a worsening of accompanying renal disease or even lead to glomerulosclerosis. There is no data about the effect of carvedilol on patients with proteinuric (>0.5 g/day) glomerulonephritis. This study aimed to compare the effects of carvedilol with ramipril and losartan in patients with proteinuric glomerulonephritis. METHODS: Twenty-one glomerulonephritis patients were followed for 12 months. Patients were divided into three groups. All patients were treated with losartan 50 mg once daily for two weeks. After two weeks (baseline), patients were given additional medications: 50 mg losartan, 5 mg ramipril, and 25 mg carvedilol were given additionally to the patients in groups 1, 2, and 3 respectively. RESULTS: Baseline mean proteinuria values of patients in groups 1, 2 and 3 were 1.6 +/- 1.1 g/day, 2.1 +/- 1.3 g/day, and 1.4 +/- 1.2 g/day, respectively. These values decreased to 0.5 +/- 0.7 g/day, 0.6 +/- 0.7 g/day, and 0.9 +/- 0.9 g/day, respectively, at the end of the 12th month. These results were statistically significant only in group 1 (p = 0.04). The rational variation of proteinuria between the first and 12th month of losartan, ramipril, and carvedilol were -61%, -62%, and -27%, respectively. The decreases in blood pressures between baseline and the first, sixth, and twelfth-month measurements were significant in all groups. CONCLUSIONS: Thee results showed that angiotensin-converting enzyme inhibitors (ACEIs) and angiotensin receptor blockers (AT1ras) provide marked decreases in proteinuria, making their use indisputable in patients with glomerulonephritis. Carvedilol was not found to be as effective as ACEIs and AT1ras in decreasing proteinuria and preserving renal function.     

Clinicopathologic study of glomerulonephritis showing double-contour lesions

Background To elucidate the pathologic significance of double-contour lesions of glomerular capillary walls, we determined how the clinical course of patients with diffuse and global double-contour lesions differs from that of patients with segmentally located double-contour lesions. Methods In 26 out of 308 cases of idiopathic glomerulonephritis double contour lesions and serum complement 3 (C3) deposition along capillary walls were histologically examined. Results Most patients with diffuse (affecting more than 80% of all glomeruli) and global (affecting more than 75% of each glomerulus) double-contour lesions exhibited a persistent profound proteinuria and a deterioration of renal function (assessed via serum creatinine measurements) during a mean observation period of 66 months, even when a transient remission was observed. The amelioration of hypocomplementemia correlated significantly with an improvement in proteinuria (P<0.05). A follow-up biopsy of patients revealed some cases in which there was: (1) no amelioration of the glomerular lesion despite improvements in renal function, proteinuria and hypocomplementemia; (2) an amelioration of the glomerular lesion paralleling an increasing stability in renal function and a reduction in proteinuria; or (3) deterioration of the glomerular lesion paralleling a decrease in renal function, an increase in proteinuria, and persistent hypocomplementemia. Conclusions These findings indicate that the clinical characteristics of mesangiocapillary glomerulonephritis differ from those of other types of mesangial proliferative glomerulonephritis with segmentally-located double-contour lesions. A spot, unrepeated biopsy in cases of atypical mild mesangiocapillary lesions provides insufficient information to reach a diagnosis of mesangiocapillary glomerulonephritis.     

Therapeutic approach of patients with IgA nephropathy

Immunoglobulin A nephropathy (IgAN) is the most commonly encountered primary glomerulonephritis and it usually follows an indolent clinical course. However, hypertensive patients with proteinuria and renal insufficiency at presentation and patients with severe histological involvement are at high risk to develop end stage renal failure. There is no consensus for the treatment of patients with IgA nephropathy. In general, patients with normal renal function, mild proteinuria (3 g/24 h) and in progressive disease despite treatment with ACE inhibitors. Fish oil might be an alternative to corticosteroids in cases with renal insufficiency and chronic histological lesions. Combinations of corticosteroids and cytotoxic drugs are saved for patients with IgA nephropathy and a rapidly progressive course.     

Comparison of Higher Dose of Losartan Treatment with Losartan Plus Carvedilol and Losartan Plus Ramipril in Patients with Glomerulonephritis and Proteinuria

Background. Proteinuria may cause a worsening of accompanying renal disease or even lead to glomerulosclerosis. There is no data about the effect of carvedilol on patients with proteinuric (>0.5 g/day) glomerulonephritis. This study aimed to compare the effects of carvedilol with ramipril and losartan in patients with proteinuric glomerulonephritis. Methods. Twenty-one glomerulonephritis patients were followed for 12 months. Patients were divided into three groups. All patients were treated with losartan 50 mg once daily for two weeks. After two weeks (baseline), patients were given additional medications: 50 mg losartan, 5 mg ramipril, and 25 mg carvedilol were given additionally to the patients in groups 1, 2, and 3 respectively. Results. Baseline mean proteinuria values of patients in groups 1, 2 and 3 were 1.6 ± 1.1 g/day, 2.1 ± 1.3 g/day, and 1.4 ± 1.2 g/day, respectively. These values decreased to 0.5 ± 0.7 g/day, 0.6 ± 0.7 g/day, and 0.9 ± 0.9 g/day, respectively, at the end of the 12^th month. These results were statistically significant only in group 1 (p = 0.04). The rational variation of proteinuria between the first and 12^th month of losartan, ramipril, and carvedilol were ?61%, ?62%, and ?27%, respectively. The decreases in blood pressures between baseline and the first, sixth, and twelfth-month measurements were significant in all groups. Conclusions. Thee results showed that angiotensin-converting enzyme inhibitors (ACEIs) and angiotensin receptor blockers (AT1ras) provide marked decreases in proteinuria, making their use indisputable in patients with glomerulonephritis. Carvedilol was not found to be as effective as ACEIs and AT1ras in decreasing proteinuria and preserving renal function.     

Clinico-pathological correlates in IgA nephropathy

Summary: There were 2758 biopsies of glomerulonephritis diagnosed in the Department of Pathology in the 20 years from 1976 to 1995. Of these 1893 (76.1%) were of primary glomerulonephritis while 577 (23.2%) were of secondary glomerulonephritis. Immunofluorescence studies were available in 1494 (80%) cases. Predominantly mesangial IgA staining was seen in 49.1% of cases, thus identifying them as IgA nephropathy. Mesangial glomerulonephritis was found in 79.1% of cases whilst 17.7% had sclerotic lesions either focal or global. One hundred and fifty-one patients were followed up. Of these, 98 (65%) were detected through health screening while 53 (35%) presented with symptoms. Uncontrolled hypertension, proteinuria of more than 2 g, the presence of crescents and glomerulosclerosis on biopsy were unfavourable prognostic factors. Hypertensive patients also had a higher incidence of medial hyperplasia of the blood vessels. However IgA nephropathy is a benign disease with a cumulative renal survival of 91% after 6 years.     

The product of duration and amount of proteinuria (proteinuria index) is a possible marker for glomerular and tubulointerstitial damage in IgA nephropathy

BACKGROUND/AIMS: IgA nephropathy (IgAN) is one of the major causes for chronic renal failure (CRF). Presence of massive proteinuria, hypertension, increased serum creatinine level and sclerotic histopathological changes of the glomerulus are known to be determinants for the progression of CRF. However, the relationships between duration of proteinuria/hematuria and histopathological changes, which may be correlated with the renal prognosis, have not been clarified. METHODS: A cross-sectional, univariate analysis of clinical parameters on the four glomerular and three tubulointerstitial histopathological grades in 57 untreated biopsy-proven IgAN patients (M/F = 32/25) was performed. RESULTS: The age at the time of renal biopsy (35.2 +/- 13.0 years; mean +/- SD), average duration of proteinuria (5.3 +/- 5.8 years), mean urinary protein excretion (0.99 +/- 1.22 g/day), serum creatinine (Cr 0.97 +/- 0.28 mg/dl), Cr clearance (Ccr 75.5 +/- 29.4 ml/min), and blood urea nitrogen (BUN 15.4 +/- 3.9 mg/dl) were well correlated with both histopathological grades. The product of duration (years) and urinary protein excretion (g/day) at the time of renal biopsy was more significantly correlated with glomerular and tubulointerstitial histopathological grades and serum Cr. CONCLUSION: The natural course of IgAN is steadily progressive depending on the duration and amount of proteinuria. The product of these two factors (proteinuria index) may be a useful predictor for glomerular and interstitial histopathological changes and the fate of renal function in IgAN.     

Henoch-Schönlein Purpura in adults: outcome and prognostic factors

Henoch-Sch?nlein Purpura nephritis (HSPN) has been extensively studied in children but, its natural history in adults is much less known. A cohort of 250 adults suffering HSP was retrospectively analyzed for a median follow-up period of 14.8 yr. All patients had biopsies consistent with HSP (predominant IgA mesangial deposits) associated with purpura, bowel angina, and/or abdominal pain. At presentation, palpable purpura was present in 96% of patients, and arthritis was reported in 61%, and gastrointestinal involvement in 48%. Thirty-two percent of the patients showed renal insufficiency (Creatinine clearance [CrCl] <50 ml/min), usually associated with proteinuria (99%) and/or hematuria (93%). Endocapillary glomerulonephritis was the most frequent lesion on renal biopsy (61%). At the end of follow-up, patient survival was only 74%. The first cause of death was carcinoma (most of them of respiratory or digestive tract). Regarding renal function, 11% of patients reached end-stage renal failure, 13% exhibited severe renal failure (CrCl <30 ml/min), and 14% moderate renal insufficiency (CrCl <50 ml/min). Clinical remission defined as the absence of proteinuria, hematuria, and a normal renal function was achieved in only 20%. This is a retrospective study; therefore, it is not possible to demonstrate any steroid and/or cyclophosphamide efficacy in diminishing the incidence of renal insufficiency. Multivariate analysis demonstrated that renal function impairment and proteinuria level at presentation and, on renal biopsy, the degree of interstitial fibrosis, percentage of sclerotic glomeruli, and presence of glomeruli with fibrinoid necrosis were associated with a poor renal prognosis. The data indicate that clinical presentation of HSPN in adults is severe and its outcome relatively poor, worse than in children. Identification of clinical and histologic prognostic factors may permit the design of appropriate therapeutic prospective studies.     

Superimposition of poststreptococcal acute glomerulonephritis on the course of IgA nephropathy

A 17-year-old male with poststreptococcal acute glomerulonephritis (PSAGN) superimposed on the course of IgA nephropathy is presented. The histological findings of the first renal biopsy showed mild IgA nephropathy with a mesangial deposition of IgA and C3. Eighteen months later, acute nephritic syndrome with hypocomplementemia and rising antihyaluronidase titer occurred 10 days following the onset of an upper respiratory infection. The second renal biopsy revealed severe diffuse endocapillary proliferative and exudative glomerulonephritis with cellular crescents in 70% of the glomeruli. Immunofluorescence showed granular staining of C3 alone along the capillary walls. The pre-existing IgA deposits had disappeared. Typical 'humps' were observed by electron microscopy. The symptoms were gradually resolved by intensive steroid and anticoagulant therapy. Five months after the episode of acute nephritic syndrome, the patient was clear of symptoms except for mild proteinuria and hematuria. The third renal biopsy at that time showed morphologic changes similar to those of the first renal biopsy with mild mesangial IgA deposits.     

Renal histopathology and clinical course in 94 patients with Wegener's granulomatosis.

BACKGROUND: The main purpose of this study was to examine histopathological changes seen in renal biopsies from patients with Wegener's granulomatosis (WG) with varying degrees of renal involvement and to study possible correlations between the morphological variables and the severity of the disease. METHODS: Ninety-four patients with WG and active renal disease were included in this retrospective study. All patients had a percutaneous renal biopsy taken on their first admission to the hospital and 14 patients had a second biopsy. The patients were followed for a median of 42.5 months (range 0.5-184). RESULTS: Segmental necrotizing glomerulonephritis and extracapillary proliferation were present in 85.1 and 91.5% respectively. Of seven patients (7.4%) with normal serum creatinine and urinary protein excretion <0.5 g/day, all had crescents and six had segmental glomerular necrosis. Serum creatinine at biopsy correlated significantly with the percentage of glomeruli with crescents (rho=0.52, P=0.0004), with necrosis (rho=0.36, P=0.002) and with the percentage of normal glomeruli (rho=-0.55, P=0.0003). On a multivariate analysis, only the percentage of normal glomeruli was significantly associated with renal function and development of end-stage renal disease. In 14 second biopsies after a mean of 41.2 (+/-26) months, chronicity scores had increased significantly in 13 biopsies in spite of full immunosuppressive treatment. CONCLUSION: Although renal biopsy is of value in defining renal involvement in WG, it is of limited help in the early stage of the disease in predicting renal outcome for the individual patient. A follow-up biopsy can be useful in revealing the degree of activity and chronicity and hence be of importance for the choice of further therapy.     

The significance of extracapillary proliferation. Clinicopathological review of 60 patients.

Renal biopsy and clinical data from 60 patients with extracapillary proliferation (crescent formation) in greater than or equal to 50% of glomeruli were correlated. Nephropathy was related to infection (15 cases) malignancy (4 cases) and trichlorethylene exposure (2 cases). Isolated proteinuria was found 0.5-20 years before biopsy in 16 patients. Outcome was significantly related to percentage of crescentic involvement. Oligoanuria and impaired function at presentation were bad prognostic signs but preceding infection was favourable. Diverse histological and immunofluorescent findings indicate that extracapillary glomerulonephritis is not a single entity. The clinical course is not always rapidly progressive.     

Analysis of allograft biopsy specimens in renal transplants with proteinuria: is proteinuria a culprit of graft loss?

It has been proposed that proteinuria occurring after renal transplantation may be not only a marker but also a culprit of allograft dysfunction. We retrospectively analyzed the data from 55 patients who underwent transplant renal biopsy for proteinuria and/or azotemia occurring beyond 1 year after transplantation. Proteinuria was considered as significant when > or = 30 mg/dL, and the results of transplant biopsy were categorized according to the Banff 97 classification. Logistic regression was used to estimate odds ratios (OR) for graft loss associated with proteinuria and transplant pathology. The patients were followed for 86.0 +/- 32.8 months after transplantation, and transplant biopsy was performed at 54.1 +/- 31.0 months. Proteinuria at 1 year after transplantation noted in 29.1% of patients was not significantly associated with graft loss (OR = 1.94, 95% CI from 0.59 to 6.41). In addition, proteinuria at the time of transplant biopsy was not significantly associated with graft loss. Chronic allograft nephropathy was the most frequent transplant pathology. Only glomerulonephritis was significantly associated with proteinuria at the time of the transplant biopsy. On the other hand, graft loss was significantly associated with the presence of proteinuria both at 1 year after transplant biopsy and at the final follow-up. These results suggest that posttransplantation proteinuria is an important marker of graft dysfunction, but is not predictive of graft loss in biopsy-proven cases. Appropriate management guided by the results of a transplant biopsy may improve the outcome.     

Factors associated with proteinuria in renal transplant recipients treated with sirolimus

Although sirolimus (SRL) use in renal allograft recipients (RTX) is associated with improved renal function, proteinuria develops in a significant proportion. 48 SRL-treated RTX were evaluated for development of proteinuria and stratified by level of proteinuria after SRL therapy. The Proteinuria Group ( n  = 25, 52.1%) had new-onset proteinuria or >25% increase in proteinuria following SRL conversion; the Nonproteinuria Group had stable proteinuria <0.5 g/day throughout. There was a higher proportion of male RTX and female donors to male recipients in the Proteinuria Group, (24% vs. 10%, P  = 0.008). Calcineurin inhibitor- and statin usage were significantly higher in the Nonproteinuria Group (8% vs. 17%, P  = 0.046; 28% vs. 83%, P  < 0.001 respectively) whereas biopsy-proven acute rejection was higher in the Proteinuria Group (68% vs. 33%, P  = 0.037). SDS-PAGE analysis of urine from 23 RTX in the Proteinuria Group demonstrated glomerular proteinuria in 100% and tubular proteinuria in 87%. While male gender and gender mismatch may impact on glomerular proteinuria through inadequate nephron dose and subsequent hyperfiltration, concurrent cyclosporine use may mitigate the development of proteinuria in SRL-treated patients, through afferent arteriolar vasoconstriction. Glomerular injury occurring following acute rejection may further contribute to glomerular proteinuria. Statins, through their anti-inflammatory and anti-fibrotic effects, may protect against development of proteinuria.     

Successful treatment of MRSA-associated glomerulonephritis with antibiotic therapy

We report a case of methicillin-resistant Staphylococcus aureus (MRSA)-associated glomerulonephritis treated with antibiotic therapy. A 67-year-old man was admitted to our hospital because of proteinuria, hematuria, purpura, and high fever one month after a graft replacement of an abdominal aortic aneurysm. MRSA was detected in specimens of his blood, sputum, and joint fluid. Before his operation, he had shown no renal abnormalities. He presented with a rapid deterioration of renal function following MRSA infection. Maximum level of proteinuria was 1.5 g/day, serum creatinine (Cr) was 3.5 mg/dl, and blood urea nitrogen was 57 mg/dl. Renal biopsy revealed necrotizing crescentic glomerulonephritis. Immunofluorescence examination showed IgA and C3 deposits. Clinical and pathological examinations showed the typical features of MRSA-associated glomerulonephritis. Vancomycin and fosfomycin were administered intravenously. The serum level of C-reactive protein fell from 22.0 mg/dl to 0.1 mg/dl. Proteinuria also decreased and the patient's renal function improved in parallel with the decreased activity of MRSA infection. After three months of antibiotic treatment, proteinuria was negative and the level of serum Cr had dropped to 0.9 mg/dl. These findings suggest that antibiotic treatment can lead to complete remission of MRSA-associated glomerulonephritis.     

Renal disease and syphilis: a report of nephrotic syndrome with minimal change disease

A case of nephrotic syndrome and acute renal failure in a 74-year-old man with latent syphilis is described. A renal biopsy demonstrated focal global sclerosis in three of nine glomeruli, however, the remaining glomeruli revealed typical lesions of minimal change disease. Previous reports of renal involvement in syphilis have described membranous glomerulonephritis, mesangial and endothelial cell proliferative glomerulonephritis, and, recently, rapidly progressive glomerulonephritis. The proteinuria and renal failure resolved after penicillin therapy alone. This response strongly suggested that there was a causal relationship between the syphilis and the nephrotic syndrome. This is the first report of such a relationship.     

Segmental glomerulosclerosis in IgA nephropathy after renal transplantation: relationship with proteinuria and therapeutic response to enalapril

INTRODUCTION: Although graft dysfunction has been increasingly reported in post-transplant IgA nephropathy (Tx-IgAN), intragraft morphological changes have been largely overlooked. We evaluated glomerular changes in Tx-IgAN to identify the histological features pertaining to significant proteinuria and therapeutic response to enalapril. MATERIALS AND METHODS: Fifty-four renal allograft biopsies, diagnosed as Tx-IgAN at a median of 46 months after transplantation, were the subject of the study. In 10 patients, glomerular morphometry was performed. In 14 patients who have been treated with enalapril for more than 12 months, we correlated the therapeutic response to enalapril with allograft histology. RESULTS: No uniform pattern was found in the glomeruli of Tx-IgAN. The glomerular mesangium was mostly indistinct. Interstitial fibrosis was negative or mild in 88.9%. By morphometry, the glomerular tuft areas and mesangial areas were significantly larger in Tx-IgAN than those of the normal native kidney (p < 0.05), but were not different from transplant cases without glomerulonephritis. Proteinuria of >/=1 g/24 h was correlated with glomerulosclerosis, interstitial fibrosis and interstitial inflammation at time of biopsy (p < 0.005). The presence of segmental sclerosis (SS) correlated well with the amount of 24-h proteinuria (p < 0.001). After treatment with enalapril, the amount of proteinuria reduced in 64.3%. Therapeutic response to enalapril tended to be less effective in patients having SS (28.6 versus 71.4%), but this finding did not reach a statistical significance. CONCLUSIONS: Significant proteinuria was associated with advanced chronic injury, especially with the presence of SS in Tx-IgAN, but anti-proteinuric effect of enalapril was not affected by graft histology. It remains to be clarified whether glomerular mesangial expansion plays a role in graft dysfunction in a subset of Tx-IgAN showing prominent mesangial changes.     

Systemic non-amyloidotic fibril deposition disease: a probable variant form of fibrillary glomerulonephritis

Fibrillary glomerulonephritis (FGN) is characterized by deposition of non-amyloidotic fibrillary material in glomeruli, and most patients with the disease show heavy proteinuria and hematuria, and progress into end-stage renal failure. We report a 62-year-old woman with FGN who showed mild proteinuria without hematuria and developed rapidly progressive renal failure requiring hemodialysis. Renal biopsy showed severe tubulointerstitial injury associated with non-amyloidotic fibrillary deposits in the tubular basement membrane, interstitium and vessel walls, in addition to glomeruli. The patient died from liver abscess 1 year after the introduction of hemodialysis. Postmortem examination showed the presence of non-amyloidotic fibrillary deposits arranged in tightly packed electron-dense and bundle-shaped structures in many organs. These findings suggest systemic non-amyloidotic fibril deposition in FGN.