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We report the case of a 58-year-old male patient who visited our hospital for the management of edema and proteinuria. He was diagnosed as having nephrotic syndrome, with serum total protein and albumin levels of 4.6 g/dL and 2.1 g/dL, respectively, and a urinary protein excretion level of 6.0 g/day. A percutaneous renal biopsy showed features of membranous glomerulonephritis, with capillary-wall granular deposits of IgG and C3 on immunofluorescence and subepithelial immune complex deposits on electron microscopy. No other secondary cause of membranous glomerulopathy was found even after extensive investigations. The patient was started on mycophenolate mofetil (MMF) monotherapy (1,500 mg/day), and 18 months after the start of this therapy, the proteinuria decreased to 0.5 g/day, with return to a normal serum albumin level. No digestive symptoms, kidney function worsening or increase in blood pressure were noted during treatment. These findings suggest that MMF monotherapy is effective and safe for the treatment of membranous nephropathy.

We examined the data of 24 patients with Henoch-Schönlein purpura nephritis (HSPN) over a 5-year follow-up period. Proteinuria, sediment RBC and CRP significantly decreased between the time of diagnosis and the end of the 5-year period. In the steroid usage group (n = 16), proteinuria was significantly higher, and crescent formation was significant higher at the time of diagnosis than in the non-steroid usage group (n = 8). However, there was no significant difference in the decrease in eGFR from the baseline at the end of the 5-year period between the two groups. Furthermore, to clarify the factors influencing the risk of renal function deterioration, we divided the patients into two groups, the (delta eGFR/pre eGFR) <0.25 group (n = 13) and (delta eGFR/pre eGFR) >0.25 group (n = 11), and compared the clinico-pathophysiological characteristics between the two groups. In the (delta eGFR/pre eGFR) >0.25 group, the ratio of glomerular obsolescence at the time of diagnosis was significantly higher than in the (delta eGFR/pre eGFR) <0.25 group. Glomerular obsolescence was identified as an independent risk factor for renal function deterioration. In this study, the prognosis of HSPN was related to glomerular obsolescence rather than to the disease activity. It may be necessary to consider the decrease in nephrons, in accordance with non-immunological glomerular obsolescence, in addition to immunological treatment to clarify the prognosis.

Background: Although membranous nephropathy is a common cause of nephrotic syndrome in adults, its treatment remains under debate., Methods: To clarify the effects of steroid therapy, the data of 51 Japanese adult patients with idiopathic membranous nephropathy who received treatment at our department were analyzed retrospectively. We divided the patients with nephrotic syndrome and a serum creatinine level <1.7 mg/dL, into two groups: the steroid therapy group (n=20) and the non-steroid therapy group (n=7), and compared the clinical characteristics between the two groups., Results: Significantly decreased proteinuria levels (p<0.05) after 2 and 5 years were observed in the steroid therapy group as compared to the non-steroid therapy group. There was no significant difference in the serum creatinine levels after 2 and 5 years between the steroid therapy group and the non-steroid therapy group., Conclusion: Steroid therapy in idiopathic membranous nephropathy showed good efficacy in patients with nephrotic syndrome.

Objective: The pathological and clinical findings, therapies and prognoses of MPO-ANCA-associated vasculitis, were investigated in cases who showed rapidly progressive nephritic syndrome and received renal biopsy., Methods: Vasculitis activity was evaluated by BVAS(Birmingham Vasculitis Activity Score). The renal biopsy findings were evaluated by scoring glomeruli, interstitial and vascular lesions. The renal prognoses were studied by dividing the cases into a dialysis group, which went onto maintenance dialysis in one year and another group without dialysis, which maintained renal functions., Results: The average age was 58.6 +/- 13.9 years, and the 60s age bracket was the largest. Vasculitis activity was 14.8 +/- 3.2 on the average by BVAS. CRP was 1.2 +/- 1.4 for the kidney-located type group, and 12.6 +/- 10.5 for the multiorgan-damaged group respectively, which shows the former to be significantly lower (p = 0.0079). Serum creatinine at renal biopsy was 3.57 +/- 2.31 mg/dL in the dialysis-independent group, and this was significantly lower than the serum creatinine level of 9.10 +/- 2.6 mg/dL of the dialysis group (p = 0.000259). As for the renal pathological findings, the percentage of global sclerosis among all the glomeruli was 24.7 +/- 19.9% in the dialysis-independent group vs. 68.5 +/- 19.7% in the dialysis group, which shows the latter to be significantly higher (p = 0.002)., Conclusion: CRP was significantly higher in the multi-organ-damaged group relative to the kidney-located type group. The percentage of global sclerosis determined by renal biopsy and the amount of serum creatinine at the renal biopsy were key factors in determining the renal prognosis. The absence of a significant correlation between the percentage of crescentic formation and the renal prognosis suggests the possibility of suppressing progress to global sclerosis.

A 69-year-old woman, who had been diagnosed with interstitial pneumonia at 66 years of age, was admitted to our hospital because of high fever, purpura occurring on her arms and legs, and renal dysfunction. At the time of admission, her renal function had severely deteriorated (sCr 8.2 mg/dl, 24 h Ccr 6 ml/min), she had a severe high fever (BT 39.5 degrees C), back pain, a white blood cell count of 19,540/,microl, and a CRP level of 26.7 mg/dl. Blood and urine cultures yielded identical strains of E. coli. We diagnosed sepsis caused by pyelonephritis, and started intravenous meropenem trihydrate(MEPM) at 0.5 g/day. Her renal dysfunction was severe, so we started hemodialysis therapy. Immunological examination revealed the presence of ANCA-associated glomerulonephritis. Renal biopsy before steroid therapy confirmed the diagnosis of pauci-immune-type crescentic glomerulonephritis. Based on purpura and interstitial pneumonia, along with rapidly MPO-ANCA-positive progressive glomerulonephritis (RPGN) with acute renal failure, we diagnosed microscopic polyangitis (MPA). To treat sepsis and severe pyelonephritis, we started intravenous immunoglobulin 5 g (100 mg/kg)/day for 5 days before starting immunosuppressive steroid therapy (m-PSL 1 g/day, PSL 20 mg/day) for 3 days. These treatments improved her general condition and immediately improved her renal function. It is important to prevent infection during treatment using conventional immunosuppressive therapy. These findings suggest immunoglobulin therapy to be a safe immuno-suppressive treatment that is efficacious against ANCA-associated glomerulonephritis.

Progression to chronic renal failure is influenced by the extent of irreversible interstitial fibrosis. For a quantitative evaluation of interstitial fibrosis, we attempted color extraction of the interstitial area treated with Masson-Trichrome stain using an image analyzer. The subjects were 20 cases of IgA nephropathy with a mean age of 33.4 +/- 12.3 years. The interstitial lesions were graded simultaneously by renal pathologists without clinical information. In addition, immunohistochemical studies using specific antibodies against type III collagen, alpha-smooth muscle actin and CD34 were performed to further characterize the interstitial lesions. The percentage of interstitium in the renal cortex evaluated by an image analyzer was significantly correlated with the pathological grade of fibrous interstitial damage(sigma = 0.861, p < 0.0002), the type III collagen-positive area(sigma = 0.954, p < 0.0001) and alpha-smooth muscle actin-positive area(sigma = 0.686, p = 0.0005). Moreover, the percentage of interstitium was negatively correlated with 24-hr creatinine clearance(r = -0.744, p < 0.0001) and the numbers of peritubular capillaries detected by CD34(r = -0.649, p < 0.0014). These findings suggest that color extraction of the interstitial area stained with Masson-Trichrome using image analysis is useful for a quantitative evaluation of the interstitial fibrous lesions in IgA nephropathy.

Immunotactoid glomerulopathy (ITG) demonstrates glomerular deposits that are characterized by highly organized ultrastructure. The deposits appear to be composed of immunoglobulin and complement that were negative for amyloid by Congo red stain. However, it has been difficult to distinguish the difference between ITG and fibrillary glomerulonephritis. In the present study, we performed an immunoelectron microscopic study to identify the factors that seemed to be associated with the glomerular deposits in a case of IgA-dominant ITG. IgA, light chain kappa and fibrinogen were colocalized in association with fibrillar deposits in the extracellular matrix of mesangial cells. Therefore, it is likely that the complex of IgA, light chain kappa and fibrinogen are associated with the formation of highly organized ultrastructural deposits in a case of IgA-dominant ITG.

We report a rare case of nephrotic syndrome in an elderly woman with positive antineutrophil cytoplasmic antibody(ANCA). The patient was 81 years of age and had a history of interstitial pneumonia. She was diagnosed rheumatoid arthritis(RA) at admission. Rapidly progressing renal damage was found with mild microscopic hematuria and positive ANCA. The renal biopsy findings indicated membranous nephropathy. Neither gold nor anti-rheumatic drugs had been previously administered. She may have had an RA-specific membranous nephropathy. Crescentic formation was not clear. With hematuria, the leukocyte infiltration in the capillary lumen and the change in epithelial cells of Bowman's capsules would be histological findings suggesting ANCA-associated nephritis. This is a rare report on membranous nephropathy in an RA patient with ANCA-associated nephritis.

Low-dose cyclosporin(CsA) therapy combined with prednisolone was performed in 10 adult patients with frequently relapsing minimal change nephrotic syndrome(MCNS). Oral CsA was administered at the dose of 1-3 mg/kg/day(50-150 mg/day) in combination with preceding prednisolone(32.5 +/- 13.1 mg/day). The whole blood traugh concentration of CsA was maintained at the level of 50-100 ng/ml (ranging in 35-160 ng/ml, mean: 68.0 +/- 42.8 ng/ml), and the therapy was continued for 31.7 +/- 12.7 months. The urinary protein excretion, serum total protein, albumin and total cholesterol significantly improved after treatment. The serum creatinine increased slightly at 3-6 months after treatment, but decreased to within the normal range thereafter. The frequency of relapse and the ratio of the complete remission period to the total observed period were compared between the pre-treatment period(36.6 +/- 42.5 months) and post-treatment period(31.7 +/- 12.7 months). The frequency of relapse was significantly decreased after CsA treatment(2.3 +/- 1.5 times/year-->0.7 +/- 0.7 times/year, p = 0.02). The ratio of the complete remission period to the total observed period was increased significantly after CsA therapy(61.7 +/- 24.3%-->88.6 +/- 14.5%, p = 0.01). Thus, the low dose cyclosporin(CsA) therapy combined with prednisolone was an effective treatment for adult MCNS patients who relapsed frequently under conventional prednisolone therapy.