Harumitsu Kumagai

The improvement of renal survival with steroid pulse therapy in IgA nephropathy

Background. The benefits of steroid therapy in immunoglobulin A nephropathy (IgAN) have not been established. Methods. The effect of steroids on kidney survival was retrospectively investigated in 702 patients with IgAN by multivariate analyses. Results. There were 295 men and 407 women. The median follow-up period was 62 months. One hundred and ninety-four patients were treated with oral steroids (oral steroid group). Thirty-four patients were treated with methylprednisolone (mPSL) pulse therapy (pulse steroid group) followed by oral prednisolone (PSL). In 474 patients, no steroid was used (no steroid group). The urinary protein-creatinine ratio and histological grade were significantly different among treatment groups and were highest in the pulse steroid group followed by the oral steroid group and lowest in the no steroid patients. Serum creatinine was significantly higher in the pulse steroid group than in other two groups. Eighty-five patients developed end-stage renal failure (ESRF) requiring haemodialysis. In multivariate analysis, steroid pulse therapy significantly decreased the risk of ESRF while oral steroid treatment did not improve renal survival in this cohort. Conclusion. We found that pulse steroid therapy improved kidney survivals in IgAN. Since the clinical findings and histological grade were the most severe in patients treated with mPSL pulse therapy, such therapy may prevent progression of IgAN.

Nodular glomerulosclerosis in patients without any manifestation of diabetes mellitus

Aim/Methods:  Diabetic nodular glomerulosclerosis is considered to be the specific renal lesion of diabetes mellitus (DM). However, some cases, in which nodular glomerulosclerosis was found without any manifestation of DM, have also occasionally been reported. We clinicopathologically examined seven cases without a prior history of DM. They consisted of six men and one woman with a mean age of 57 years, and included three cases with family history of DM and six cases with hypertension.

Recurrent nephrotic syndrome after living-related renal transplantation resistant to plasma exchange: report of two cases

Abstract:  We encountered two patients of recurrent nephrotic syndrome (NS) after renal transplantation that was resistant to plasma exchange (PEX). Case 1 was a 34‐year‐old man with a living‐related renal transplant for type‐I membranoproliferative glomerulonephritis (MPGN) related end‐stage renal disease (ESRD). He developed overt proteinuria 7 months post‐transplant and presented with NS 5 months later. Biopsy of the transplant kidney revealed recurrent type I MPGN, but no features of acute rejection (AR) or chronic allograft nephropathy (CAN). He was treated with cyclophosphamide (CP), oral prednisolone (40 mg/d), an anti‐platelet agent, heparin sulfate, and PEX, but the nephrotic state persisted and renal function was deteriorated. He recommenced hemodialysis 3 yr and 9 months after renal transplant. Case 2 was a 47‐year‐old male who underwent living‐related renal transplant for ESRD due to focal segmental glomerulosclerosis (FSGS). He presented with proteinuria shortly after renal transplantation. He also had frequent episodes of AR. Graft biopsy revealed recurrent FSGS. Treatment of pulse methylprednisolone and PEX was transiently effective, but NS relapsed shortly after PEX. Graft biopsy at our hospital showed features of CAN with moderate interstitial fibrosis and tubular atrophy, presence of intraglomerular foam cells but no segmental sclerosis. Treatment with 12 courses of low‐density lipoprotein apheresis (LDL‐A) reduced proteinuria from 9.6 to 2.0 g/d, and incomplete remission has been maintained for more than 1 yr after LDL‐A with slowly progressive renal dysfunction. Despite recent therapeutic advances, including the use of immunosuppressants and PEX, treatment of recurrent disease remains difficult. The LDL‐A might be useful in cases with recurrent FSGS resistant to PEX.

Physical activity, high-density lipoprotein cholesterol subfractions and lecithin:cholesterol acyltransferase in dialysis patients.

Background/Aims: Although a low level of high-density lipoprotein cholesterol (HDL-C) is an independent risk factor for atherosclerotic heart disease, the mechanism of HDL-C abnormality in hemodialysis (HD) as well as peritoneal dialysis (PD) patients is not fully understood. The purpose of this study was to investigate the relationship of physical activity with HDL-C subfractions and lecithin:cholesterol acyltransferase activity in HD and PD patients. Methods: Thirty-five HD and 26 PD patients were studied. Physical activity was estimated as the average number of steps taken per day over 7 days (steps/day). Results: When possible confounding factors were included in the stepwise multiple regression analyses, in HD patients, steps/day was significantly positively related to HDL2-C and apolipoprotein (Apo) A-I, while it was significantly positively related to HDL3-C in PD patients. When subjects were subdivided into 3 groups according to steps/day, in HD patients, the highest category of steps/day had significantly higher HDL2-C and Apo A-I than the lowest category, while such results were not observed in PD patients. Conclusion: These results suggest that the associations of physical activity with HDL-C subfractions and Apo A-I that are known in the general population are more pronounced in HD patients than PD patients.

A Case of microscopic polyarteritis nodosa with interstitial pneumonia successfully treated with steroid pulse therapy and immunosuppresive agents

We report a patient with microscopic polyarteritis nodosa (mPN) and interstitial pneumonia, who was subjected to investigation by bronchoalveolar lavage (BAL), thoracic computerized tomography (CT) and gallium-67 citrate (67Ga) scintigraphy before and after administration of glucocorticoid and immunosuppressive agents. Renal function, renal histology, interstitial inflammation of the lung, and pulmonary function and histology improved cytoplasmic autoantibody (MPO-ANCA), which decreased with decreasing disease activity after starting treatment. Interstitial pneumonia may be associated with pulmonary capillaritis due to mPN. Methylprednisolone pulse therapy followed by oral prednisolone and immunosuppressive agents is considered to be an effective therapeutic strategy for combined mPN and interstitial pneumonia.

Clinical study concerning the factors which influence the progression of left ventricular hypertrophy in chronic hemodialysis patients

1回透析の除水量 (UF) が慢性血液透析 (HD) 患者の左室肥大 (LVH) 進展の要因になりうるか否かについてretrospectiveに検討した. 透析導入後1年以内 (平均6ヵ月) に心エコーを施行し, 以後2年間にわたり左室壁厚と左室心筋重量 (LVM) の変化を観察しえたHD患者29例を対象に, UFにより1kg未満 (group 1, n=10), 1kg以上2kg未満 (group 2, n=13), 2kg以上 (group 3, n=6) の3群に分類し, 各群での左室形態の変化を比較した. 透析時間は全例1回5時間で, 週3回透析を施行し, 観察期間中透析方法の変更はなかった. 心エコーは透析前に施行し, Mモード心エコー図から心室中隔厚, 左室後壁厚, 左室拡張末期径, 左室収縮末期径, LVM, Fractional shortening (FS) を測定した. なお, LVHに影響する因子として平均血圧, Htを測定するとともに, 体重管理の状況を評価するためdry weightの経過も観察した.その結果, LVMはgroup 1ではcontrol (C) 期 (212±66g), 1年目 (216±79g), 2年目 (205±102g) と経過中不変であり, group 2ではC期 (202±62g), 1年目 (224±84g), 2年目 (234±103g) とC期に比し1年目に有意の増加 (p<0.05) を, group 3ではC期 (194±37g), 1年目 (254±55g), 2年目 (283±60g) と経年的に有意の増加 (p<0.05) を認めた. 一方, dry weightはgroup 1では経年的に, group 2では2年目のみ減少が認められたが, group 3では経過中不変であった. なお, 平均血圧, Htは各群とも有意の差は認められず, また経過中いずれも不変であった. 左室収縮能はgroup 2, group 3においてLVHの進展にもかかわらず, 正常範囲に保たれていた.以上の結果から, UFはHD患者においてLVH進展の要因となりうることが示唆された. また, 1kg未満にUFを保つことがその予防に重要であると考えられた.