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Background of this guideline Rapidly progressive glomerulonephritis (RPGN) is defined in Japan as ''a syndrome that progresses rapidly within a few weeks or months to renal failure and is accompanied by urinary findings of nephritis.'' The clinical concept of RPGN includes various renal diseases that cause renal function to deteriorate over a subacute course. Necrotizing crescentic glomerulonephritis is often observed in histopathological findings. In 2002, a joint committee formed by JSN and a research group on progressive renal disorders from the specific disease program of the Ministry of Health, Labour, and Welfare released Japan's first ''Clinical Guidelines for Rapidly Progressive Glomerulonephritis.'' These landmark guidelines were based on the results of research conducted overseas and a national survey on RPGN and took the particular characteristics of Japan into consideration. The RPGN guidelines were divided into diagnostic guidelines for early discovery and guidelines for making definitive diagnoses. RPGN was categorized into either a myeloperoxidase (MPO-ANCA) or proteinase-3 antineutrophil cytoplasmic (PR3-ANCA) type based on ANCA-related vasculitis. Furthermore, a practical therapeutic algorithm was created for MPO-ANCA types that took into consideration factors such as clinical severity, age, and presence of dialysis. Treatment guidelines for anti-GBM antibody RPGN were also presented. These guidelines were widely used in Japan and contributed greatly to improving RPGN prognosis. These guidelines were revised 9 years later, in 2011, and published as ''Clinical Guidelines for Rapidly Progressive Glomerulonephritis-2nd edition.'' This edition took into account medical advances that had occurred since 2002, and eGFR, not serum creatinine level, was adopted for diagnosing RPGN. Moreover, MPO-ANCA RPGN and PR3-ANCA RPGN were combined under ANCA-positive RPGN. The new edition also included concise statements for treatments and dealing with complications. Since then, marked progress has been made in RPGN research both in Japan and overseas. Globally, kidney disease improving global outcomes (KDIGO) released

As a proof of concept that removal of blood amyloid-β (Aβ) can reduce Aβ deposition in the brains of patients with Alzheimer's disease, cortices of patients who had undergone hemodialysis (HD), which removes Aβ from the blood, were histochemically analyzed; postmortem brain sections were stained with anti-Aβ antibodies. Brains from patients who had undergone HD had significantly fewer senile plaques than those of patient who had not undergone HD. This significant difference was also confirmed by silver staining. Our findings suggest that removal of blood Aβ by hemodialysis results in lower accumulation of Aβ in the brain.

To obtain the proof of concept of a novel therapy for Alzheimer's disease (AD), we conducted two prospective studies with hemodialysis patients who had amyloid β protein (Aβ) removed from their blood three times a week. One major pathological change in the brain associated with AD is Aβ deposition, mainly 40 amino acids Aβ1-40 and 42 amino acids Aβ1-42. Impaired Aβ clearance is proposed to be one cause of increased Aβ in the AD brain. Thus, we hypothesized that an extracorporeal removal system of Aβ from the blood may remove brain Aβ and be a useful therapeutic strategy for AD. In the first prospective study, plasma Aβ levels and the cognitive function of 30 hemodialysis patients (65-76 years old) were evaluated at baseline as well as 18 or 36 months after. Although plasma Aβ1-40 levels either decreased or remained unchanged, levels of Aβ1-42 either remained unchanged or increased at the second time point. Mini-Mental State Examination scores of most subjects increased or were maintained at the second time point. Aβ1-40 influx into the blood correlated with MMSE at the second time point. In the second prospective study, five patients (51-84 years old) with renal failure were evaluated before and after the initiation of hemodialysis. Plasma Aβ levels decreased, while cognitive function improved after initiating blood Aβ removal. Therefore, long-term hemodialysis, which effectively removes blood Aβ, might alter Aβ influx and help maintain cognitive function.

The accumulation of amyloid β protein (Aβ) in the brain reflects cognitive impairment in Alzheimer's disease. We hypothesized that the rapid removal of Aβ from the blood by an extracorporeal system may act as a peripheral Aβ sink from the brain. The present study aimed to determine the optimal materials and modality for Aβ removal by hemodialyzers. In a batch analysis, hollow-fiber fragments of polysulfone (PSf) and polymethyl methacrylate (PMMA) showed greater removal efficiency of Aβ than did other materials, such as cellulose-triacetates and ethylene-vinyl alcohol copolymer (PSf:PMMA at 30 min, 98.6 ± 2.4 %:97.8 ± 0.4 % for Aβ1-40 and 96.6 ± 0.3 %:99.0 ± 1.0 % for Aβ1-42). In a modality study, the Aβ solution was applied to PSf dialyzers and circulated in the dialysis and Air-filled adsorption-mode (i.e., the outer space of the hollow fibers was filled with air) or phosphate-buffered saline (PBS)-filled adsorption modes. The Aβ1-40 removal efficiency of the pre/post dialyzer in the Air-filled adsorption-mode was the highest (62.4 ± 12.6 %, p = 0.007). In a flow rate study in the Air-filled adsorption-mode, 200 mL/min showed the highest Aβ1-40 reduction rate of pool solution (97.3 ± 0.8 % at 15 min) compared with 20 mL/min (p = 0.00001) and 50 mL/min (p = 0.00382). PMMA dialyzers showed similar high reduction rates. Thus, the optimal modality for Aβ removal was the adsorption-mode with PSf or PMMA hollow fibers at around 50 mL/min flow rate, which seems to be suitable for clinical use.

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In this report, we describe 5 patients with cholesterol atheroembolic renal failure. In 3 of the 5 patients, combined therapy with corticosteroids and plasma exchange was performed. These 3 patients survived, with 2 showing an improvement in renal function. The 2 remaining patients died of multifactorial causes. The literature on therapy for cholesterol atheroembolic renal failure is reviewed and the efficacy of combined therapy by use of corticosteroids and plasma exchange is evaluated.

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