近年,末期腎不全から透析療法に導入される患者数は増加の一途を辿っているが,その代表的な原因疾患としてIgA腎症があげられる。IgA腎症は,わが国に高率にみられることから本症治療の確立が強く望まれている。1995年,「IgA腎症診療指針」が提唱され,それまで混沌としていた予後判定基準とその基準に従った治療指針が発表された。この診療指針によって,治療の基本的方向性が示されたことは,IgA腎症の治療に大きく貢献してきたものと思われる。　今回,厚生労働省特定疾患進行性腎障害に関する調査研究班IgA腎症分科会が行った全国予後調査や多施設共同研究によって集積されたエビデンスをもとに「IgA腎症診療指針」(第2版)を刊行するに至った。臨床や病理診断の場で活用されることを願うものである。この指針は,現時点での"まとめ"であり,皆様のご意見や新知見に基づいて今後とも改訂されていくものと期待している。そうすることで,末期腎不全への進展を少しでも抑制したいと考えている。

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Possible role of soluble erythropoietin receptors in renal anemia Mika SAKAGUCHI, Yasuhiro MAEDA, Yoshito NAIKI, Hirofumi HASEGAWA, and Akihisa KANAMARU Department of Internal Medicine, Division of Hematology, Nephrology, and Rheumatology, Kinki University School of Medicine, Osaka, Japan Recombinant human erythropoietin (rHuEpo) is effective for the treatment of renal anemia associated with chronic renal failure (CRF) . However, we have encountered some patients with CRF who have sometimes developed a resistance to rHuEpo. This resistance can be due to iron or folate deficiency, aluminum toxicity, hyperparathyroidism, or auto-antibodies for rHuEpo. In this study, we focused on the soluble erythropoietin receptor(sEpoR), which can bind to rHuEpo. To demonstrate the possibility that the sweeping of rHuEpo by sEpoR results in resistance to rHuEpo, we performed a bioassay using the rHuEpo-dependent cell line, UT7/EPO. The results showed that recombinant mouse sEpoR (rmsEpoR) can reduce the proliferation of UT7/EPO induced by rHuEpo in a dose-dependent manner. We consider that this cell line could be a useful tool in a bioassay to detect the inhibitory factor(s) against Epo. We selected sera from three groups of patients with renal anemia associated with CRF who were receiving hemodialysis three times a week : the first was a patient group that needed a high dose of rHuEpo (7, 500-9, 000 unit/dialysis), the second was a patient group that needed an intermediate dose of rHuEpo (4, 500 unit/dialysis), the third was a patient group that needed a low dose of rHuEpo (below 1, 500 unit/ dialysis). Interestingly, the proliferation of UT7/EPO determined with [³H]-thymidine incorporation was reduced by the addition of sera from the first group, but not by the addition of sera from the third group. These results suggested that serum sEpoR may play an important role in signal transduction via EpoR on erythroid progenitor in CRF patients. Jpn J Nephrol 2002 ; 44 : 710-715.

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The administration of angiotensin II receptor antagonist (AIIA) to patients with advanced chronic renal failure(CRF) is not actively recommended. This study was performed to verify the appropriateness of this situation and to determine if there are any substantial differences between patients with a serum creatinine(SCr) level higher than 3 mg /dl and those with a lower SCr level in terms of the clinical effects such as renal function, serum potassium level and systemic blood pressure (BP) after the administration of AIIA. Sixteen patients with advanced CRF who were admitted to the outpatient clinic in Jikei University Hospital (1998/ 1-1999/12) were enrolled (average age : 65 years, underlying renal disease : diabetic nephropathy 6, CGN 5, and other l ) . They had never been administered AIIA before. The patients were classified into two groups in accordance with their level of SCr : group A (SCr lower than 3.0 mg/dl ; n=11), and Group B (SCr higher than 3.0 mg/dl ; n=5) . Losartan (50 mg day) administration was started in order to examine parameters such as the SCr, potassium, BP at the outpatient clinic, and urinary protein excretion at the 0, 1, 3, 6, 9, and 12 month time points. Although the 1 /SCr values provided negative slopes with time in both groups, no significant difference was found between the two slopes. There were no changes in the serum potassium levels or urinary protein excretion during the study period in either group, and no statistical difference was found between the two groups. Although the serum potassium level exceeded 5.5 mEq/l in two patients each in both groups, the level was controlled by diet therapy with restricted potassium. BP was reduced significantly in both groups during the study period, and no statistical difference in BP reduction was observed between the two groups. In conclusion, the results indicate there were no differences in the effect on renal function, serum potassium levels or systemic BP between the patients with a SCr level higher than 3.0 mg/dl and those with a lower level. The results also support the notion that patients with advanced renal dysfunction are not precluded from AIIA administration. Jpn J Nephrol 2002 ; 44 : 716-722.

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We investigated fluctuation of compliance with a low protein diet and the influence on the rate of decline in renal function. Twenty-seven patients with diabetic renal failure who were prescribed a low protein diet of 0.6 g/kg/day were followed during a period of 12 months. Dietary compliance was evaluated based on a 4-day dietary diary, interview with patients and calculation of the protein catabolic rate from 24-hour urea excretion at every hospital visit. They were judged on a 4-rank system, A (adhered over 75 % of the days), B (74±501), C (49±25 %), D (less than 24%). During the twelve months, 55.5% of the patients showed fluctuation of their compliance with the diet. In 17 patients whose compliance fluctuated between rank A and B, their rate of GFR decline (ml/min/ month) was significantly faster(-2.40±2.59 vs 0.99±1.41, p<0.01), their rate of serum creatinine elevation(mg/dl/month) was significantly higher(0.90±0.79 vs -0.42±0.45, p<0.01) and their rate of serum urea nitrogen increase (mg/dl/month) was significantly larger (15.3±12.4 vs -10.0±12.9, p<0.01) during the period of rank B than rank A. There were no significant differences in the rates of changes in serum levels of albumin, transferin, HbAic and body weight between the two periods. In conclusion, fluctuation of compliance with a low protein diet affects the rate of renal function decline synchronizedly in patients with diabetic renal failure.

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A50-year-old man diagnosed as having AGA(Churg-Strauss syndrome)was administered steroid. After treatment with mizoribine, hyperuricemia and acute renal failure occurred as side effects ofthis drug. Accordingly we started dialysis treatment, terminated mizoribine treatment, and administered allopurinol dosage. Hemodialysis was necessary every day for ll days and his renal function recovered after one month. In 67Ga scintigraphy, accumulation of 67Ga was seen in the kidney.

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We describe a 71-year-old man, who had been treated for hypertension, myocardial infarction and abdominal aortic aneurysm, and was admitted to our hospital because of proteinuria (3.9 g/day at the out patient clinic and 1.5 g/day at the time of admission) and edema in the extremities. Light microscopic study of the kidney biopsy specimen revealed mesangial proliferative glomerulonephritis and glomerular paralysis. Electron microscopic findings showed endothelial damage, including widening of the subendoth elial space and detachment of endothelial cells from the glomerular basement membrane. Deposition of immunoglobulins and complement was not detected by immunofluorescence studies. These pathological findings resemble the findings of thrombotic microangiopathy, but there were no clinical pictures of HUS/TTP. These findings suggest that hypertension, atherosclerosis and circulating turbulence caused by an aortic aneurysm induced severe glomerular endothelial damage leading to mesangial proliferative glomer ulonephritis without an immune response.

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Diabetic nephropathy is a complication of diabetes mellitus that is characterized by the appearance of diffuse and nodular glomerulosclerosis A 46-year-old man presented with generalized edema. He had severe nephrotic syndrome, renal insufficiency and hypertension without a family history or clinical evidence of diabetes mellitus. Oral glucose tolerance test showed impaired glucose tolerance, but several fasting plasma glucose determinations and serum hemoglobin A1C levels were normal. Renal biopsy revealed nodular and diffuse glomerulosclerosis characteristic of diabetic nephropathy. The present case demonstrates that nodular glomerulosclerosis may be present without clinically overt diabetes mellitus.

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A 66-year-old man with erysipelas was admitted with complaints of oliguria and massive proteinur ia/hematuria. He was diagnosed as having acute poststreptococcal glomerulonephritis (APSGN) due to erysipelas infected by group A streptococcus pyogenes. On admission, his white cell count increased to 31, 000, and CRP was 27.3 mg/dl. Serum urea nitrogen and creatinine were increased to 90.1 mg/dl and 4.5 mg/dl, respectively. He had diabetes mellitus (HbA1C 7.9 %) and liver dysfunction (total bilirubin 3.5 mg/dl, AST 76 IU, ALT 41 IU) caused by alcoholic liver cirrhosis. Hypocomplementemia was found in addition to ASO 216 U/ml and ASK 10, 240x. After antibiotics treatment was initiated, inflammation of the erysipelas began to improve. Disseminated intravascular coagulation syndrome, probably due to sepsis, occurred on the 5th hospital day. He died of gastrointestinal bleeding on the 18th hospital day. Renal autopsy revealed 37 % formation of fibrocellular crescents, and marked mesangiolysis was noted by light microscopy. Granular deposition of C3 and IgG was seen along the capillary walls on immunofluorescence study. Intramembranous deposits were scattered on electron microscopy. This case illustrates a fulminant type of APSGN, which was in part attributed to the presence of diabetes and alcoholic liver cirrhosis. Histological findings of crescent formation and marked mesangiolysis may account for the fulminant clinical course.

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