There have been numerous studies on elder-onset systemic lupus erythematosus, but few on elderonset lupus nephritis. Many studies have shown that the severity of systemic lupus erythematosus declines with the advance of age. We retrospectively studied the clinical characteristics and prognosis of a Japanese lupus nephritis population to review the behavior of 12 elder-onset patients whose onset of disease, defined as the initial manifestation of systemic lupus erythematosus, occurred after the age of 50 years. Data on the clinical features and laboratory findings of 37 patients with lupus nephritis were collected. Elder-onset patients tended to have a decreased incidence of class V histology and an increased incidence of class II histology compared with younger-onset patients. The incidences of nephrotic syndrome, renal failure and class IV histology as well as the requirements of immunosuppressive therapies were similar in the two groups. In the intensive therapy (IV methylprednisolone, plasmapheresis and their combination) group, elder-onset patients had a higher mortality rate. In this study, elder-onset lupus nephritis patients did not belong to a benign subgroup of the lupus nephritis population, and it was found that intensive therapy of elder-onset patients potentially increased the risk of death.

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Tight blood pressure control over a long period is important to prevent end-organ damage to the brain, heart, and kidneys, and to avoid the complications of hypertension. Control requires an accurate evaluation of treatment through an appropriate monitoring of home blood pressure.
In this study, we evaluated a method of home blood pressure monitoring, in which we propose a home blood pressure distribution diagram to evaluate the effectiveness of anti-hypertensive therapy. 1) In home blood pressure measurements, the first reading was high, while the second and third readings were essentially similar and thus stable. 2) Home blood pressure showed great daily variations, thus necessitating the use of the mean blood pressure over a fixed period, and not the individual blood pressure readings. The mean (morning, mid-day, and evening) over a one-week period was unstable, while the readings were stable over a three-week period. 3) The diagrams showing the distribution of home blood pressure measurements obtained in the morning and at night over a long period allowed the degree of early morning hypertension and the effectiveness of long-term blood pressure control to be assessed, and thus were useful for selecting anti-hypertensive agents. 4) In elderly patients, a mid-day systolic pressure of 100mmHg or less (particularly 90mmHg or less) resulted in the onset of symptoms of excessive hypotensive effects, such as lightheadedness and fainting, which affected ADL and QOL. 5) Because of great daily variations, it is difficult to achieve the blood pressure target of 130/80mmHg or less in patients with hypertension associated with diabetes mellitus or renal insufficiency. 6) The home blood pressure distribution diagram facilitated an understanding of the status of anti-hypertensive therapy by patients, and was useful for motivating patients to continue treatment.

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Background: Long-term therapy of mizoribine (MZR 2-5mg/kg/day) has been reported in the management of children with frequently relapsing nephrotic syndrome (FRNS). It had minimum adverse effects, however, MZR therapy does not sufficiently suppress the relapse of FRNS. Previous reports suggested that modification of MZR therapy with a total dosage and administration schedule may improve the therapeutic effect. To elucidate the issue, we retrospectively evaluated the efficacy and safety of high-dose MZR therapy for children with FRNS.
Methods: The subjects comprised 13 affected children with FRNS (9 boys and 4 girls: median age of 11.7 years, ranging from 7.8 to 20.1 years). They were divided into a high-dose group (MZR 7-10mg/kg/day; Max 400mg) and a low-dose group (MZR 4-6mg/kg/day). We compared the therapeutic benefits between both groups, including the incidence of relapse (times/year) and daily dosages of prednisolone (PSL, mg/kg/day). The Wilcoxon test was used for statistical analysis. We also evaluated the relationship between the therapeutic effects and serum concentration of MZR two hours after the administration.
Results: The low-dose and high-dose groups were well matched in terms of baseline characteristics. After the initiation of MZR, beneficial therapeutic effects ensued in the high-dose group (incidence of relapse: 3.61 vs. 1.59 times/year before and after the therapy, p<0.05), daily dosages of PSL (0.65 vs. 0.29mg/kg/day before and after therapy, p<0.001), but did not occur in the low-dose group (3.97 vs. 2.84times/year; 0.84 vs. 0.53mg/kg/day, n.s.). All patients with a serum MZR concentration of over 3μg/ml had relapses less than three times a year. One patient in the high-dose group and the other in the low-dose group showed hyperuricemia, and responded well to medical treatment. No other adverse effect was observed.
Conclusions: High-dose MZR therapy in the management for FRNS may provide more beneficial effects without significant adverse effects.

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Case 1: An 82-year-old female had a drop-foot, livedo reticularis and paresthesia. MPO-ANCA was positive. Because of developing renal dysfunction, she underwent steroid pulse therapy and dialysis, but died from the complication of congestive heart failure. Autopsy revealed necrotizing arteritis in the interlobular arteries and the arcuate arteries of the kidneys. Crescent formation was not found in the glomeruli.
Case 2: A 49-year-old male had a drop foot and numbness. MPO-ANCA was positive. Because of developing renal failure, he underwent dialysis and steroid pulse therapy. MPO-ANCA became negative. Nevertheless, he developed perforation of multiple ulcers in the small intestine and died. Pathohistology revealed arteritis in medium-size arteries at the branch level of the mesenteric arteries.
Since the presented two cases showed rapidly and irreversibly aggravated renal function with positive MPO-ANCA, MPA was suspected. However, pathological findings from the autopsy and operation and not the kidney biopsy supported the diagnosis of polyarteritis nodosa (PN). Vasculitis in the arterioles was absent. MPO-ANCA is not specific, and it can be detected in not only MPA, but also PN. The clinical course and pathological findings must be considered to differentiate PN from MPA.

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A 49-year-old woman with a history of chronic hepatitis C virus infection and Hashimoto disease was admitted to our hospital because of proteinuria, hematuria, purpura, and edema in the lower extremities. Laboratory data on admission revealed proteinuria (0.2g/day), microscopic hematuria (3+) with RBC casts, renal dysfunction (serum creatinine 1.4mg/dl), positive anti-nuclear antigen (×640, speckled type), hypocoplementemia, mixed cryoglobulinemia (type III), and hepatitis C virus infection (AST 45IU/l, ALT 33IU/l). MPO-ANCA level was found to be high (356 EU). In renal biopsy, most glomeruli showed crescentic formation with the weak deposition of IgG, IgM, and C3 in the mesangial area and along the capillary wall. She was diagnosed as having systemic vasculitis associated with MPO-ANCA. Methylprednisolone pulse therapy followed by oral prednisolone (40mg/day) effectively normalized MPO-ANCA level.
It has been reported that ANCA is found in patients with HCV-associated mixed cryoglobulinemia. Therefore, in chronic hepatitis C patients with systemic vasculitis, we should consider the possibility of ANCA-related microscopic polyangiitis and make a correct diagnosis by renal biopsy.

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