Single low-dose rituximab for the treatment of steroid-resistant nephrotic syndrome with acute kidney injury.

The efficacy of rituximab for kidney disease, such as frequent relapsing nephrotic syndrome, has been reported recently. Herein, we report a case of a patient with acute kidney injury that was steroid-resistant nephrotic syndrome who responded to a single administration of low-dose rituximab. An 86-year-old Japanese woman with hypertension presented with severe peripheral edema within several days after onset. Due to the patient's age, renal biopsy was not performed, nephrotic syndrome was diagnosed and prednisolone was administered at 40 mg/day on the day after admission. However, anuria developed and hemodialysis was inevitably initiated on the 5th hospital day. The renal function did not recover, and the general condition gradually became aggravated. On the 50th hospital day, 100 mg rituximab was administered, which led to immediate depletion of CD20-positive cells. The urine volume gradually increased from 2-3 weeks after the rituximab administration, and the renal function recovered slightly. After 5 weeks, it became possible to wean the patient from dialysis, which had been applied for 3 months. Rituximab might be an option for the treatment of acute kidney injury due to steroid-resistant nephrotic syndrome.

Impact of fibroblast growth factor 23 on lipids and atherosclerosis in hemodialysis patients.

Levels of fibroblast growth factor (FGF) 23, a phosphatonin, are frequently elevated in patients with end-stage renal disease (ESRD) who are on maintenance hemodialysis (MHD). However, the role of FGF23 remains unclear because renal FGF receptor function might be impaired. The present cross-sectional study examines a cohort of patients (n = 196) on MHD who were not undergoing therapy with lipid-lowering drugs including sevelamer. Non-fasting venous blood samples were withdrawn before the hemodialysis (HD) session on the third day after the previous HD session to measure serum levels of albumin, calcium (Ca), phosphate (P), alkaline phosphatase, intact parathyroid hormone (PTH), total cholesterol (C), high-density lipoprotein (HDL)-C, low-density lipoprotein(LDL)-C, oxidative LDL-C, high-sensitivity C-reactive protein (HsCRP), interleukin-6 (IL-6), and FGF23. Nutritional status was assessed using the geriatric nutritional risk index (GNRI). Carotid intima-medial thickness (CIMT) was assessed using a B-mode ultrasound scanner. FGF23 was positively correlated with P, Ca(alb)xP product, and intact PTH, and inversely correlated with C and non-HDL-C. In the higher FGF23 tertile, levels of both non-HDL-C and C were significantly decreased and CIMT was less elevated compared to the lower FGF23 tertile. Multivariate analysis showed that the higher FGF23 tertile was independently associated with decreases in C (adjusted r(2) = 0.14) and non-HDL-C (adjusted r(2) = 0.20) levels and with a less-pronounced increase in CIMT (adjusted r(2) = 0.14). High FGF23 appears to be an independent biomarker of a decrease in C and non-HDL-C that is negatively associated with atherosclerosis in patients on MHD.

[Case of microscopic polyangiitis presenting initially as prostatic vasculitis].

A 79-year-old man was admitted to our hospital to evaluate prostatic vasculitis that was diagnosed on a prostatic biopsy performed due to an elevated prostate-specific antigen (PSA)level. He complained of general malaise and had hematuria, proteinuria, and interstitial pneumonitis in the lower lung field on admission. A renal biopsy showed necrotizing crescentic glomerulonephritis. He was diagnosed as having microscopic polyangiitis (MPA) based on the presence of small-vessel vasculitis and positivity for myeloperoxidase-anti-neutrophil cytoplasmic antibody (MPO-ANCA) (465 EU). He was effectively managed with methylprednisolone pulse therapy (500 mg/day x 3 days) followed by progressive dosage tapering. In the present case, the identification of prostatic vasculitis led to the early detection of MPA, which resulted in an excellent clinical course. Prostatic involvement has been also reported in patients with Wegener's granulomatosis or Churg-Strauss syndrome. Thus, physicians should be aware of the potential for the involvement of various organs, including the prostate, in patients with ANCA-associated vasculitis.

Ultrapure dialysate influences serum myeloperoxidase levels and lipid metabolism.

BACKGROUND: Ultrapure dialysate (UD) might contribute to improvements in the morbidity and mortality of hemodialysis (HD) patients. However, it is unclear whether increasing dialysate purity affects chronic inflammation, oxidative stress, and lipid abnormalities. METHODS: In a prospective cohort study, 126 patients undergoing maintenance HD using conventional dialysate (CD) with one endotoxin cut filter were assigned to either continuation of the same HD or HD using UD (more purified dialysate). At baseline and 6 months we measured lipids, high-sensitive (hs)CRP, oxidative LDL-cholesterol, and myeloperoxidase. RESULTS: Serum myeloperoxidase and hsCRP levels in the UD group were significantly decreased at 6 months compared with the CD group. Multivariate analysis showed that decreases in non-HDL-cholesterol and ApoB at 6 months were independently correlated with changes in myeloperoxidase. CONCLUSION: Endotoxin-free UD can improve the chronic inflammatory status, oxidative stress, and lipid abnormalities, suggesting a possible contribution to reduced cardiovascular disease risk and ultimately to lowered mortality in HD patients.

MPO-ANCA crescentic glomerulonephritis complicated by membranous nephropathy: MPO demonstrated in epimembranous deposits.

An elderly woman presented with haematuria and proteinuria accompanied by elevated serum myeloperoxidase (MPO)-specific anti-neutrophil cytoplasmic antibodies (MPO-ANCA). A renal biopsy revealed mild mesangial proliferation with fibrocellular crescent formation and a membranous glomerular lesion. Immunofluorescence microscopy using FITC-labelled rabbit anti-human MPO antibodies revealed granular MPO deposition along the glomerular capillary walls (GCW) with a staining profile similar to that of glomerular IgG deposition. The one-year follow-up renal biopsy revealed minimal IgG and undetectable MPO deposition. Both MPO and MPO-ANCA might have been responsible for the IgG immune depositions along the GCW in this patient.

Lupus nephritis associated with positive MPO-ANCA in a patient with underlying autoimmune hemolytic anemia.

A 19-year-old female was admitted with general malaise and systemic edema. She had been diagnosed as having autoimmune hemolytic anemia (AIHA) eight years earlier and was successfully managed with oral prednisolone. During the current admission, she was diagnosed as having systemic lupus erythematosus (SLE) based on the presence of renal involvement, hematological abnormalities, and antinuclear and anti-double-stranded DNA antibodies, along with a recurrence of AIHA; her serology revealed a high myeloperoxydase-antineutrophil cytoplasmic antibody (MPO-ANCA) titer. She was treated with prednisolone (50 mg day(-1)), but her renal function started to deteriorate. She responded to treatment with hemodialysis, plasmapheresis, and methylprednisolone pulse therapy; her MPO-ANCA titer and renal function improved. Treatment with intravenous cyclophosphamide gradually suppressed her AIHA and SLE activity. A renal biopsy revealed a diffuse proliferative lupus nephritis (class IV-G (A)) with necrotizing crescentic glomerulonephritis that was presumed to be associated with MPO-ANCA. The association of MPO-ANCA with SLE in this refractory case is discussed.

Elderly onset systemic lupus erythematosus (SLE) presenting with disseminated intravascular coagulation (DIC).

We describe an unusual case of elderly onset systemic lupus erythematosus (SLE) that presented with disseminated intravascular coagulation (DIC). An 86-year-old man who complained of general malaise was admitted for evaluation and treatment of thrombocytopenia. He was diagnosed as having SLE and DIC based on the criteria of the American College of Rheumatology for SLE (renal involvement, hematological abnormalities, and positivity for antinuclear antibody and lupus anticoagulant) and the criteria for DIC presented by the subcommittee on DIC of the ISTH (a large increase of fibrin degradation products [3 points] and a platelet count <50 x 10(3)/ml [2 points], resulting in a score of 5; a score > or =5 is compatible with DIC). The patient was treated with corticosteroid therapy (30 mg/day); the DIC and SLE remitted, and his renal function improved, but he developed pulmonary tuberculosis. Timely diagnosis, appropriate treatment, and an awareness of the potential for serious infections are of utmost importance when dealing with patients with elderly onset SLE.