Successful use of avacopan in a case of ANCA-associated vasculitis with treatment-resistant medium-sized vessel involvement.

We report the case of a 75-year-old woman who presented with fever, right back pain, paresthesia in the right extremities, erythema, purpura, and nodules. She had previously initiated dialysis due to rapidly progressive glomerulonephritis and was transferred to our hospital. Imaging studies revealed multiple cerebral and splenic infarcts and hemorrhage encapsulating the right kidney, likely due to microaneurysms in multiple renal arteries.

Anti-glomerular basement membrane disease developing 3 years after the development of Sweet syndrome and 1 year after the development of anti-neutrophil cytoplasmic antibody-associated vasculitis: a case report.

A 73-year-old Japanese woman, with a history of Sweet syndrome diagnosed 3 years earlier and anti-myeloperoxidase (MPO) antibody anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis diagnosed 1 year earlier, presented with an episode of rapidly progressive glomerulonephritis (RPGN) with anti-glomerular basement membrane (GBM) disease. At the time of diagnosis of the ANCA-associated vasculitis 1 year earlier, serological testing yielded a negative result for anti-GBM antibody. However, at the present visit, serology for anti-MPO antibody was negative, while that for anti-GBM antibody was positive.

The efficacy and safety of mizoribine for maintenance therapy in patients with myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA)-associated vasculitis: the usefulness of serum mizoribine monitoring.

Background: The life prognosis of elderly patients with myeloperoxidase-anti-neutrophil cytoplasmic antibodies-associated vasculitis (MPO-AAV) has been improved by reducing the corticosteroid or cyclophosphamide dose to avoid opportunistic infection. However, many elderly MPO-AAV patients experience recurrence and renal death. An effective and safer maintenance treatment method is necessary to improve the renal prognosis of MPO-AAV.

Identification of histopathological and clinical spectrum of diabetic kidney disease based on an unsupervised hierarchical clustering analysis of elderly autopsy specimens.

Aim: Diabetic kidney disease (DKD), a chronic kidney disease caused by diabetes and other comorbidities, is the leading cause of end-stage renal disease. The pathogenesis of DKD is diverse and influenced by various causes, some but not all of which cause proteinuria. Some factors such as hypertension can modify DKD.

Autopsy study examining non-chronic kidney disease versus chronic kidney disease caused by hypertensive-nephrosclerosis in elderly subjects.

Background: The aim of this autopsy study was to clarify the differences of renal histopathology between non-chronic kidney disease (CKD) and CKD caused by hypertensive-nephrosclerosis in the elderly and during the aging process.

Methods: We examined autopsy specimens from 105 elderly patients (53 male subjects; mean age, 86.2 years) including 44 patients with CKD as a result of nephrosclerosis.

Fabry Nephropathy in a Young Female Patient Presenting with Only Urinary Mulberry Bodies Treated with Chaperone Therapy.

Fabry disease (FD) is an X-linked disorder of the sphingolipid metabolism, caused by deficiency or decreased activity of α-galactosidase A. We report a rare case of Fabry nephropathy (FN) in a 21-year-old Japanese female patient presenting with only urinary mulberry bodies; she was treated with pharmacological chaperone therapy (PCT) after renal biopsy. The patient underwent a detailed examination because her mother was diagnosed with FD in the Division of Community Medicine of our hospital.

Reduced Plasma Ascorbate and Increased Proportion of Dehydroascorbic Acid Levels in Patients Undergoing Hemodialysis.

Ascorbate functions as an electron donor and scavenges free radicals. Dehydroascorbic acid (DHA), the oxidized form of ascorbate, is generated as a result of these reactions. While low plasma ascorbate levels have been reported in hemodialysis patients worldwide, no studies have measured DHA because it is not generalized.

Toward the development of a vibrant, super-aged society: The future of medicine and society in Japan.

Background: As Japan's population continues to age, it is estimated that the number of people aged ≥75 years will exceed 20 million by 2025. Furthermore, over the past 10 years, we have not reduced the difference between life expectancy and healthy life expectancy. Therefore, the extension of healthy life expectancy and the development of a healthy society are the most urgent issues.

Efficacy and safety of low-dose corticosteroid with azathioprine in the treatment of elderly-onset IgA vasculitis with nephritis.

The diagnosis of elderly-onset IgA vasculitis (IgAV) and its prognosis can be difficult to ascertain because of its rarity and the frequent presence of comorbidities. Furthermore, the treatment of elderly-onset IgAV remains controversial. We report a case of IgAV in an 87-year-old patient.

Intraosseous epidermal cyst of the great toe that was difficult to distinguish from chronic osteomyelitis: A case report and literature review.

Obtaining a correct intraosseous epidermal cyst diagnosis is difficult due to the extreme rarity of this cyst. Further, the clinical manifestations and radiographic findings are very similar to those of a malignancy or infection. Early histopathological analysis is required for accurate diagnosis and for avoiding unnecessary antibiotic administration and amputation.

A case of light chain (AL) amyloidosis with heart failure, renal dysfunction, and heparin-induced thrombocytopenia successfully treated with peritoneal dialysis.

A 65-year-old woman was hospitalized for heart failure and pneumonia in a nearby hospital. She had been previously diagnosed as light chain (AL) amyloidosis and treated with melphalan plus dexamethasone (Mel-Dex), and lenalidomide plus dexamethasone (Len-Dex). She started treatment including antimicrobials and diuretics, but her renal function worsened progressively, and she was transferred to our hospital for nephrological care.

Low-dose corticosteroid with mizoribine might be an effective therapy for elderly-onset ISKDC grade VI IgA vasculitis.

Both the diagnosis of elderly-onset IgA vasculitis (IgAV) and its prognosis can be difficult because of its rarity and the likely presence of comorbidities. Furthermore, the treatment of elderly-onset IgAV remains controversial: the ideal dosages of corticosteroid and/or immunosuppressants have not been determined. In the elderly, corticosteroid adverse effects can lead to severe outcomes, and a consensus regarding its benefit and risk balance has not been reached.

Geriatric assessment of estimated glomerular filtration rate: a cross-sectional study.

Background: Estimated glomerular filtration rate (eGFR) is routinely calculated based on the serum creatinine level. However, the validity of such calculation in the geriatric population has not been sufficiently assessed. To examine whether the discrepancies between the eGFR determined based on the serum creatinine (eGFRcr) and that based on the serum cystatin C (eGFRcys) may be influenced to a lesser degree, by factors such as aging and muscle mass.

Citrullinated histone H3 expression in anti-neutrophil cytoplasmic antibody-associated vasculitis in older Japanese autopsy patients.

Aim: Neutrophil extracellular traps play key roles in the necrotizing vasculitis associated with anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). However, the relationships between neutrophil extracellular traps formation and the distribution and phase of vasculitis are not well understood. In the present study, we clarified the clinicopathological characteristics of older AAV patients, as well as the expression of citrullinated histone H3 (citH3), a marker of neutrophil extracellular traps, in autopsied AAV patients.

[Egocentric disorientation and heading disorientation: evaluation by a new test named card placing test].

We recently developed a new clinical test named card placing test (CPT) which can assess a subject's ability to deal with visuospatial information. The CPT requires a subject to recreate an array of three cards, each of which was randomly placed on eight grids around the subject, before (CPT-A) and after the subject's rotation (CPT-B). With this design, the CPT can assess a subject's ability to represent visuospatial information either egocentrically (CPT-A) or allocentrically (CPT-B).

Japan as the front-runner of super-aged societies: Perspectives from medicine and medical care in Japan.

Background: The demographic structure of a country changes dramatically with increasing trends toward general population aging and declining birth rates. In Japan, the percentage of the elderly population (aged ≥65 years) reached 25% in 2013; it is expected to exceed 30% in 2025 and reach 39.9% in 2060.

Marked improvement of Churg-Strauss syndrome neuropathy by intravenous immunoglobulin and cyclophosphamide.

A 42-year-old Japanese man developed Churg-Strauss syndrome 7 years after being diagnosed with chronic eosinophilic pneumonia. Prominent eosinophilia, subcutaneous nodules, and neuropathy in the left leg were seen. A pathological diagnosis of necrotizing vasculitis was determined by a biopsy of a subcutaneous nodule.

Assessment of the Birmingham vasculitis activity score in patients with MPO-ANCA-associated vasculitis: sub-analysis from a study by the Japanese Study Group for MPO-ANCA-associated vasculitis.

Objectives: In the study cohort enrolled in a prospective open-label, multicenter trial conducted by the Japanese Study Group for MPO-ANCA-associated vasculitis (JMAAV), we conducted this sub-analysis to establish the validity of the Birminghan vasculitis activity score (BVAS) for Japanese patients with MPO-ANCA-associated vasculitis.

Methods: We recorded the BVAS at the time of diagnosis, at 6 weeks after the diagnosis, and at 3, 6, 9, 12, 15 and 18 months after the diagnosis in this study.

Results: The most frequently involved organs in the patients were the lungs, kidneys and the nervous system.

A novel autoantibody against moesin in the serum of patients with MPO-ANCA-associated vasculitis.

Background: Antineutrophil cytoplasmic autoantibody (ANCA) directed against myeloperoxidase (MPO), a diagnostic criterion in MPO-ANCA-associated vasculitis (MPO-AAV), does not always correlate with disease activity. Here, we detected autoantibodies against moesin, which was located on the surface of stimulated endothelial cells, in the serum of patients.

Methods: The anti-moesin autoantibody titer was evaluated by ELISA.

Correlation of interleukin-6 and monocyte chemotactic protein-1 concentrations with crescent formation and myeloperoxidase-specific anti-neutrophil cytoplasmic antibody titer in SCG/Kj mice by treatment with anti-interleukin-6 receptor antibody or mizoribine.

Myeloperoxidase-specific anti-neutrophil cytoplasmic antibody (MPO-ANCA) is associated with rapidly progressive glomerulonephritis (RPGN) and glomerular crescent formation. Pathogenic factors in RPGN were analyzed by using SCG/Kj mice, which spontaneously develop MPO-ANCA-associated RPGN. The serum concentration of soluble IL-6R was determined by using ELISA and those of another 23 cytokines and chemokines by Bio-Plex analysis.