The Japanese Society for Apheresis clinical practice guideline for therapeutic apheresis.

Most of the diseases for which apheresis therapy is indicated are intractable and rare, and each patient has a different background and treatment course prior to apheresis therapy initiation. Therefore, it is difficult to conduct large-scale randomized controlled trials to secure high-quality evidence. Under such circumstances, the American Society for Apheresis (ASFA) issued its guidelines in 2007, which were repeatedly revised until the latest edition in 2019. The ASFA guidelines are comprehensive. However, in the United States, a centrifugal separation method is mainly used for apheresis, whereas the mainstream procedure in Japan is the membrane separation method. The target diseases and their backgrounds are different from those in Japan. Due to these differences, the direct adoption of the ASFA guidelines in Japanese practice creates various problems. One of the features of apheresis in Japan is the development of treatment methods using hollow-fiber devices such as double filtration plasmapheresis (DFPP) and selective plasma exchange and adsorption-type devices such as polymyxin B-immobilized endotoxin adsorption columns. Specialists in emergency medicine, hematology, collagen diseases/rheumatology, respiratory medicine, cardiovascular medicine, gastroenterology, neurology, nephrology, and dermatology who are familiar with apheresis therapy gathered for this guideline, which covers 86 diseases. In addition, since apheresis therapy involves not only physicians but also clinical engineers, nurses, dieticians, and many other medical professionals, this guideline was prepared in the form of a worksheet so that it can be easily understood at the bedside. Moreover, to the clinical purposes, this guideline is designed to summarize apheresis therapy in Japan and to disseminate and further develop Japanese apheresis technology to the world. As diagnostic and therapeutic techniques are constantly advancing, the guidelines need to be revised every few years. In order to ensure the high quality of apheresis therapy in Japan, both the Japanese Society for Apheresis Registry and the guidelines will be inseparable.

Development of aortic valve stenosis in myeloperoxidase antineutrophil cytoplasmic antibody-associated vasculitis with renal involvement.

INTRODUCTION: Degenerative aortic valve stenosis (AS) is a chronic progressive disease that resembles atherosclerosis development. Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is reportedly associated with accelerated atherosclerosis. This study aimed to examine the development of AS in patients with myeloperoxidase-AAV (MPO-AAV) with renal involvement at more than 1 year after the onset of vasculitis. METHODS: We performed a retrospective review of clinical records of MPO-AAV patients with renal involvement without AS at the onset of vasculitis who were treated in three hospitals and three dialysis clinics. RESULTS: The study included 97 MPO-AAV patients with renal involvement and 230 control patients with chronic kidney disease (CKD). Among them, 64 patients had AS. The prevalence rates of AS were 28.9% and 15.7% in MPO-AAV and control patients, respectively (p = 0.006). The multivariable logistic regression analysis showed that MPO-AAV, dialysis dependence, and hypertension were independently associated factors for AS. In MPO-AAV patients, systolic blood pressure was positively significantly associated with AS, whereas glucocorticoid dose of induction therapy was negatively significantly associated. The use of cyclophosphamide tended to be negatively associated with AS. The survival rate was significantly lower for patients with AS than for those without AS. CONCLUSIONS: The AS prevalence rate was significantly higher in MPO-AAV patients at more than 1 year after the onset of vasculitis than in control CKD patients. Therefore, regular monitoring of echocardiography during MPO-AAV treatment is suggested.

Thrombospondin type-1 domain-containing 7A-associated membranous nephropathy after resection of rectal cancer: a case report.

BACKGROUND: Thrombospondin type-1 domain-containing 7A (THSD7A) is a target antigen in idiopathic membranous nephropathy (MN). Patients with THSD7A-associated MN are known to have a high possibility of developing malignancy. However, there are few case reports on THSD7A-associated MN with malignancy, and details of its characteristics have not been clarified thoroughly. Here, we report the case of a 77-year-old male patient who was diagnosed with THSD7A-associated MN after resection of rectal cancer. CASE PRESENTATION: A 77-year-old man who had developed bilateral peripheral edema, persistent proteinuria, and nephrotic syndrome was admitted to our hospital. He was diagnosed with MN based on a renal biopsy 3 years after resection of rectal cancer, and positive staining for THSD7A in both kidney and rectal cancer tissues suggested that these two diseases were related. Furthermore, THSD7A staining of metastatic lymph nodes revealed deposition of THSD7A in the secondary lymph follicles and sinus. Recurrence of rectal cancer was suspected; however, tumor recurrence was not observed on chest and abdominal computed tomography (CT) and colonoscopy. There was no lymph node enlargement. The patient was kept on observation with supportive therapy. Consequently, although nephrotic syndrome persisted, obvious recurrence and metastasis of the primary tumor were not observed. CONCLUSION: This is the first case in which pathological examination results suggested that THSD7A-associated MN was caused by rectal cancer. Based on the reports of THSD7A-associated MN with malignancy and the pathogenesis of MN, lymph node metastasis may be a risk for cancer-related MN.

Open surgery for descending thoracic aorta in an endovascular era.

BACKGROUND: Thoracic endovascular aortic repair has become the preferred treatment for a variety of descending thoracic aortic pathologies. However, there are unresolved issues such as morphologic appearance of chronic dissection, persistent false lumen perfusion, and adequacy of landing zone. Enthusiasm for improving the technique of open aortic repair and perioperative management is fading. In this study, we would like to demonstrate how we improve our surgical outcomes by establishing a dedicated aortic multidisciplinary team at the Kawasaki Aortic Center. METHOD: We performed a single-center retrospective study from January 2015 to December 2016. All patients with open descending thoracic aortic replacement were recruited. Preoperative patient demographic data, bypass strategies, operative details, and postoperative outcomes were reviewed. RESULT: From January 2015 to December 2016, we treated 168 cases of descending thoracic aortic repair using a left thoracotomy. Median age was 69.0 ± 21.8 years old, and 63.1% were aortic dissection (acute, 4.8%; chronic, 58.3%); 81.3% patients underwent elective operations. Left heart bypass, deep hypothermic circulatory arrest, and partial cardiopulmonary bypass were performed in 88.6%, 9.0%, and 2.4% of patients, respectively. Mean operative time was 312 ± 94 minutes. In-hospital mortality in total was 0.6%. The rate of transient spinal cord injury was 4.7%. CONCLUSIONS: Under a dedicated aortic multidisciplinary team, we demonstrated that open descending thoracic aorta replacement can be performed with excellent early outcomes with low reintervention rates, regardless of the nature of the aortic pathologies.

A retrospective study on the outcomes of MPO-ANCA-associated vasculitis in dialysis-dependent patients.

OBJECTIVES: This study investigated the clinical course of myeloperoxidase-antineutrophil cytoplasm autoantibody (MPO-ANCA)-associated vasculitis after starting dialysis. METHODS: A retrospective review was conducted of the clinical charts of dialysis-dependent patients with MPO-ANCA-associated vasculitis who attended one of 8 associated clinics over the past 21 years. RESULTS: Eighty-nine patients were included in the study; 88 had microscopic polyangiitis (MPA) and 1 had granulomatosis with polyangiitis. Of the 88 patients with MPA, 18 had renal-limited vasculitis. Twenty-one relapses occurred among 13 patients (frequency, 0.05 relapses/person-year; 95% confidence interval, 0.03-0.08). Mean time from start of dialysis to relapse was 65 ± 59 months. Cox multivariate analysis showed that pulmonary involvement was a predictor of relapse (hazard ratio [HR], 21.4) and mortality (HR, 4.60), and that patient age (HR, 1.10) and cyclophosphamide use (HR, 0.20) were significant predictors of mortality. Postdialysis 1- and 5-year survival rates were 83.0% and 65.6%, respectively; infection was the most frequent cause of death. CONCLUSION: Pulmonary involvement was a predictor of relapse and mortality. Although relapse can occur long after the start of dialysis, incidence was low among dialysis-dependent patients. Prolonged maintenance immunosuppressive therapy might be limited to patients with pulmonary involvement in dialysis-dependent ANCA-associated vasculitis.

Practical approach to evaluate asymptomatic coronary artery disease in end-stage renal disease patients at the initiation of dialysis.

The high prevalence of significant asymptomatic coronary artery disease (CAD) has been reported in patients with end-stage renal disease (ESRD) at the initiation of dialysis. However, the approach to evaluate asymptomatic CAD for these patients has not been established. The aim of this study is to assess the applicability of our practical approach at the initiation of dialysis. We prospectively enrolled 182 consecutive ESRD patients who initiated dialysis. After echocardiography as primary screening, pharmacologic stress thallium-201 scintigraphy and/or coronary angiography (CAG) were performed to diagnose CAD. The patients were classified into two groups: those with coronary artery stenosis by CAG (CAD+ group), those without coronary artery stenosis by CAG or with negative scintigraphy examination (CAD- group). Of the eligible 93 patients without the history of CAD, 22 patients were allocated to the CAD+ group (18 of 26 patients with abnormal echocardiography and 4 of 13 patients with positive scintigraphy examination) and 71 patients to the CAD- group. Patients were followed up for an average of 520 ± 304 days. The event-free survival rate of major adverse cardiac events was significantly lower in the CAD+ group than in the CAD- group (P < 0.001). There was no cardiovascular event including major adverse cardiac events, unstable angina, coronary revascularization or stroke in the CAD- group during the first year of dialysis. Patients without CAD diagnosed by our approach had favorable clinical outcomes. Our approach may be useful for screening of occult CAD in ESRD patients at the initiation of dialysis.

Serum levels of 5-s-cysteinyldopa are correlated with skin colors in hemodialysis patients but not in peritoneal dialysis patients.

BACKGROUND: Diffuse hyperpigmentation is common in patients with chronic renal failure undergoing hemodialysis (HD) or peritoneal dialysis (PD). We previously reported that serum levels of 5-S-cysteinyldopa (5SCD, a pheomelanin precursor) and pheomelanin were significantly elevated in HD patients. METHODS: Skin color was assessed using a Mexameter that measures the melanin index (MI) and the erythema index (EI). The upper inner arms (non-sun-exposed site) and the foreheads (sun-exposed site) of HD and PD patients and control subjects were analyzed. RESULTS: MI values on the upper inner arms and on the foreheads of HD and PD patients were significantly higher than in controls. In HD patients, significant correlations were found for serum 5SCD levels with MI and EI on the upper inner arm, and for EI on the forehead. In PD patients, no such correlations were found. CONCLUSIONS: Hyperpigmentation in HD patients results partly from accumulation of pheomelanin in the skin.