The impact of C4d-negative acute antibody-mediated rejection on short-term prognosis among kidney transplant recipients.

AIM: We aimed to investigate the clinical and pathological features of C4d-negative acute antibody-mediated rejection (aAMR), and examined the impact of C4d-negative aAMR on short-term prognosis. METHODS: From 2005 to 2011, 626 kidney transplantations were performed in our institution and related hospitals. We excluded 174 ABO-incompatible transplantations, and analysed clinical and pathological data from the remaining 452 until December 2013. RESULTS: During the follow-up period, 39 patients underwent aAMR. We divided them into two groups. According to C4d positivity in each patient's first AMR, we divided the cohort into a C4d-positive aAMR group and a C4d-negative aAMR group, using the new Banff 2013 classification. We compared each aAMR patient's features to controls. Clinical and pathological characteristics were similar in both groups and the short-term outcomes of the two groups were similar, but both were worse than control. CONCLUSION: C4d-negative aAMR resembles C4d-positive aAMR in terms of clinical and pathological features, and that C4d positivity has no influence on short-term outcome.

Impaired laryngeal voice production in a patient with foreign accent syndrome.

We report a Japanese-speaking monolingual woman who developed foreign accent syndrome (FAS) following an infarction in the precentral and premotor cortices (Brodmann Area 6) at and around the inferior frontal sulcus. Her speech sounded Chinese or Korean to our bilingual coauthor who speaks Chinese and Japanese. Quantitative acoustic analyses of words and sentences showed that pitch (fundamental frequency variation) and intensity variances appeared lowered and fully voiced glottal pulses were reduced. These findings suggest laryngeal dysfunction that contributes to the unusual speech production in a case of FAS. This may be caused by damage to a restricted area of the motor and premotor cortices that controls laryngeal function.

AP-VAS 2012 case report: two patients with rheumatoid arthritis suspected of relapsed microscopic polyangiitis after initiation of dialysis.

We report two patients with rheumatoid arthritis (RA) who were suspected of microscopic polyangiitis during maintenance dialysis. Case 1 was a 52-year-old woman with RA diagnosed at the age of 38 years and treated successfully with gold compounds. At the age of 43 years, she presented with progressive renal dysfunction and abnormal urine sediments, and a renal biopsy revealed crescentic nephritis with advanced glomerular sclerosis. Myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) was not measured on that occasion. She reached end-stage renal failure within 4 months and started peritoneal dialysis. Eight years later, soon after she was switched to hemodialysis, she developed fever of unknown origin. MPO-ANCA was elevated to 37 EU, although there were no other signs or symptoms suggestive of vasculitis. After taking prednisolone orally (10 mg/day), her fever withdrew, and MPO-ANCA became undetectable. Case 2 was a 71-year-old woman with RA diagnosed at the age of 60 years and treated with gold compounds. She developed renal failure of unknown cause (no biopsy was performed), and started hemodialysis at the age of 69 years. One year later, she presented with fever and subsequently developed cough with hemoptysis. MPO-ANCA was elevated to 62 EU. Treatment with azathioprine 50 mg and prednisolone 35 mg daily brought remarkable clinical improvement, and MPO-ANCA became undetectable. These cases highlight the importance of measuring ANCA even in RA patients on dialysis who present with fever of unknown origin or with underlying kidney disease of uncertain etiology.

BK polyomavirus nephropathy complicated with acute T-cell-mediated rejection in a kidney transplant recipient: a case report.

We present a case of severe BK polyomavirus nephropathy (BKVN) complicated with persistent acute T-cell-mediated rejection (ATMR) that progressed to allograft failure. A 54-yr-old man received a living donor kidney transplant from his wife. Approximately four months after transplantation, the patient's serum creatinine (SCr) increased from a baseline value of 1.5-2.4 mg/dL. A histological analysis showed BKVN, and the SV40 antigen was detected in the tubular nuclei. The doses of immunosuppressants were reduced, and immunoglobulin was administered intravenously. The SCr increased further, to 5.3 mg/dL, and a second renal biopsy revealed the presence of severe ATMR. Antirejection treatment was performed, and low-dose cidofovir was started. The SCr decreased, to 3 mg/dL, and BK virus antigen in the serum and urine samples became negative at the time of hospital discharge. However, the histological findings subsequently showed gradually progressive interstitial fibrosis and tubular atrophy, and the SCr increased gradually. Two years after the transplantation, the patient resumed hemodialysis. BK polyomavirus nephropathy is usually treated with a reduction in immunosuppressant therapy, although in some patients, the reduction in immunosuppressants induces a subsequent exacerbation of acute rejection and results in progressive graft failure, which suggests difficulty in treating BKVN after kidney transplantation.

Arteriovenous access closure in hemodialysis patients with refractory heart failure: a single center experience.

Arteriovenous dialysis access may impose a burden on the cardiac system. The objective of this study is to examine the usefulness of access closure in hemodialysis patients with refractory heart failure and to identify possible factors associated with symptomatic improvements. The study population comprised 33 hemodialysis patients with symptomatic heart failure (New York Heart Association [NYHA] class ≥ II), who underwent arteriovenous access closure (30 fistulas and three grafts) between 1991 and 2008. In all patients, heart failure was refractory to all possible medical and surgical treatments, and persisted after optimal dry weight control. First, short-term changes in hemodynamics, clinical symptoms and echocardiographic morphology were examined. Second, clinical and echocardiographic parameters were compared between responders (N=23), who demonstrated NYHA class improvement after access closure, and non-responders (N=10). After access closure, systolic blood pressure rose and the heart rate decreased significantly. Body weight and echocardiographic parameters did not change significantly. Twenty-three patients (70%) demonstrated NYHA class improvement and were designated as responders. In responders, the duration from access creation to closure was significantly shorter and fewer had ischemic heart disease, compared with non-responders. Access flow, cardiac output and ejection fraction were comparable between the two groups. Although the five-year survival was 20.2% in all patients, responders showed better early survival than non-responders. Arteriovenous access closure improved clinical symptoms in 70% of patients with refractory heart failure. This improvement was especially likely to be achieved in patients without ischemic heart disease and those who developed heart failure within a relatively short time after access creation.

Myeloperoxidase-antineutrophil cytoplasmic antibody-associated crescentic glomerulonephritis with rheumatoid arthritis: a comparison of patients without rheumatoid arthritis.

BACKGROUND: Several cases of rheumatoid arthritis (RA) with myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA)-associated crescentic glomerulonephritis (CrGN) have been reported. However, its clinical characteristics are not clear. METHODS: We summarized 3 patients of concurrent RA and MPO-ANCA-associated CrGN, diagnosed in our hospital from 1992 to 2006, and compared their clinicopathological data with those of 10 MPO-ANCA-associated CrGN patients without RA in the same period. RESULTS: All three RA patients were middle-aged or young adult women with 7-14 years of RA history. The initial clinical symptom was microhematuria, and mean duration from hematuria onset to histological confirmation of CrGN was 17 months. At renal biopsy, serum creatinine concentration (sCr) was modestly elevated, with the mean value of 3.4 mg/dl. Crescents were detected in 30% of glomeruli, whereas advanced glomerular sclerosis, tubular atrophy, and interstitial fibrosis were also observed. In comparison with patients without RA, patients with RA were significantly younger and showed a longer duration from the onset to histological confirmation of CrGN. Serum creatinine concentration at referral was significantly lower; however, estimated glomerular filtration rate (eGFR) was comparable. The Birmingham Vasculitis Activity Score and the Disease Extent Index were significantly lower, and pathological examination showed less crescent formation and a tendency to advanced glomerular sclerosis in patients with RA. CONCLUSIONS: In patients with RA, MPO-ANCA-associated CrGN appeared to develop at younger ages and often showed a slowly progressive deterioration of the renal function with slight extrarenal manifestations. These smoldering clinical features may result in late referral from rheumatologists to nephrologists and therefore poor prognosis.