Anti-neutrophil cytoplasmic antibody-associated vasculitis is an uncommon inflammatory disease of small to medium-sized vessels that frequently presents with rapidly progressive glomerulonephritis and renal failure though it can affect any organ system. If untreated, the vast majority of patients will die within a year. Current treatments improve prognosis but affected patients remain at a substantially higher risk of death and adverse outcomes. We review the classification of the disease, our understanding of the pathogenesis and epidemiology, and propose future directions for research. We also evaluate the evidence supporting established treatment regimens and the progress of clinical trials for newer treatments to inform the design of future studies.

Atherosclerotic changes in carotid arteries of hemodialysis (HD) patients reflect global atherosclerotic changes in vasculature. Carotid intima-media thickness (CIMT) can be used for atherosclerosis prediction and assessment of cardiovascular risks in HD patients, and thus screening high-risk patients. In this cross-sectional study, CIMT was measured using ultrasonography (B-mode with 5-10-MHz multifrequency linear probe) in HD patients in our hospitals. Additionally, we assessed the relationship between their CIMT and some cardiovascular risk factors. A total of 62 HD patients (64.5% male) were included. Age, body mass index, low-density lipoprotein, fasting blood sugar, history of diabetes mellitus and cardiovascular disease, serum albumin, and duration and adequacy of HD in study patients had significant association with their CIMT. There were no significant relationships between CIMT and patient's gender, smoking, serum calcium, phosphate, calcium x phosphate product, hemoglobin, and uric acid level. More diagnostic modalities must be performed for detecting the impact of atherosclerosis on HD patients with high CIMT.

Vesicoureteral reflux (VUR) and its sequelae may lead to reduced renal perfusion and loss of renal function. Methods to describe and monitor tissue perfusion are needed. We investigated dynamic tissue perfusion measurement (DTPM) with the PixelFlux-software to measure microvascular changes in the renal cortex in 35 children with VUR and 28 healthy children. DTPM of defined horizontal slices of the renal cortex was carried out. A kidney was assigned to the "low grade reflux"-group if the reflux grade of the voiding cystourethrogram was 1 to 3 and to the "high grade reflux"-group if the reflux grade was 4 to 5. Kidneys with VUR showed a significantly reduced cortical perfusion. Compared to healthy kidneys, this decline reached in low and high grade refluxes within the proximal 50% of the cortex: 3% and 12 %, in the distal 50% of the cortex: 21% and 44 % and in the most distal 20 % of the cortex 41% and 44%. DTPM reveals a perfusion loss in kidneys depending on the degree of VUR, which is most pronounced in the peripheral cortex. Thus, DTPM offers the tool to evaluate microvascular perfusion, to help planning treatment decisions in children with VUR.

The long-term prognosis of patients with IgA nephropathy (IgAN) who present with preserved renal function and minimal proteinuria is not well described. We investigated the long-term outcomes of IgAN patients with an apparently benign presentation and evaluated prognostic factors for renal survival and clinical remission. We studied Japanese patients with biopsy-proven IgAN who had an estimated glomerular filtration rate (eGFR) ≥60 mL/min/1.73 m ² and proteinuria <0.5 g/day at the time of renal biopsy. The renal biopsies were reviewed using the Oxford classification. Twenty patients met the inclusion criteria. At diagnosis, the median eGFR (interquartile range) was 76.8 (65.2-91.1) mL/min/1.73 m ² , and the median proteinuria level was 0.31 (0.16-0.39) g/day. Only one patient had an increase in serum creatinine of over 50% and no patient progressed to end-stage renal disease. The 15-year renal survival rate was 93.8%. Clinical remission was observed in 9 (45%) patients. Baseline proteinuria was the only factor significantly associated with the absence of clinical remission. The long-term prognosis of Japanese patients with IgAN who presents with minor urinary abnormalities and preserved renal function is excellent.

ABO incompatibility has been considered as an important immunological barrier for renal transplantation. With the advent of effective preconditioning protocols, it is now possible to do renal transplants across ABO barrier. We hereby present a single center retrospective analysis of all consecutive ABOi renal transplants performed from November 2011 to August 2014. Preconditioning protocol consisted of rituximab, plasmapheresis and intravenous immunoglobulin (IVIG) and maintenance immunosuppression consisted of tacrolimus, mycophenolate sodium, and prednisolone. The outcome of these ABOi transplants was compared with all other consecutive ABO-compatible (ABOc) renal transplants performed during same time. Twenty ABOi renal transplants were performed during the study period. Anti-blood group antibody titer varied from 1:2 to 1:512. Patient and graft survival was comparable between ABOi and ABOc groups. Biopsy proven acute rejection rate was 15% in ABOi group, which was similar to ABOc group (16.29%). There were no antibody-mediated rejections in ABOi group. The infection rate was also comparable. We conclude that the short-term outcome of ABOi and ABOc transplants is comparable. ABOi transplants should be promoted in developing countries to expand the donor pool.

Urine neutrophil gelatinase-associated lipocalin (uNGAL) is a reliable early biomarker of acute kidney injury (AKI) in a homogeneous patient population. However, its utility in a heterogeneous population of critically ill, in whom the time of onset of renal insult is often unclear, is not clearly established. We evaluated the ability of a single measurement of uNGAL in a heterogeneous adult population, on admission to intensive care unit (ICU), to predict the occurrence of AKI and hospital mortality. One hundred and two consecutive adult patients had uNGAL measured within 8 h of admission to ICU. The demographic and laboratory data were collected at admission. The diagnosis of AKI was based on AKI Network (AKIN) criteria. The primary outcome was the development of AKI, and the secondary outcome was hospital mortality. The mean age was 54 ± 16.4 years and 65% were males. Urine NGAL (ng/ml) was 69 ± 42 in patients with AKI (n = 42) and 30.4 ± 41.7 in those without AKI (P < 0.001). The area under the receiver operating characteristic (ROC) curve for prediction of AKI was 0.79 and for serum creatinine (SCr) was 0.88. The sensitivity and specificity for a cut-off value of uNGAL of 75 ng/ml to predict AKI were 0.5 and 0.85 respectively. uNGAL > 75 ng/ml was a strong (odd ratio = 5.17, 95% confidence interval: 1.39-19.3) and independent predictor of hospital mortality. A single measurement of uNGAL at admission to ICU exhibited good predictive ability for AKI though the sensitivity was low. The predictive ability of uNGAL was inferior to simultaneously measured SCr at admission, hence limited its clinical utility to predict AKI. However, admission uNGAL was a strong, independent predictor of hospital mortality.

Chronic kidney disease (CKD) is associated with early mortality, decreased quality of life and increased health care expenditures. The aim of this study was to determine whether or not urinary NGAL (uNGAL) level is associated with renal damage and kidney disease progression in patients with CKD and to evaluate the predictive value of uNGAL in progression of CKD. Totally, 91 cases of CKD stage II, III, IV, and 50 age-matched healthy controls were enrolled. The follow-up end-point was 18 months; end-point of the study was progression to an estimated glomerular filtration rate (eGFR) of <15 ml/min and/or CKD stage V. Forty-five cases (49.4%) were progressors and 46 were nonprogressors. uNGAL levels were significantly higher in CKD subjects as compared to healthy controls (log 1.09 ± 0.22 μg/ml in controls versus log 1.22 ± 2.08 μg/ml in stage II, log 3.34 ± 2.74 μg/ml in stage III and log 3.70 ± 0.18 μg/ml in stage IV). Univariate Cox proportional hazards model showed that only eGFR (hazard ratio [HR]: 0.95; 95% confidence interval [CI]: 0.93-0.96; P < 0.001) and uNGAL (HR: 1.11; 95% CI: 1.01-1.20; P < 0.001) were significantly associated with end-point of CKD stage V, but multiple Cox proportional regression model showed significant association of uNGAL (HR: 1.11; 95% CI: 1.01-1.20; P < 0.001) and eGFR (HR: 0.962, 95% CI: 0.95-0.98; P < 0.001) with end-point of CKD stage V. This suggests that uNGAL would not be a simple surrogate index of baseline eGFR, but a marker of CKD progression beyond the information provided by eGFR estimation.

Angiomyolipomas (AML) of the kidney are non-encapsulated benign neoplasms with the incidence of 45-80% in patients with tuberous sclerosis and 1-3% in sporadic cases. There are very few case reports in the literature in which kidneys with AML have been used for transplantation. We report here a 27-year-old female patient who received a live related renal transplant from her mother with isolated angiomyolipoma in donor kidney and on follow-up after 5 years, has stable graft function and tumor size.

Two cases are described of previously unreported false positivity on the Luminex crossmatch assay due to non HLA specific antibodies directed against the beads. In both cases the Luminex crossmatch indicated the presence of donor specific antibodies to class II HLA antigens, which was not substantiated by the clinical scenario or other assays. We could demonstrate the non specificity of these antibodies through using the same assay in a modified form where beads were unexposed to cell lysate and therefore did not carry HLA antigens at all. These cases further serve to emphasize the absolute necessity of correlating positive results with the priming history, and confirming their relevance using other platforms.

Collapsing glomerulopathy (CG) is a pathological entity characterized by collapse and wrinkling of glomerular tuft, podocyte dedifferentiation and hyperplasia. CG may be idiopathic or secondary to other diseases. CG has been described with IgA nephropathy, membranous glomerulopathy, diabetic nephropathy, and lupus nephritis. However, till date there is no report of CG in association with the anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV). Here, we present a case of CG that developed during follow-up in a case of AAV with biopsy proven pauci-immune glomerulonephritis.

Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by systemic vasculitis, asthma and eosinophilia. Severe pulmonary hemorrhage is rare. Renal involvement is seen in approximately 25% and can vary from isolated urinary abnormality to rapidly progressive glomerulonephritis. There is limited evidence to support the use of rituximab in this condition. We present a patient with EGPA who had severe pulmonary hemorrhage and rapidly progressive glomerulonephritis. He responded to standard treatment including prednisolone, cyclophosphamide, and plasma exchange. He subsequently had a relapse of pulmonary hemorrhage that was treated successfully with rituximab.

Nodular glomerulosclerosis, a pathological finding characterized by areas of marked mesangial expansion with accentuated glomerular nodularity can be seen in a number of conditions including diabetic nephropathy, amyloidosis, light chain deposition disease, fibrillary and immunotactoid glomerulopathy, collagen type III disease, nodular membranoproliferative glomerulonephritis, and Takayasu's arteritis. Idiopathic nodular glomerulosclerosis is a diagnosis of exclusion and is reported in patients with hypertension, smoking, chronic obstructive pulmonary disease, obesity, metabolic syndrome, etc. We report two cases of idiopathic nodular glomerulosclerosis, one in obese hypertensive male and the other in nonhypertensive, nonobese female patient.