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Tanja Kersnik Levart, Dušan Ferluga, Alenka Vizjak, Jerica Mraz, Nika Kojc
BACKGROUND: Understanding the role of alternative complement pathway dysregulation in membranoproliferative glomerulonephritis (MPGN) has led to a dramatic shift in its classification into two subgroups: immune complex-mediated MPGN and complement-mediated MPGN, consisting of dense deposit disease and C3 glomerulonephritis (C3GN). A limited number of C3GN cases have been published to date with not yet conclusive results since the novel therapeutic approach with eculizumab was introduced...
October 7, 2016: Diagnostic Pathology
Samir G Mallat, Houssam S Itani, Rana M Abou-Mrad, Rima Abou Arkoub, Bassem Y Tanios
Rituximab is a chimeric anti-CD20 antibody that results in depletion of B-cell lymphocytes. It is currently used in the treatment of a variety of autoimmune diseases, in addition to CD20-positive lymphomas. The use of rituximab in the treatment of the adult primary glomerular diseases has emerged recently, although not yet established as first-line therapy in international guidelines. In patients with steroid-dependent minimal change disease or frequently relapsing disease, and in patients with idiopathic membranous nephropathy (IMN), several retrospective and prospective studies support the use of rituximab to induce remission, whereas in idiopathic focal and segmental glomerulosclerosis (FSGS), the use of rituximab has resulted in variable results...
2016: Therapeutics and Clinical Risk Management
Nirupama Gupta, Dara N Wakefield, William L Clapp, Eduardo H Garin
BACKGROUND: Membranoproliferative glomerulonephritis (MPGN type I, II and III) was reclassified in 2013 as MPGN and C3 glomerulopathy (C3G) based on the complement system activation mechanism. OBJECTIVES: To evaluate whether C4d, a component of the classical pathway, could be a diagnostic tool in differentiating between MPGN and C3G. METHODS: We conducted a retrospective study of 15 MPGN type I, II and III and 13 minimal change disease (MCD) patients diagnosed between 2000 and 2012...
August 29, 2016: Nefrología: Publicación Oficial de la Sociedad Española Nefrologia
Daisuke Kaneyuki, Yukiko Komeno, Hiroshi Yoshimoto, Naoki Yoshimura, Kuniko Iihara, Tomiko Ryu
A 47-year-old woman with ulcerative colitis (UC) was admitted to our hospital for renal dysfunction and progressive anemia. Colonoscopy revealed intestinal lesions and pathological findings showed intravascular large B-cell lymphoma (IVLBCL). According to the polymerase chain reaction analysis of sequential rectal specimens, we concluded that she suffered from intestinal BCL, not UC. After chemotherapy, her renal function progressed to nephrotic syndrome. The pathological findings of renal biopsy specimens indicated membranoproliferative glomerulonephritis (MPGN)...
2016: Internal Medicine
Swaty Arora, Daniel Levitan, Narottam Regmi, Gurinder Sidhu, Raavi Gupta, Anthony D Nicastri, Subodh J Saggi, Albert Braverman
The incidence of glomerulonephritis, as a manifestation of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL), has always been considered low. Though renal infiltration is usually detected at post-mortem, it does not often interfere with kidney function [1]. Though immunoglobulin (Ig) levels in most CLL patients are subnormal, small monoclonal Ig peaks are occasionally detected in serum. They were present in a number of reported CLL nephropathy patients, and not all were cryoglobulins; serum and glomerular staining were concordant for Ig type [2,3,4]...
September 2016: Blood Cells, Molecules & Diseases
Vlad Florin Iovănescu, Adriana Florentina Constantinescu, Costin Teodor Streba, Sorin Ioan Zaharie, Cristin Constantin Vere, Eugen Mandache, Mircea Niculae Penescu, Eugen MoŢa
Chronic viral hepatitis B and C may associate different extrahepatic manifestations and renal disease is the most frequent. Kidney damage is represented in most cases by glomerulopathies, which include membranous nephropathy, membranoproliferative glomerulonephritis (MPGN), IgA nephropathy, focal and segmental glomerulosclerosis and diabetic nephropathy. We conducted a retrospective study on 639 patients diagnosed with chronic viral hepatitis B and C and different renal diseases. Complete evaluation of liver and renal status was performed and, in selected cases, renal biopsy...
2016: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
A Mitchell, B Nokay, H Guberina, U Behlen-Wilm, M Nagel, M Kirschfink, T Wiech, A Kribben, A Bienholz
OBJECTIVE: Malignant hypertension as borne out by uncontrolled hypertension with accelerated target organ damage has a variable and often dire prognosis. Loss of kidney function is a hallmark of the condition and is frequently accompanied by thrombotic microangiopathy (TMA). DESIGN AND METHOD: Here we present the case of a patient with malignant hypertension, who was successfully treated using a combination of in-label and off-label medication according to the following pathophysiological concepts: 1) Innate immunity has been identified as a player in blood pressure (BP) control, 2) the complement inhibitor eculizumab has become available for treating atypical hemolytic uremic syndrome (aHUS), which shares many features of malignant hypertension, 3) endothelin (ET) antagonism, although not currently approved for these indications, has been shown to be effective for treating proteinuria and as a possible adjunct for the treatment of resistant hypertension...
September 2016: Journal of Hypertension
Maurizio Salvadori, Giuseppina Rosso
This review revises the reclassification of the membranoproliferative glomerulonephritis (MPGN) after the consensus conference that by 2015 reclassified all the glomerulonephritis basing on etiology and pathogenesis, instead of the histomorphological aspects. After reclassification, two types of MPGN are to date recognized: The immunocomplexes mediated MPGN and the complement mediated MPGN. The latter type is more extensively described in the review either because several of these entities are completely new or because the improved knowledge of the complement cascade allowed for new diagnostic and therapeutic approaches...
July 6, 2016: World Journal of Nephrology
Punit Jain, Rashmi Kanagal-Shamanna, William Wierda, Alessandra Ferrajoli, Michael Keating, Nitin Jain
OBJECTIVE/BACKGROUND: Membranoproliferative glomerulonephritis (MPGN) is a common extramedullary renal presentation in chronic lymphocytic leukemia (CLL) and can present with either a frank renal failure or proteinuria. One of its etiologies has been attributed to a paraneoplastic, immune complex phenomenon occurring in CLL. Although there is no standard of care in such patients, use of anti-CD20 monoclonal antibodies like rituximab have been used before in such patients with variable responses...
June 23, 2016: Hematology/oncology and Stem Cell Therapy
Md Ghulam Yusuf, Bidhu Bhushan Das, Amaresh Chandra Shaha, Md Zakir Hossain
In Bangladesh, there are very few studies about biopsy proven adult Nephrotic syndrome (NS) with histological types and their clinical findings. To determine the histological types of glomerulonephritis (GN) in adult NS and correlate them with the clinical presentations and biochemical parameters, we studied 100 biopsies in 87 patients who underwent ultrasonography- guided renal biopsy in Rangpur Medical College and Hospital from July 2010 to June 2012. The mean age of the patients was 32.8 ± 13.2 years; male was preponderance (72...
May 2016: Saudi Journal of Kidney Diseases and Transplantation
Mabrouk I Ismail, Khaled Lakouz, Eman Abdelbary
To evaluate the clinicopathological correlation of renal pathology and outline the frequencies of different renal diseases, beside assessment of lupus nephritis (LN), we studied 150 patients [72 (48%) males and mean age of 33.82 ± 15.4 years] who were subjected to native renal biopsy; 112 (72.8%) biopsied patients were diagnosed to have glomerulonephritis (GN). The most frequent clinical renal syndrome was nephrotic syndrome found in 55 (36.6%) patients, followed by nephritic syndrome in 38 (25.3%) patients, chronic kidney disease in 28 (18...
May 2016: Saudi Journal of Kidney Diseases and Transplantation
M K Arif, M Arif, N Amjad
The developing world is observing changing histopathological patterns of idiopathic nephrotic syndrome (INS). However, the true burden of non-minimal change disease (non-MCD) presenting as INS remains unestimated owing to a paucity of data on renal biopsies. Data were collected from January 2006 to June 2014 on 75 children up to 16 years of age who underwent renal biopsies for INS. Mean age at biopsy was 11.2 ± 3.7 years. The male to female ratio was 1.5:1. A total of 25 (33.3%) children were steroid sensitive, 36 (48%) were steroid resistant, 10 (13...
May 2016: Indian Journal of Nephrology
R Nada, A Kumar, V G Kumar, K L Gupta, K Joshi
Renal biopsy interpretation requires histopathology, direct immunofluorescence (DIF) and electron microscopy. Formalin-fixed, paraffin-embedded tissue (FFPE) sent for light microscopy can be used for DIF after antigen retrieval. However, complement staining has not been satisfactory. We standardized DIF using proteinase-K for antigen retrieval in FFPE renal biopsies. A pilot study was conducted on known cases of membranous glomerulonephritis (MGN), membranoproliferative type-1 (MPGN-1), immunoglobulin A nephropathy (IgAN), and anti-glomerular basement disease (anti-GBM)...
May 2016: Indian Journal of Nephrology
Ying Chen, Jinquan Wang, Feng Xu, Caihong Zeng, Zhihong Liu
AIMS: Kimura's disease (KD) with renal involvement is a rare disease. Optimal treatments are still not well established. It is necessary to analyze clinicopathological features, treatment responses, and prognosis for improving KD diagnosis and treatment. MATERIALS AND METHODS: Clinicopathological data, treatment responses, and prognosis were collected and analyzed retrospectively. RESULTS: The patients consisted of 27 males and 2 females, with an average age of 35...
June 2016: Clinical Nephrology
Shi-Jun Li, Shu-Tian Xu, Hui-Ping Chen, Ming-Chao Zhang, Feng Xu, Shui-Qin Cheng, Zhi-Hong Liu
AIM: Cryoglobulinemic glomerulonephritis related to hepatitis B virus (HBV) infection has been rarely reported. The aim of this study was to investigate the clinical features, renal biopsy findings in patients with HBV-associated cryoglobulinemia. METHODS: Twelve patients with HBV-associated cryoglobulinemia were identified in this study. The demographic, clinical, pathological characteristics, treatment and follow-up data were analyzed. RESULTS: Renal involvement was characterized by nephrotic range proteinuria with microscopic hematuria in all patients, and impaired renal function in 9 patients (75%)...
April 7, 2016: Nephrology
Arvind Madan, Snezana Mijovic-Das, Ana Stankovic, Geoffrey Teehan, Amber S Milward, Anupa Khastgir
BACKGROUND: Current first-line anti-proteinuric treatments for nephrotic syndrome (NS) do not produce an effective response in all patients and are not tolerated by some patients. Additional effective and tolerable treatment options in NS are strongly needed. This retrospective case series is the largest to date to examine Acthar gel (adrenocorticotropic hormone, ACTH) in patients with varied-etiology NS. METHODS: This multicenter retrospective case series included adult patients with NS (N = 44) treated with Acthar gel at 6 clinical practices...
March 31, 2016: BMC Nephrology
Lauren A Dalvin, Fernando C Fervenza, Sanjeev Sethi, Jose S Pulido
PURPOSE: Membranoproliferative glomerulonephritis (MPGN) recently was reclassified to reflect the underlying cause as a complement-mediated and immune complex-mediated disease. This classification is based on renal biopsy immunofluorescence examination, making the former electron-microscopy classification obsolete. In this report, we describe related eye findings in patients with MPGN based on the new classification. DESIGN: Retrospective case series. PARTICIPANTS: All Mayo Clinic Rochester patients with pathology-confirmed complement- and immune complex-mediated MPGN who had available ophthalmology records from 1997 through 2014 were included in this study...
July 2016: Ophthalmology
Ken Iseri, Masayuki Iyoda, Yasutaka Yamamoto, Naoto Kobayashi, Takashi Oda, Yutaka Yamaguchi, Takanori Shibata
We herein report the case of an 18-year-old boy who developed nephrotic syndrome and hypertension after upper airway inflammation. Post-streptococcal acute glomerulonephritis was diagnosed on the basis of a high antistreptolysin O titer, hypocomplementemia, proteinuria, and microscopic hematuria. A renal biopsy was performed due to persistent proteinuria, and the pathological diagnosis was membranoproliferative glomerulonephritis (MPGN) type I. Glomeruli showed positive staining for nephritis-associated plasmin receptor (NAPlr), a nephritogenic group A streptococcal antigen, and plasmin activity was found in a similar distribution as NAPlr deposition...
2016: Internal Medicine
Silvia Berra, Alberto Clivio
Several mutations have been identified in the gene coding for Complement Factor H (FH) from patients with atypical Hemolytic Uraemic Syndrome (aHUS), Age-related Macular Degeneration (AMD) and Membranoproliferative Glomerulonephritis (MPGN). These data allow for a precise description of the structural changes affecting FH, but a simple test for specifically assessing FH function routinely is not yet of common use. We have produced and characterised a monoclonal antibody (5H5) which discriminates between FH and the smaller FH-like 1 and FH-related proteins and show here that it specifically binds to FH without detecting the smaller isoforms...
April 2016: Molecular Immunology
Rishi Pruthi, Mark McClure, Anna Casula, Paul J Roderick, Damian Fogarty, Mark Harber, Rommel Ravanan
Glomerulonephritis (GN) is the primary diagnosis in 20% to 40% of patients receiving a renal transplant. Here we studied patient survival and graft outcomes in patients with GN transplanted in the UK. UK Renal Registry data were used to analyze patient survival and graft failure in incident transplant patients between 1997 to 2009 who had a diagnosis of primary GN, in comparison to patients transplanted with adult polycystic kidney disease (APKD) or diabetes. Multivariable regression analysis adjusted for age, sex, donor type, ethnicity, donor age, time on dialysis, human leukocyte antigen mismatch, cold ischemic time, and graft failure (for patient survival)...
April 2016: Kidney International
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