DOCSLIB.ORG

Overview of Primary Glomerulonephritidies

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Overview of Primary Glomerulonephritidies Overview of Primary Glomerulonephritidies Madeleine V. Pahl, M.D., FASN Division of Nephrology and Hypertension University of California, Irvine Objectives • To define common primary glomerular disorders • To discuss involved pathophysiology • To review their clinical presentation • To review clinical course and therapy Definition (R. J. Glassock) • A disease where the sole organ involved is the kidney (the glomeruli) • Clinical manifestations due to the consequences or pathophysiologic derangements that resulted in glomerular damage • In contrast secondary disorders have manifestations due to systemic disease and glomerular injury Presentation: Clinical Syndromes • Asymptomatic: hematuria (gross or microscopic) and/or proteinuria (< 3g/day) • Acute glomerulonephritis (AGN) • Rapidly progressive glomerulonephritis (RPGN) • Chronic glomerulonephritis (CGN) • Nephrotic Syndrome (NS) Clinical Syndromes: AGN • Abrupt onset of variable degrees of hematuria, proteinuria, reduced GFR, sodium and fluid retention, HTN and oliguria • Tendency to spontaneous recovery • Association with bacterial infection Clinical Syndromes: RPGN • More insidious onset, not abrupt • Dominated by progressive loss of GFR and oliguria • Little tendency for spontaneous recovery • Classification and terminology of this group of disorders (Type I, II, II) Clinical Syndromes: CGN • Clinical expression of a wide variety of glomerular diseases • Vague, all inclusive term refers to progressive reduction in GFR • Varying degrees of hematuria, proteinuria, HTN • Course may be protracted but usually relentless Clinical Syndromes: NS • Heavy proteinuria (> 3-3.5g/day ) • Reduced serum albumin • Edema • HTN • Hyperlipidemia • Lipiduria Pathology • Minimal change • Focal and segmental glomerulosclerosis • Proliferative GN • Pure mesangial proliferative • Endocapillary proliferative • Extra-capillary proliferative (crescentic) • Membrano-proliferative • Membranous GN • Fibrillary/ Immunotactoid GN, Collageno-fibrotic GN, Lipoprotein GN (deposits of Apo E Nephron 83: 193, 1999) It’s nephrotic syndrome It’s membranous GN It’s It’s cancer idiopathic related It’s SLE It’s drug related Normal Glomerular Histology From tutorial: J. Charles Jennette, M.D. Minimal Change Disease UpToDate® MPGN: I UpToDate® Common Primary Glomerulonephritidies • Minimal Change Disease • Focal Segmental Glomerulosclerosis (FSGS) • IgA Nephropathy (Bergèr’s Disease) • Membranoproliferative GN (Types I, II, III) • Membranous GN Case #1 (R. J. Glassock) • 32 yo Hispanic male develops edema and HTN. Has 22g/day urinary protein excretion and Cr = 2mg/dl. BP 180/98 is on no medication and is HIV, Hep C antibody, HBSAg negative. Focal Segmental Glomerulosclerosis • Incidence among all biopsied patients rising, up to 25% of all adults • Most common pattern among African Americans, some secondary forms more common in whites (obesity and hereditary) • Children usually present with NS, adults with asymptomatic proteinuria UpToDate® FSGS UpToDate® FSGS: Histopathologic Variants • Glomerular “tip” lesion: • epithelial cell proliferation and sclerosis in segment adjacent to origin of proximal tubule, • common in whites with heavier proteinuria, preserved GFR, good response to treatment and outcome • “Collapsing/malignant” variant: • global collapse of capillary loops with visceral epithelial cell proliferation (similar to HIV), • Some think hypercellular variant is a type of collapsing FSGC • common in AA with massive proteinuria, worse prognosis • Perihilar variant : • frequently seen with secondary hyperfiltration • Obesity: • fewer glomeruli with FSGS but remaining with glomerulomegally. FSGS-Pathophysiology • Increased circulating levels of soluble urokinase receptor (suPAR) may be a common causative factor (Nat Med. 2011;17(8):952) • Controversial role of Parvovirus B-19 (KI 59:2126, 2001) • Evidence for hyperfiltration: Glomerulomegally and obesity, sleep apnea, or remnant kidney • Genetic Disease: NPHS1 or 2 (nephrin) , alpha actinin 4, TRPC-6 (calcium permeable cation channels) , INF-2 (formin family of actin regulating proteins) Myo1-E (non-muscle myosin) • Genetic Defect: role of MYH9 or APOL1 in African Americans (Nat Genetics. 2008 Oct;40(10):1185-92; Science. 2010 Aug 13;329(5993):841-5) FSGS: Clinical Course • Tendency for progressive renal failure, ESRD in 5-20 yrs • Factors associated with progression: • magnitude of proteinuria, higher serum Cr, • AA ethnicity, response to corticosteroids, • degree of sclerosis, TI damage, “collapsing” variety • Recurrence in allograft in up to 30%. Treatment with plasmapheresis (Clin Nephrol 56:271, 2001) and Rituximab (Transplantation. 2009 Aug 15;88(3):417-20) FSGS: Primary vs. Secondary • Clinical: primary commonly Obesity Idiopathic p presents with acute/subacute Age 44 32 < 0.001 symptomatic NS vs. Cr 1.55 1.96 NS progressive increase in NS (%) 7 54 < 0.001 proteinuria, less edema Proteinuria 4.24 6.89 < 0.004 • Pathology: foot process (g/day) fusion greater in idiopathic disease Foot 41 75 < 0.001 process fusion (%) Kidney Int. 2001 Apr;59(4):1498 FSGS: Treatment • Prednisone 0.5-2.0 mg/kg/day. Total duration of 6 month before declaring pt steroid resistant, then alternate day. Complete and partial response rate 50-60% ( JASN 9:1333, 1998) • Cytotoxic therapy (cyclophosphamide, chlorambucil, MMF) may be useful but not proven (KI 55(S70): S26, 1999). • CsA at 5 mg/kg/day may be effective, relapses common, long term use may be required (KI 55(S70): S26, 1999). • Plasmapheresis in transplant recurrence • Rituximab (Nat Rev Nephrol 2013 9(3):154 Case #2 • 29 yr old Asian man admits to episodes of dark urine associated with mild flank pain. BP 146/90, Urine analysis reveals significant hematuria, protein excretion 1.5 g/day serum Cr = 1.5 IgA Nephropathy • First described in the late 60’s by Bergèr (J Urol Nephrol 74:694, 1968) • Most common GN worldwide, Japan 50% of all primary GN’s, US prevalence 10-15% • Rare in AA, common in Southwest American Indians • Familial form in Kentucky and Italy linked to genetic locus at 6q22-23 (Nat Genet 26:354, 2000) • Presenting symptoms: 40-50% with gross hematuria, 30-40% microscopic hematuria, < 10% with AGN, or NS (?minimal change) • Recurrence after transplant UpToDate® IgA UpToDate® IgA: Pathophysiology (NephSAP JASN suppl 1(1):July 2002) • Deposition of IgA1 but not IgA2 with IgG and C3 in mesangial extracellelular space, • complement activation in-situ, cytokines, chemokines and growth factor activaton • Origin of IgA1 is bone marrow/lymphoid plasma cells (?stem cell disease) • Pt’s overproduce IgA1 in response to ubiquitous antigens (food, bacteria, viruses) • IgA1 is under-galactosylated: • binds to mesangium, self-aggregates into polymeric molecules, provokes autoimmunity with IgG-antiIgA1 complexes, prevents efficient removal by Kupfer cells IgA: Clinical Course • Variable course: • complete remissions reported in up to 23%, most follow benign course, • those that show progression usually take many years (1-3 ml/yr, in NS 9 ml/yr KI 40:1050, 1991) • Australia 41% of ESRD cases are due to IgA • Can develop AKI from ATN following macroscopic hematuria, crescentic GN Determinants of Progression • Oxford classification (mesangial hypercellularity, segmental sclerosis, endocapillary hypercellularity, TA/IF) KI 2011; 80(3): 310) • Renal Risk Score: get points HTN, protein excretion > 1g, global optical pathology score (GOS) to predict risk of death or dialysis at 10 and 20 yrs (JASN 2011; 22(4):752) • Poorly galactosylated IgA1 levels (JASN; 2012 23(9): 1579) Biopsy Proven IgA: Treatment (KI 55(Suppl 70): S56, 1999) BP should be proteinuria controlled with ACEi < 1g 1-3 g > 3g observation GFR GFR nl Renal Insufficiency > 70 ml/min < 70 Fish Oils observation Fish Oils Prednisone IgA: Treatment of Rapidly Progressive Course (KI 55(Suppl 70): S56, 1999) Re-biopsy ATN Crescenteric ?Pulse steroids Dialysis Plasmapheresis Immunosuppressors IgA: Treatment of Macroscopic Hematuria (KI 55(Suppl 70): S56, 1999) Monitor kidney function Stable Recurrent with tonsillitis Deterioration observation Tonsillectomy Consider re-biopsy Treat according to findings Case #3 (R. J. Glassock) • 65 yr old man develops NS. Noticed edema 3 months ago, now severe. Admits to occasional cough and mild intermittent chest pain and SOB. Urine has 20 mg protein/mg Cr, serum Cr = 1.9 mg/dl. Membranous Glomerulonephropathy • Characterized by proteinuria, 75% NS and normal or reduced GFR • Common in adults > 40, represents 40% of NS in adults • Commonly presents with insidious proteinuria, often severe, may selective or non-selective • Thrombotic complications, including RVT incidence of 5-35% MAJOR CAUSES OF MEMBRANOUS GN Idiopathic (75% in adults) Anti-PLA2R antibody SLE WHO class V Drugs: penicillamine bucillamine gold Anti-TNF NSAID Troponin (Thiola) captopril Infections: Hep C (rare) syphillis malaria Malignancy (5-20% > 65 yrs old) Renal cell, gastric, colonic Hematopoetic cell transplant GVHD Kidney transplant De-novo vs recurrence Sarcoid rare IgG4-related disease Rare, major renal form is TI nephritis Membranous GN UpToDate® Membranous: Pathophysiology • M-type phospholipase A2 receptor (PLA2R): podocytes transmembrane receptor, antigen in idiopathic MN (Engl J Med. 2009;361(1):11) • Association with HLA-DQA1 allele, may facilitate autoimmune response to PLA2R1, presence of PLA2R1 polymorphism, or production of hypogalactosylated IgG4 anti-PLA2R1 that activates mannan C’ pathway • Role of external/virus stimuli?? • Injury is C’-dependent:
Load more

Recommended publications
  • Extrarenal Complications of the Nephrotic Syndrome
    Kidney International, Vol. 33 (/988), pp. 1184—1202 NEPHROLOGY FORUM Extrarenal complications of the nephrotic syndrome Principal discussant: DAVID B. BERNARD The University Hospital and Boston University Sc/zoo!ofMedicine, Boston, Massachusetts present and equal. The temperature was 100°F. The blood pressure was 110/70 mm Hg in the right arm with the patient supine and standing. The Editors patient had no skin rashes, peteehiae, clubbing, or jaundice. Examina- JORDANJ. COHEN tion of the head and neck revealed intact cranial nerves and normal fundi. Ears, nose, and throat were normal. The jugular venous pressure Jot-IN T. HARRtNOTON was not increased. No lymph glands were palpable in the neck or JEROME P. KASSIRER axillae, and the trachea was midline, cardiac examination was normal. NICOLA05 E. MAmAs Examination of the lungs revealed coarse rales at the right base but no other abnormalities. Abdominal examination revealed aseites, but no Editor abdominal guarding, tenderness, or rigidity. The liver and spleen were Managing not palpable and no masses were present. The urine contained 4± CHERYL J. ZUSMAN protein; microscopic examination revealed free fat droplets, many oval fat bodies, and numerous fatty casts. Five to 10 red blood cells were seen per high-power field, but no red blood cell casts were present. A Universityof'Chicago Pritzker School of Medicine 24-hr urine collection contained 8 g of protein. The BUN was 22 mg/dl; creatinine, 2.0 mg/dl; and electrolytes were and normal. Serum total calcium was 7.8 mg/dl, and the phosphorus was 4.0 Taf is University School of' Medicine mg/dl.
    [Show full text]
  • Glomerulonephritis Management in General Practice
    Renal disease • THEME Glomerulonephritis Management in general practice Nicole M Isbel MBBS, FRACP, is Consultant Nephrologist, Princess Alexandra lomerular disease remains an important cause Hospital, Brisbane, BACKGROUND Glomerulonephritis (GN) is an G and Senior Lecturer in important cause of both acute and chronic kidney of renal impairment (and is the commonest cause Medicine, University disease, however the diagnosis can be difficult of end stage kidney disease [ESKD] in Australia).1 of Queensland. nikky_ due to the variability of presenting features. Early diagnosis is essential as intervention can make [email protected] a significant impact on improving patient outcomes. OBJECTIVE This article aims to develop However, presentation can be variable – from indolent a structured approach to the investigation of patients with markers of kidney disease, and and asymptomatic to explosive with rapid loss of kidney promote the recognition of patients who need function. Pathology may be localised to the kidney or further assessment. Consideration is given to the part of a systemic illness. Therefore diagnosis involves importance of general measures required in the a systematic approach using a combination of clinical care of patients with GN. features, directed laboratory and radiological testing, DISCUSSION Glomerulonephritis is not an and in many (but not all) cases, a kidney biopsy to everyday presentation, however recognition establish the histological diagnosis. Management of and appropriate management is important to glomerulonephritis (GN) involves specific therapies prevent loss of kidney function. Disease specific directed at the underlying, often immunological cause treatment of GN may require specialist care, of the disease and more general strategies aimed at however much of the management involves delaying progression of kidney impairment.
    [Show full text]
  • Management of Adult Minimal Change Disease
    Kidney CaseCJASN Conference: ePress. Published on April 5, 2019 as doi: 10.2215/CJN.01920219 How I Treat Management of Adult Minimal Change Disease Stephen M. Korbet and William L. Whittier Clin J Am Soc Nephrol 14: ccc–ccc, 2019. doi: https://doi.org/10.2215/CJN.01920219 Introduction Initial Treatment and Course in Adult Minimal Minimal change disease is responsible for idiopathic Change Disease Division of nephrotic syndrome in .75% of children and up to Minimal change disease in adults is highly steroid Nephrology, 30% of adults (1–5). Although secondary causes of sensitive, but steroid resistance is seen in 5%–20% of Department of Medicine, Rush minimal change disease (i.e., nonsteroidal anti- adult patients (1–6). When steroid resistance is ob- fl University Medical in ammatory drugs, lithium, and lymphoproliferative served, the patient often has FSGS on re-examination Center, Chicago, disorders) are uncommon in children, they account of the initial biopsy or on rebiopsy (1,3,6). Although Illinois for up to 15% of minimal change disease in adults 95% of children attain a remission with steroid (1,3). Thus, it is important to assess adults with therapy by 8 weeks, only 50%–75% of adults do so Correspondence: minimal change disease for secondary causes as the (1–6). It is not until after 16 weeks of treatment that Dr. Stephen M. Korbet, – Division of prognosis, and therapeutic approach is determined most adults (75% 95%) enter a remission, with the Nephrology, by the underlying etiology. majority attaining a complete remission (proteinuria Department of of #300 mg/d) and a minority attaining a partial Medicine, Rush remission (proteinuria of .300 mg but ,3.5 g/d).
    [Show full text]
  • T Cells and Minimal Change Disease
    EDITORIALS J Am Soc Nephrol 13: 1409–1411, 2002 T Cells and Minimal Change Disease ROBYN CUNARD AND CAROLYN J. KELLY Research Service, VA San Diego Healthcare System; and Department of Medicine, University of California, San Diego, California. It is one of the ironies of medical practice that as physicians we the lymphokine may exert a direct effect on the GBM or can competently and confidently treat diseases of whose patho- activate mesangial cells to produce a factor that altered glo- genesis we remain woefully ignorant. merular permeability (2). Such is the case with minimal change disease, the most This publication preceded by over a decade the molecular common diagnosis associated with the nephrotic syndrome in definition of the T cell antigen receptor as a heterodimeric children (MCNS). The disease manifestations of nephrotic- structure derived from gene rearrangements (3) and the cloning range proteinuria, hypoalbuminemia, and hyperlipidemia in of the first cytokine or lymphokine (4). It preceded by several such patients are typically reversible with corticosteroid ther- years the earliest attempts to characterize subsets of T lympho- apy. MCNS has, by definition, no abnormalities apparent on cytes by their functional and phenotypic heterogeneity (5,6). analysis of kidney biopsy specimens by light microscopy. Shalhoub’s hypothesis has resurfaced multiple times since Humoral components of the immune system, such as Ig and 1974, as clinical investigators take advantage of increased complement, are absent on immunofluorescent analysis of cor- knowledge about immunology and technical breakthroughs to tical kidney sections. The sole abnormalities seen in the kidney reexamine the role of T cells in MCNS.
    [Show full text]
  • Portal Vein Thrombosis in Minimal Change Disease
    Ewha Med J 2014;37(2):131-135 Case http://dx.doi.org/10.12771/emj.2014.37.2.131 Report pISSN 2234-3180 • eISSN 2234-2591 Portal Vein Thrombosis in Minimal Change Disease Gyuri Kim, Jung Yeon Lee, Su Jin Heo, Yoen Kyung Kee, Seung Hyeok Han Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea Among the possible venous thromboembolic events in nephrotic syndrome, renal Received October 22, 2013, Accepted January 6, 2014 vein thrombosis and pulmonary embolism are common, while portal vein thrombosis (PVT) is rare. This report describes a 26-year-old man with histologically proven mini- Corresponding author mal change disease (MCD) complicated by PVT. The patient presented with epigastric Seung Hyeok Han pain and edema. He had been diagnosed with MCD five months earlier and achieved Department of Internal Medicine, Yonsei complete remission with corticosteroids, which were discontinued one month before University College of Medicine, 50 Yonsei-ro, Seodaemun-gu, Seoul 120-752, Korea the visit. Full-blown relapsing nephrotic syndrome was evident on laboratory and clini- Tel: 82-2-2228-1975, Fax: 82-2-393-6884 cal findings, and an abdominal computed tomography revealed PVT. He immediately E-mail: [email protected] received immunosuppressants and anticoagulation therapy. An eight-week treatment resulted in complete remission, and a follow-up abdominal ultrasonography showed disappearance of PVT. In conclusion, PVT is rare and may not be easily diagnosed in patients with nephrotic syndrome suffering from abdominal pain. Early recognition of Key Words this rare complication and prompt immunosuppression and anticoagulation therapy Minimal change disease; Portal vein are encouraged to avoid a fatal outcome.
    [Show full text]
  • Minimal Change Disease and Focal Segmental Glomerulosclerosis
    4 Minimal Change Disease and Focal Segmental Glomerulosclerosis Agnes B. Fogo Introduction/Clinical Setting Minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) are both common causes of the nephrotic syndrome. Minimal change disease accounts for greater than 90% of cases of nephrotic syn- drome in children, vs. 10% to 15% of adults with nephrotic syndrome (1). Focal segmental glomerulosclerosis has been increasing in incidence in the United States in both African Americans and in Hispanics, in both adult and pediatric populations (2–4). It is now the most common cause of nephrotic syndrome in adults in the U.S. Patients with FSGS may have hypertension and hematuria. Serologic studies, including complement levels, are typically within normal limits in both MCD and FSGS. Pathologic Features Light Microscopy Minimal change disease shows normal glomeruli by light microscopy (Fig. 4.1). In FSGS, sclerosis involves some, but not all, glomeruli (focal), and the sclerosis affects a portion of, but not the entire, glomerular tuft (seg- mental) (Fig. 4.2) (1,5–7). Sclerosis is defined as increased matrix with obliteration of the capillary lumen. Uninvolved glomeruli in FSGS show no apparent lesions by light microscopy; FSGS may also entail hyalinosis, caused by insudation of plasma proteins, producing a smooth, glassy (hyaline) appearance (Fig. 4.3). Adhesions (synechiae) of the capillary tuft to Bowman’s space are a very early sclerosing lesion. Focal segmental glomerulosclerosis is diagnosed when even a single glomerulus shows segmental sclerosis. Therefore, samples must be ade- quate to detect these focal and segmental lesions. A biopsy with only 10 glomeruli has a 35% probability of missing a focal (10% involved) lesion, decreasing to 12% if 20 glomeruli are sampled (8).
    [Show full text]
  • Minimal Change Disease: a Case Report of an Unusual Relationship Fahad Edrees Washington University School of Medicine in St
    Washington University School of Medicine Digital Commons@Becker Open Access Publications 2016 Minimal change disease: A case report of an unusual relationship Fahad Edrees Washington University School of Medicine in St. Louis Robert M. Black Saint Vincent Hospital Laszlo Leb Saint Vincent Hospital Helmut Rennke Harvard Medical School Follow this and additional works at: https://digitalcommons.wustl.edu/open_access_pubs Recommended Citation Edrees, Fahad; Black, Robert M.; Leb, Laszlo; and Rennke, Helmut, ,"Minimal change disease: A case report of an unusual relationship." Saudi Journal of Kidney Diseases and Transplantation.27,4. (2016). https://digitalcommons.wustl.edu/open_access_pubs/5287 This Open Access Publication is brought to you for free and open access by Digital Commons@Becker. It has been accepted for inclusion in Open Access Publications by an authorized administrator of Digital Commons@Becker. For more information, please contact [email protected]. Saudi J Kidney Dis Transpl 2016;27(4):816-820 © 2016 Saudi Center for Organ Transplantation Saudi Journal of Kidney Diseases and Transplantation Case Report Minimal Change Disease: A Case Report of an Unusual Relationship Fahad Edrees1, Robert M. Black2,4,Laszlo Leb3,4, Helmut Rennke5 1Department of Medicine, Division of Nephrology, Washington University School of Medicine, Barnes Jewish Hospital, Saint Louis, MO, 2Division of Renal Medicine and 3Department of Hematology Oncology, Saint Vincent Hospital, 4Reliant Medical Group, Worcester, 5Department of Renal Pathology, Harvard Medical School, Brigham and Women’s Hospital, Boston, MA, USA ABSTRACT. Kidney injury associated with lymphoproliferative disorders is rare, and the exact pathogenetic mechanisms behind it are still poorly understood. Glomerular involvement presen- ting as a nephrotic syndrome has been reported, usually secondary to membranoproliferative glomerulonephritis.
    [Show full text]
  • Characterization of Iga Deposition in the Kidney of Patients with Iga Nephropathy and Minimal Change Disease
    Journal of Clinical Medicine Article Characterization of IgA Deposition in the Kidney of Patients with IgA Nephropathy and Minimal Change Disease Won-Hee Cho 1, Seon-Hwa Park 2, Seul-Ki Choi 2, Su Woong Jung 2, Kyung Hwan Jeong 3, Yang-Gyun Kim 2 , Ju-Young Moon 2, Sung-Jig Lim 4 , Ji-Youn Sung 5, Jong Hyun Jhee 6, Ho Jun Chin 7,8, Bum Soon Choi 9 and Sang-Ho Lee 2,* 1 Department of Medicine, Graduate School, Kyung Hee University, Seoul 02447, Korea; [email protected] 2 Division of Nephrology, Department of Internal Medicine, Kyung Hee University Hospital at Gangdong, Seoul 05278, Korea; [email protected] (S.-H.P.); [email protected] (S.-K.C.); [email protected] (S.W.J.); [email protected] (Y.-G.K.); [email protected] (J.-Y.M.) 3 Division of Nephrology, Department of Internal Medicine, Kyung Hee University Medical Center, Seoul 02447, Korea; [email protected] 4 Department of Pathology, Kyung Hee University Hospital at Gangdong, Kyung Hee University, Seoul 05278, Korea; [email protected] 5 Department of Pathology, Kyung Hee University Hospital, Kyung Hee University College of Medicine, Seoul 02447, Korea; [email protected] 6 Division of Nephrology, Department of Internal Medicine, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul 06273, Korea; [email protected] 7 Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam 13620, Korea; [email protected] 8 Department of Internal Medicine, College of Medicine, Seoul National University, Seoul 03080, Korea 9 Division of Nephrology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul 06591, Korea; [email protected] * Correspondence: [email protected] Received: 5 July 2020; Accepted: 9 August 2020; Published: 12 August 2020 Abstract: Approximately 5% of patients with IgA nephropathy (IgAN) exhibit mild mesangial lesions with acute onset nephrotic syndrome and diffuse foot process effacement representative of minimal change disease (MCD).
    [Show full text]
  • Minimal Change Disease
    CJASN ePress. Published on December 23, 2016 as doi: 10.2215/CJN.05000516 Minimal Change Disease Marina Vivarelli,* Laura Massella,* Barbara Ruggiero,† and Francesco Emma* Abstract Minimal change disease (MCD) is a major cause of idiopathic nephrotic syndrome (NS), characterized by intense proteinuria leading to edema and intravascular volume depletion. In adults, it accounts for approximately 15% of patients with idiopathic NS, reaching a much higher percentage at younger ages, up to 70%–90% in children >1 year of age. In the pediatric setting, a renal biopsy is usually not performed if presentation is typical and the patient *Division of responds to therapy with oral prednisone at conventional doses. Therefore, in this setting steroid-sensitive NS Nephrology and can be considered synonymous with MCD. The pathologic hallmark of disease is absence of visible alterations by Dialysis, Bambino light microscopy and effacement of foot processes by electron microscopy. Although the cause is unknown and it is Gesu` Children’s likely that different subgroups of disease recognize a different pathogenesis, immunologic dysregulation and Hospital, IRCCS, Rome, Italy; and modifications of the podocyte are thought to synergize in altering the integrity of the glomerular basement †Clinical Research membrane and therefore determining proteinuria. The mainstay of therapy is prednisone, but steroid-sensitive Center for Rare forms frequently relapse and this leads to a percentage of patients requiring second-line steroid-sparing immu- Diseases “Aldo e Cele nosuppression. The outcome is variable, but forms of MCD that respond to steroids usually do not lead to chronic Dacco`”, IRCCS – renal damage, whereas forms that are unresponsive to steroids may subsequently reveal themselves as FSGS.
    [Show full text]
  • Glomerular Diseases Associated with Malignancies: Histopathological Pattern and Association with Circulating Autoantibodies
    antibodies Article Glomerular Diseases Associated with Malignancies: Histopathological Pattern and Association with Circulating Autoantibodies Sophia Lionaki 1,* , Smaragdi Marinaki 1, Konstantinos Panagiotellis 1, Ioanna Tsoumbou 1, George Liapis 2, Ioanna Vlahadami 3, Athanasios Tzioufas 3, Petros Sfikakis 4 and Ioannis Boletis 1 1 Nephrology Department & Transplantation Unit, Faculty of Medicine, Laiko Hospital, National & Kapodistrian University of Athens, 11527 Athens, Greece; [email protected] (S.M.); [email protected] (K.P.); [email protected] (I.T.); [email protected] (I.B.) 2 Pathology Department, Faculty of Medicine, Laiko Hospital, National & Kapodistrian University of Athens, 11527 Athens, Greece; [email protected] 3 Department of Pathophysiology, Faculty of Medicine, Laiko Hospital, National & Kapodistrian University of Athens, 11527 Athens, Greece; [email protected] (I.V.); [email protected] (A.T.) 4 1st Department of Propaedeutic and Internal Medicine and Joined Rheumatology Program, Medical School, Laiko Hospital, National and Kapodistrian University of Athens, 11527 Athens, Greece; psfi[email protected] * Correspondence: sofi[email protected]; Tel.: +30-2110195670; Fax: +30-2132061856 Received: 31 March 2020; Accepted: 18 May 2020; Published: 25 May 2020 Abstract: Aim: Glomerular diseases (GD) associated with malignancies (AM, GDAM) have unique features, which are important to recognize, in the light of the progress made in cancer therapy. We aimed to describe the clinical and histopathological characteristics of patients with GDAM in relation to the presence of circulating autoantibodies, pointing to potential immune pathogenic pathways connecting cancer to GD. Materials and Methods: The included patients were studied retrospectively on the basis of a kidney biopsy proving GD and a related biopsy to establish the diagnosis of AM.
    [Show full text]
  • History of the International Pediatric Nephrology Association: a Lifecourse Journey 18Th Congress Venice, Italy, October 17-21, 2019
    History of the International Pediatric Nephrology Association: A Lifecourse Journey 18th Congress Venice, Italy, October 17-21, 2019 Rick Kaskel, MD., PhD, Chief Emeritus Children’s Hospital at Montefiore Albert Einstein College of Medicine IPNA History and Archive Project Collaboration: Aaron Friedman, Jochen Ehrich, Robert Chevalier, Sally Jones & George Reusz Historical Interviews: Henry Barnett, Ira Greifer, John Lewy, Chester Edelmann, Jr. & Russell Chesney (All Deceased) Special Thanks to the ASPN History Project Committee Frederick Kaskel Lucie Semanská Historical Dates of Interest • 1948 – 1st Conference on the Nephrotic Syndrome – Boston (Metcoff, Barnett) • 1952 – National Nephrosis Foundation – New York • 1958 – NNF and AHA Council on the kidney merged – National Kidney Foundation begins • 1965 – 17th and Last Annual Conference on the Kidney (Acute Glomerulonephritis) • The period from 1948 to the founding of the International Study of Kidney Disease in Children, the European Society of Pediatric Nephrology in 1966, the Japanese Society of Pediatric Nephrology 1967, and the American Society of Pediatric Nephrology in 1969, was based on six major advances in the field. The Six Critical Discoveries that Underlie Pediatric Nephrology as a Discipline • The use of ACTH and glucocorticoids in the treatment of idiopathic nephrotic syndrome of childhood. • Percutaneous renal biopsies in children for classification of diseases. • Evidence that immunologic factors are essential in many renal diseases. • End stage renal disease can be treated with dialysis. • Children can receive renal allografts from living and cadaveric donors. • Hypertension in children is largely the consequence of renal disease in 80%. First Textbooks in Pediatric Nephrology • An influential textbook of adult and pediatric kidney disease in children was the 1950 publication Addis'Glomerular Nephritis which contained precise descriptions of childhood renal syndromes including acute and chronic glomerulonephritis.
    [Show full text]
  • The Transition from Minimal Change Disease to Focal and Segmental Glomerulosclerosis in a Patient with Nephrotic Syndrome: a Case Report
    The Transition From Minimal Change Disease to Focal and Segmental Glomerulosclerosis in a Patient With Nephrotic Syndrome: a Case Report Long Tang Beijing Hospital of Traditional Chinese Medicine https://orcid.org/0000-0003-4609-413X Zhen Cai Beijing Hospital of Traditional Chinese Medicine Yuan Meng Beijing Hospital of Traditional Chinese Medicine Wen-jing Zhao Beijing Hospital of Traditional Chinese Medicine Su-Xia Wang ( [email protected] ) Peking University First Hospital Case Report Keywords: minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), nephrotic syndrome(NS), Nephrotoxic metal Posted Date: January 27th, 2021 DOI: https://doi.org/10.21203/rs.3.rs-153788/v1 License: This work is licensed under a Creative Commons Attribution 4.0 International License. Read Full License Page 1/12 Abstract Background: Although minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) have been described as two separate forms of nephrotic syndrome(NS), they are not completely independent. We report a patient presenting a transition from MCD to FSGS, review the literature and explore the relationship between the two diseases. Case presentation: A 42-year-old male welder, Asian, presenting lower extremity edema and elevated serum creatinine, had laboratory exams indicating NS and end-stage renal disease(ESRD). The patient had a kidney biopsy 20 years earlier for NS, which indicated MCD, and this repeated kidney biopsy suggested FSGS. After treatment follow-up, the patient was eventually admitted to renal replacement therapy. Conclusions: MCD and FSGS may be different stages of the same disease. The transition from MCD to FSGS in this case indicates the progression of the disease, which may be related to the excessive metal caused by occupation.
    [Show full text]