Membranoproliferative glomerulonephritis

https://https://www.youtube.com/watch?v=uriIHhO5kVQ%7C350}}

Membranoproliferative glomerulonephritis
ICD-10	N00-N08 with .2 suffix
ICD-9	581.2, 582.2, 583.2
MeSH	D015432

Membranoproliferative glomerulonephritis Microchapters
Home
Patient Information
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Membranoproliferative glomerulonephritis from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
Diagnostic Study of Choice
History and Symptoms
Physical Examination
Laboratory Findings
Electrocardiogram
Chest X Ray
CT
MRI
Echocardiography or Ultrasound
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Primary Prevention
Secondary Prevention
Cost-Effectiveness of Therapy
Future or Investigational Therapies
Case Studies
Case #1
Membranoproliferative glomerulonephritis On the Web
Most recent articles
Most cited articles
Review articles
CME Programs
Powerpoint slides
Images
American Roentgen Ray Society Images of Membranoproliferative glomerulonephritis All Images X-rays Echo & Ultrasound CT Images MRI
Ongoing Trials at Clinical Trials.gov
US National Guidelines Clearinghouse
NICE Guidance
FDA on Membranoproliferative glomerulonephritis
CDC on Membranoproliferative glomerulonephritis
Membranoproliferative glomerulonephritis in the news
Blogs on Membranoproliferative glomerulonephritis
Directions to Hospitals Treating Membranoproliferative glomerulonephritis
Risk calculators and risk factors for Membranoproliferative glomerulonephritis

• Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Ali Poyan Mehr, M.D. [2]; Associate Editor-In-Chief: Olufunmilola Olubukola M.D.[3]Cafer Zorkun, M.D., Ph.D. [4]

Overview

Membranoproliferative Glomerulonephritis (MPGN) is a relatively uncommon inflammatory glomerulopathy that can cause chronic nephritis. Based on the histological pattern of glomerular injury it has been described as a chronic kidney disease found mostly in children and young adults. Like many forms of glomerulopathies, membranoproliferative glomerulonephritis (glomerulopathy) has been a diagnosis of tissue pathology rather the diagnosis of a specific disease entity. Therefore, the term membranoploriferative glomerulonephritis (MPGN) relates to a pattern of glomerular injury characterized by mesangial proliferation and expansion, lobularization of the glomerular tufts and double contours which can be caused by many disease states . Glomerular injury occurs due to deposition of immune complexes on the glomerular mesangium or on the glomerular basement membrane. MPGN has been categorized into 3 types based on the histological pattern of glomerular damage. Clinically, MPGN often present with hematuria, varying degrees of proteinuria, with or without Glomerular filtration rate impairment depending on the severity of glomerular injury, and the underlying etiology.

Historical Perspective

• The term membranous glomerulonephritis was used first by Bell in 1946 to describe a category of glomerular renal disease classified within the spectrum of Ellis type II glomerulonephritis. This category also included lipoid nephrosis, lobular glomerulonephritis, and chronic glomerulonephritis .

• In 1957, David Jones, a renal pathologist from Syracuse University in New York, separated membranous glomerulonephritis as a distinct morphologic entity using the special stain periodic acid–silver methenamine (now known as Jones stain). Jones fully illustrated the special features of this lesion such as lobular glomerulonephritis (now known as membranoproliferative glomerulonephritis), lipoid nephrosis (now known as minimal change disease), and chronic glomerulonephritis (now known as focal and segmental glomerulosclerosis). The thickening of the capillary wall and alteration in basement membrane structure, so characteristic of the membranous lesion, were convincingly shown . 

• The electron-dense subepithelial location of the were also subsequently identified by Movat and McGregor in 1959 using electron microscopic methods applied to renal biopsy specimens in 1957. Mellors  in 1957 had identified the third component of the unique lesion of membranous glomerulonephritis; namely, the presence of immunoglobulin in the deposits, using the immunofluorescence technique. Thus, over the span of just 2 years, the triad of essential features of membranous glomerulonephritis were delineated. These are still the fundamental features used today to identify membranous glomerulonephritis, now called Membranoproliferative glomerulonephritis.

Classification

Classification of MPGN based on immunofluorescence microscopy is a result of all advances in the understanding of the pathogenesis of the disease. Based on this advanced techniques, there are three types of MPGN ;

• Immune-complex-mediated MPGN (Type I)
• Complement-mediated MPGN (Type II)
• Non-Ig/complement-mediated MPGN (Type III)

Pathophysiology

Causes

Differentiating Membranoproliferative glomerulonephritis from other Diseases

Epidemiology and Demographics

• Membranoproliferative glomerulonephritis (MPGN) is observed in 6-12% of US patients receiving renal biopsies to evaluate glomerular diseases. This entity accounts for 7% of children and 12% of adults with idiopathic nephrotic syndrome.
• MPGN causes a significant proportion of the cases of nephritis among patients in nonindustrialized countries. For example, in Mexico, MPGN accounts for 40% of all patients with nephritis. Most of these patients have type I disease; MPGN type II is uncommon. However, the incidence of MPGN type I is decreasing progressively in developed countries, which may be explained by a change in environmental factors, especially a decline in infections.
• In an investigation of the changing patterns of adult primary glomerular disease occurrence in a single region of the United Kingdom, Hanko analyzed the results of 1844 native renal biopsies taken between 1976 and 2005 (inclusive) and found the presence of primary glomerulonephritis was revealed in 49% of the biopsies, with the most common forms being immunoglobulin A (IgA) nephropathy (38.8%).
• Other common forms were membranous nephropathy (29.4%), minimal-change disease (MCD) (9.8%), MPGN type 1 (9.6%), and focal segmental glomerulosclerosis (FSGS) (5.7%). The incidence of IgA nephropathy increased significantly over the study period, whereas the occurrence of membranous nephropathy decreased.
• In the United States, MPGN predominantly affects the white population. Type I disease affects women more often than men, whereas a nearly equal sex distribution is seen in MPGN type II.
• The idiopathic forms of MPGN are more common in children and young adults (range, 6-30 y). Isolated reports of involvement in patients as young as 2 years and as old as 80 years are noted in the literature. Secondary types of MPGN predominate among adults.

Risk Factors

Risk Factor
Immune complex–mediated disease	Autoimmune Systemic lupus erythematosus (SLE), Sjögren syndrome Rheumatoid arthritis Inherited complement deficiencies (in particular, C2 deficiency) Scleroderma Celiac disease  Chronic infections Viral – Hepatitis B, hepatitis C, [12] cryoglobulinemia type II Bacterial – Endocarditis, infected ventriculoatrial (or jugular) shunt, multiple visceral abscesses, leprosy Protozoal – Malaria, schistosomiasis
thrombotic microangiopathies	Healing phase of hemolytic uremic syndrome (HUS) and/or thrombotic thrombocytopenic purpura (TTP) Syndromes of circulating antiphospholipid (anticardiolipin) antibodies Radiation nephritis Nephropathy associated with bone marrow transplantation Sickle cell anemia and polycythemia Transplant glomerulopathy
Paraprotein deposition diseases	Glomerulonephropathies associated with cryoglobulinemia type I Waldenström macroglobulinemia Immunotactoid glomerulopathy Immunoglobulin light-chain or heavy-chain deposition diseases Fibrillary glomerulonephritis Monoclonal gammopathy of unknown significance
Malignant neoplasms	Lymphoma Leukemia, and carcinoma are associated with a membranoproliferative pattern of renal injury.

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | Chest X Ray | CT | MRI | Echocardiography or Ultrasound | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1

Template:Nephrology

Template:WikiDoc Sources