Membranoproliferative glomerulonephritis overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Ali Poyan Mehr, M.D. [2] Associate Editor(s)-in-Chief: Olufunmilola Olubukola M.D.[3]
Overview
Membranoproliferative glomerulonephritis or MPGN, also called mesangiocapillary glomerulonephritis is a type of glomerulonephritis caused by immune complexes depositing in the kidney glomerular mesangium and basement membrane (GBM), these immune complexes activate complement resulting in damaging the glomeruli. The GBM is rebuilt ontop of the deposits, and shows "tram-tracking" appearance under the microscope. 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 basis of 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. The treatment of membranoproliferative glomerulonephritis (MPGN) needs to address the underlying cause of the MPGN, eg, infection. The factors that predict renal prognosis should be assessed, and finally immunosuppressive drugs can be used when the underlying cause is autoimmune diseases.
Historical Perspective
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 [1]:Immune-complex-mediated MPGN (Type I) ,Complement-mediated MPGN (Type II), Non-Ig/complement-mediated MPGN (Type III)
Pathophysiology
MPGN membrano proliferative glomerulonephritis, also known as mesangiocapillary glomerulonephritis, is a glomerular injury on light microscopy that is characterized by mesangial hypercellularity, endocapillary proliferation, and double-contour formation along the glomerular capillary walls. "MPGN" includes two characteristic histologic changes:Thickened glomerular basement membrane (GBM) due to deposition of immune complexes and/or complement factors, intrusion of the mesangial cells and other cellular elements between the glomerular basement membrane and the endothelial cells, and new basement membrane formation. Mesangial and endocapillary cellularity is increased resulting in lobular appearance of the glomerular tuft. Proliferation of mesangial cells and circulating monocytes results in increased cellularity.Two mechanisms are involved in the pathogenesis of MPGN,Immune complex deposition leading to activation of complement (immune complex-mediated) and dysregulation and persistent activation of the alternative complement pathway.
Causes
Differentiating membrano proliferative glomerulonephritis from Other Diseases
There are some differential diagnosis mentioned for MPGN as they have some symptoms and signs in shared, the most relevant diseases are acute glomerulonephritis, IgA Nephropathy, LupusNephritis, poststreptococcal glomerulonephritis, and rapidly progressive Glomerulonephritis (RPGN).
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications, and Prognosis
Diag nosis
Diagnostic Study of Choice
History and Symptoms
Physical Examination
Laboratory Findings
Electrocardiogram
X-ray
Echocardiography and Ultrasound
CT scan
MRI
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Interventions
Surgery
Primary Prevention
Secondary Prevention
References
- ↑ Sethi S, Zand L, Leung N, Smith RJ, Jevremonic D, Herrmann SS; et al. (2010). "Membranoproliferative glomerulonephritis secondary to monoclonal gammopathy". Clin J Am Soc Nephrol. 5 (5): 770–82. doi:10.2215/CJN.06760909. PMC 2863981. PMID 20185597.