Membranoproliferative glomerulonephritis
Classification according to ICD-10 | |
---|---|
N00.5 | Diffuse Mesangiocapillary Glomerulonephritis - Membranoproliferative Glomerulonephritis, Types I and III, or onA |
N00.6 | Dense Deposit Disease - Membranoproliferative Glomerulonephritis, Type II |
ICD-10 online (WHO version 2019) |
The membranoproliferative glomerulonephritis (MPGN, mesangiocapillary glomerulonephritis, lobular glomerulonephritis) is a rare disease of the renal corpuscle (glomerulus) . The idiopathic form (with no known cause) occurs most often between the ages of 8 and 30 years.
Symptoms
Symptoms are
- Incidence of red blood cells in the urine (hematuria) , which in the phase contrast microscope characteristic deformations (dysmorphic erythrocytes acanthocytes ) show and erythrocytes cylinder may form.
- Detection of protein in the urine (proteinuria) , protein excretion is usually moderate and is less than 1.5 g / d
- Processes with nephritic syndrome ( hematuria , high blood pressure and impaired kidney function ) or nephrotic syndrome ( proteinuria > 3.5 g / d, water retention (edema) and lipid metabolism disorders ) are possible
diagnosis
The diagnosis is made by histological examination of a tissue sample from the kidney.
Examination under a light microscope reveals:
- a thickening of the basement membrane of the kidney corpuscle due to the storage of immune complexes ,
- the penetration of mesangial cells into the gap between the basement membrane and the endothelium ,
- an increased cell content through proliferation of mesangial cells and immigration of monocytes from the bloodstream.
Classification
On the basis of electron microscopic findings, three types can be distinguished:
Type I membranoproliferative glomerulonephritis
In type I , immune complexes are found in the mesangium and the gap between the endothelium and the basement membrane , which lead to a double contouring of the basement membrane (tram track sign, streetcar rail). Type I is the most common form of membranoproliferative glomerulonephritis. The disease can occur without a demonstrable cause (idiopathic) or as a result of diseases that lead to the formation of circulating immune complexes that are deposited in the kidney, e.g. B. Hepatitis C with or without cryoglobulinemia , systemic lupus erythematosus , chronic hepatitis B , subacute bacterial endocarditis and infected ventriculoatrial shunt .
Membranoproliferative Glomerulonephritis Type II (Dense Deposit Disease)
In type II , continuous band-like deposits are found within the basement membrane of kidney corpuscles (glomeruli) , kidney tubules (tubules) and Bowmann's capsule of the kidney corpuscle. In immunofluorescence microscopy, complement factor C3 , but not immunoglobulin, can be detected in the deposits .
Membranoproliferative glomerulonephritis type III
In type III , as in type I, there are immune complex deposits between the endothelium and basement membrane as well as a double contouring of the basement membrane. In addition, immune depots between the basement membrane and podocytes (as in epimembranous glomerulonephritis ) can be detected.
Web links
- Membranoproliferative Glomerulonephritis Pathology - Pathopic image database of the University of Basel ( PathoPic - Instructions ; PDF; 2.2 MB)
- Agnes Fogo: Membranoproliferative Glomerulonephritis . In: Am J Kidney Dis . No. 31 , 1998, pp. E1 ( entry ).
- Agnes Fogo: Dense Deposit Disease . In: Am J Kidney Dis . No. 31 , 1998, pp. E1 ( entry ).