Glomerulopathy manifested by nephrotic syndrome
This group of diseases is mainly manifested by proteinuria or nephrotic syndrome, which includes proteinuria, generalized edema, hyperlipidemia, and hypoalbuminemia.
Minimal Change Disease
Membranous glomerulopathy
Membranous glomerulonephritis (MGN; also membranous nephropathy, MN) mainly affects middle-aged or older adults. Most often (70-80% of cases) it is a primary idiopathic autoimmune disease, 20-30% of the disease is secondary to infectious, tumor, systemic autoimmune diseases or after the administration of certain drugs[1]. MGN is the most common cause of nephrotic syndrome in adults (20-40%), more common in men.
Microscopically we find a diffuse thickening of the glomerular capillary wall caused by the deposition of immunocomplexes into the subepithelial space (between the podocytes and the basement membrane).
Etiology
- Idiopathic (primary) form of MGN (most common)
- Most diseases of this group are associated with the formation of autoantibodies against the M-type receptor for phospholipase A2 (PLA2R). Some polymorphisms of the PLA2R gene in combination with some HLA-DQA1[1] polymorphisms are associated with a high risk of this form of the disease.
- Secondary form of MGN (20-30%)
Clinical picture
Clinical manifestations may be unremarkable.
- sudden appearance of DKK swellings + their progression
- non-selective proteinuria + erythrocyturia, often fully developed nephrotic syndrome
- arterial hypertension (20-40%)
- impairment of renal function (at the time of dg. in 5-10%)
Therapy
- Idiopathic MGN: corticoids, cyclophosphamide, chlorambucil, cyclosporine,
- secondary MGN: stop precipitating drugs / treat primary disease.
Prognosis
- The fundamental importance of influencing the formation of immunocomplexes,
- with successful therapy, nephrotic syndrome may disappear,
- many years stationary or developing CKD.
Kidney amyloidosis
Links
Related Articles
- Glomerulopathy
- Glomerulonephritis: Acute glomerulonephritis • Rapidly progressive glomerulonephritis • Chronic glomerulonephritis
- Nephrotic syndrome
- Amyloidosis
- Kidneys
- Diabetic nephropathy
References
- PROMOTED, Ctibor. Special Pathology. 2nd part. 3. edition. Prague : Karolinum, 2004. ISBN 80-7184-484-5.
- TEPLAN, Vladimir. Nephrology minimum for clinical practice. 1. edition. Prague : Mladá fronta, 2013. ISBN 978-80-204-2881-3.
- CHILD, P.. Internal medicine. 2. edition. Prague : Galen, 2007. ISBN 978-80-7262-496-6.
- RED, Romana. Secondary glomerulonephritis [lecture for subject Nephrology, specialization General medicine, 1.LF Charles University in Prague]. Prague. 2011-01-07.
- ↑ a b FLOEGE, Jürgen – AMANN, Kerstin. Primary glomerulonephritides. The Lancet. 2016, y. 10032, vol. 387, p. 2036-2048, ISSN 0140-6736. DOI: 10.1016/s0140-6736(16)00272-5.
- ↑ LEEAPHORN, Napat – KUE-A-PAI, Pogsathorn – THAMCHAROEN, Natanong. Prevalence of Cancer in Membranous Nephropathy: A Systematic Review and Meta-Analysis of Observational Studies. American Journal of Nephrology. 2014, y. 1, vol. 40, p. 29-35, ISSN 1421-9670. DOI: 10.1159/000364782.
