Rapidly progressive glomerulonephritis
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Rapidly progressive glomerulonephritis
Paediatrics Sara
Paediatrics Sara
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Crescentic glomerulonephritis, which is sometimes called rapidly progressive glomerulonephritis, is a type of nephritic syndrome, meaning it involves inflammation of the kidney’s glomeruli.
This inflammation ultimately causes a proliferation of cells in the Bowman’s space, which forms a crescent shape and this change leads to renal failure relatively quickly—within weeks to months.
The development of crescents in Bowman’s space can happen in several ways.
In some cases it’s the idiopathic, meaning there’s no identifiable cause.
When the cause is identifiable, though, it can be split into several types.
Type I is caused by anti-glomerular basement membrane, or GBM, antibodies, antibodies that target the GBM.
Type I is associated with Goodpasture syndrome, which also involves pulmonary hemorrhages.
Type II is immune-complex-mediated, meaning caused by immune complexes, composed of antigens and antibodies.
This might be the case with poststreptococcal glomerulonephritis, systemic lupus erythematosus, IgA nephropathy, and Henoch-Schonlein purpura.
And finally type III is known as pauci-immune, meaning little or no anti-GBM antibodies or immune-complex deposits.
In these cases, often anti-neutrophilic cytoplasmic antibodies or ANCAs are in the blood, which are autoantibodies against the body’s own neutrophils.
Furthermore, you can break it down into the type of ANCA. C-ANCAs, or cytoplasmic ANCAs, are associated with Wegener granulomatosis.
P-ANCAs or perinuclear ANCAs on the other hand are associated with microscopic polyangiitis and Churg-Strauss syndrome, the latter of which can be distinguished by having granulomatous inflammation, which is when immune cells attempt to wall off a substance perceived as foreign, asthma, and eosinophilia
Whatever the underlying cause is, the common feature is severe glomerular injury and the development of a crescent shape.
Typically, as a result of cell-mediated immunity as well as macrophage involvement, the glomerular basement membrane breaks.
Key Takeaways
Rapidly progressive glomerulonephritis (RPGN) is a type of kidney disease characterized by a rapid loss of renal function, with characteristic glomerular crescent-shaped scars seen on renal biopsies. It is caused by inflammation and damage to the glomeruli, which can result from conditions like infections, immune disorders, and vasculitis.
Symptoms of RPGN can include blood in the urine, swelling in the legs and face, high blood pressure, and decreased urine output. The condition can progress rapidly, leading to kidney failure if left untreated. Treatment options may include immunosuppressive drugs to reduce inflammation, as well as a specific treatment for the underlying cause of the condition. If the renal failure becomes irreversible, dialysis or a kidney transplant are required.
Sources
- "Robbins Basic Pathology" Elsevier (2017)
- "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
- "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
- "CURRENT Medical Diagnosis and Treatment 2020" McGraw-Hill Education / Medical (2019)
- "What is new in the management of rapidly progressive glomerulonephritis?" Clinical Kidney Journal (2015)
- "ANCA Glomerulonephritis and Vasculitis" Clinical Journal of the American Society of Nephrology (2017)
- "Incidence and outcome of pauci‐immune rapidly progressive glomerulonephritis in Wessex, UK: a 10‐year retrospective study" Nephrology Dialysis Transplantation (2000)