Rapidly progressive glomerulonephritis
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Rapidly progressive glomerulonephritis
Renal system
Renal and ureteral disorders
Renal agenesis
Horseshoe kidney
Potter sequence
Hyperphosphatemia
Hypophosphatemia
Hypernatremia
Hyponatremia
Hypermagnesemia
Hypomagnesemia
Hyperkalemia
Hypokalemia
Hypercalcemia
Hypocalcemia
Renal tubular acidosis
Minimal change disease
Diabetic nephropathy
Focal segmental glomerulosclerosis (NORD)
Amyloidosis
Membranous nephropathy
Lupus nephritis
Membranoproliferative glomerulonephritis
Poststreptococcal glomerulonephritis
Goodpasture syndrome
Rapidly progressive glomerulonephritis
IgA nephropathy (NORD)
Lupus nephritis
Alport syndrome
Kidney stones
Hydronephrosis
Acute pyelonephritis
Chronic pyelonephritis
Prerenal azotemia
Renal azotemia
Acute tubular necrosis
Postrenal azotemia
Renal papillary necrosis
Renal cortical necrosis
Chronic kidney disease
Polycystic kidney disease
Multicystic dysplastic kidney
Medullary cystic kidney disease
Medullary sponge kidney
Renal artery stenosis
Renal cell carcinoma
Angiomyolipoma
Nephroblastoma (Wilms tumor)
WAGR syndrome
Beckwith-Wiedemann syndrome
Bladder and urethral disorders
Posterior urethral valves
Hypospadias and epispadias
Vesicoureteral reflux
Bladder exstrophy
Urinary incontinence
Neurogenic bladder
Lower urinary tract infection
Transitional cell carcinoma
Non-urothelial bladder cancers
Renal system pathology review
Congenital renal disorders: Pathology review
Renal tubular defects: Pathology review
Renal tubular acidosis: Pathology review
Acid-base disturbances: Pathology review
Electrolyte disturbances: Pathology review
Renal failure: Pathology review
Nephrotic syndromes: Pathology review
Nephritic syndromes: Pathology review
Urinary incontinence: Pathology review
Urinary tract infections: Pathology review
Kidney stones: Pathology review
Renal and urinary tract masses: Pathology review
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Rapidly progressive glomerulonephritis
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Rapidly progressive glomerulonephritis
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Chest x-ray reveals bilateral nodular densities. Serum c-ANCA is positive. Renal biopsy is shown:
Which of the following additional findings are most likely to be found on further evaluation?
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Crescentic glomerulonephritis p. 620
Hypersensitivity reaction (type II)
rapidly progressive glomerulonephritis p. 622
Rapidly progressive glomerulonephritis (RPGN) p. 622
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Content Reviewers
Rishi Desai, MD, MPHContributors
Tanner Marshall, MS
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.
Summary
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)
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