Rapidly progressive glomerulonephritis overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Rapidly progressive glomerulonephritis (RPGN) is a disease of kidney which occurs following severe damage to the kidneys and it can lead to rapid deterioration of kidney function in a few days.It is characterized by the presence of crescents in the glomeruli and hence is also called crescentic glomerulonephritis.
Patients with RPGN present with nephritic syndrome, but some may also have proteinuria.RPGN progresses to end stage renal disease if it is not treated in time.
RPGN is classified into three types, all of which involve immune-mediated damage to the glomeruli. In type I RPGN,, injury is caused by antibodies directed against the glomerular basement membrane. Type II RPGN accountsory is characterized by the deposition of immune complexes in the glomerulus. are type III, or pauci-immune RPGN, which features antibodies directed against neutrophils (anti-neutrophil cytoplasmic antibodies, ANCA).
Treatment depends on the underlying disease process. For example, plasmapheresis, corticosteroids, and cytotoxic drugs may promote recovery in Goodpasture syndrome, a cause of type I RPGN. Despite even early treatment, however, many patients with RPGN may ultimately require dialysis and possibly renal transplant.
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating rapildy progressive glomerulonephritis from Other Diseases
The various types of glomerulonephritides should be differentiated from each other based on associations, presence of pitting edema, hemeturia, hypertension, hemoptysis, oliguria, peri-orbital edema, hyperlipidemia, type of antibodies, light and electron microscopic features.
Epidemiology and Demographics
Risk Factors
Screening
There is insufficient evidence to recommend routine screening for rapidly progressive glomerulonephritis.
Natural History, Complications, and Prognosis
Diagnosis
Diagnostic Study of Choice
Determination of ANCAs can aid in the diagnosis, but positivity is not conclusive and negative ANCAs are not sufficient to reject the diagnosis. Cytoplasmic staining ANCAs that react with the enzyme proteinase 3 (cANCA) in neutrophils (a type of white blood cell) are associated with Granulomatosis with polyangiitis. If the patient has renal failure or cutaneous vasculitis, these are the most logical organs to obtain a biopsy from. Rarely, thoracoscopic lung biopsy is required.
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
Treatment of RPGN depends on the underlying disease process. For example, plasmapheresis, corticosteroids, and cytotoxic drugs may promote recovery in Goodpasture syndrome, a cause of type I RPGN. Despite even early treatment, however, many patients with RPGN may ultimately require dialysis and possibly renal transplant.
Surgery
Surgery is not the first-line treatment option for patients with rapidly progressive glomerulonephritis. Renal transplantation is usually reserved for patients who present with undetectable circulating anti-glomerular basement antibodies in serum for 12 months and at least 6 months after stopping the use of cytotoxic agents.
Primary Prevention
There are no established measures for the primary prevention of Rapidly progressive glomerulonephritis.
Secondary Prevention
There are no established measures for the secondary prevention of Rapidly progressive glomerulonephritis.