Rapidly progressive glomerulonephritis overview: Difference between revisions
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==Overview== | ==Overview== | ||
'''Rapidly progressive glomerulonephritis''' ('''RPGN''') is a [[syndrome]] of the [[kidney]] that, if left untreated, rapidly progresses into [[acute renal failure]] and death within months. In 50% of cases, RPGN is associated with an underlying disease such as [[Goodpasture syndrome]], [[systemic lupus erythematosus]], or [[Wegener granulomatosis]]; the remaining cases are [[idiopathic]]. Regardless of the underlying cause, RPGN involves severe injury to the kidney's [[glomeruli]], with greater than 50% of the glomeruli containing characteristic crescent-shaped [[scar]]s. Because of this microscopic feature, RPGN is also called '''[[crescentic glomerulonephritis'''''. | '''Rapidly progressive glomerulonephritis''' ('''RPGN''') is a [[syndrome]] of the [[kidney]] that, if left untreated, rapidly progresses into [[acute renal failure]] and death within months. In 50% of cases, RPGN is associated with an underlying disease such as [[Goodpasture syndrome]], [[systemic lupus erythematosus]], or [[Wegener granulomatosis]]; the remaining cases are [[idiopathic]]. Regardless of the underlying cause, RPGN involves severe injury to the kidney's [[glomeruli]], with greater than 50% of the glomeruli containing characteristic crescent-shaped [[scar]]s. Because of this microscopic feature, RPGN is also called '''[[crescentic glomerulonephritis]]'''''. | ||
Patients with RPGN have blood in the urine ([[hematuria]]), urinary protein ([[proteinuria]]), and occasionally high blood pressure ([[hypertension]]) and [[edema]]. The clinical picture is consistent with [[nephritic syndrome]], although the degree of proteinuria may occasionally exceed 3 [[gram|g]]/24 | Patients with RPGN have blood in the urine ([[hematuria]]), urinary protein ([[proteinuria]]), and occasionally high blood pressure ([[hypertension]]) and [[edema]]. The clinical picture is consistent with [[nephritic syndrome]], although the degree of proteinuria may occasionally exceed 3 [[gram|g]]/24 hr, a range associated with [[nephrotic syndrome]]. Untreated disease may progress to decreased urinary volume ([[oliguria]]), which is associated with poor kidney function. | ||
RPGN is classified into three types, all of which involve immune-mediated damage to the glomeruli. In type I RPGN, which accounts for approximately 20% of RPGN cases, injury is caused by [[antibodies]] directed against the [[glomerular basement membrane]]. Type II RPGN accounts for roughly 25% of RPGN cases and is characterized by the deposition of [[immune complex]]es in the glomerulus. The remainder of RPGN cases are type III, or pauci-immune RPGN, which features antibodies directed against [[neutrophil]]s ([[anti-neutrophil cytoplasmic antibodies]], ANCA). | RPGN is classified into three types, all of which involve immune-mediated damage to the glomeruli. In type I RPGN, which accounts for approximately 20% of RPGN cases, injury is caused by [[antibodies]] directed against the [[glomerular basement membrane]]. Type II RPGN accounts for roughly 25% of RPGN cases and is characterized by the deposition of [[immune complex]]es in the glomerulus. The remainder of RPGN cases are type III, or pauci-immune RPGN, which features antibodies directed against [[neutrophil]]s ([[anti-neutrophil cytoplasmic antibodies]], ANCA). | ||
Revision as of 13:17, 28 September 2012
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Rapidly progressive glomerulonephritis (RPGN) is a syndrome of the kidney that, if left untreated, rapidly progresses into acute renal failure and death within months. In 50% of cases, RPGN is associated with an underlying disease such as Goodpasture syndrome, systemic lupus erythematosus, or Wegener granulomatosis; the remaining cases are idiopathic. Regardless of the underlying cause, RPGN involves severe injury to the kidney's glomeruli, with greater than 50% of the glomeruli containing characteristic crescent-shaped scars. Because of this microscopic feature, RPGN is also called crescentic glomerulonephritis.
Patients with RPGN have blood in the urine (hematuria), urinary protein (proteinuria), and occasionally high blood pressure (hypertension) and edema. The clinical picture is consistent with nephritic syndrome, although the degree of proteinuria may occasionally exceed 3 g/24 hr, a range associated with nephrotic syndrome. Untreated disease may progress to decreased urinary volume (oliguria), which is associated with poor kidney function.
RPGN is classified into three types, all of which involve immune-mediated damage to the glomeruli. In type I RPGN, which accounts for approximately 20% of RPGN cases, injury is caused by antibodies directed against the glomerular basement membrane. Type II RPGN accounts for roughly 25% of RPGN cases and is characterized by the deposition of immune complexes in the glomerulus. The remainder of RPGN cases 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.