Rapidly progressive glomerulonephritis

Rapidly progressing glomerulonephritiscrescentsglomerular crescentprimary pauci-immune necrotizing crescentic glomerulonephritisRPGN
Rapidly progressive glomerulonephritis (RPGN) is a syndrome of the kidney that is characterized by a rapid loss of kidney function, (usually a 50% decline in the glomerular filtration rate (GFR) within 3 months) with glomerular crescent formation seen in at least 50% or 75% of glomeruli seen on kidney biopsies.wikipedia
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Granulomatosis with polyangiitis

Wegener's granulomatosiscoin lesion, pulmonary
In 50% of cases, RPGN is associated with an underlying disease such as Goodpasture syndrome, systemic lupus erythematosus or granulomatosis with polyangiitis; the remaining cases are idiopathic. In most cases of the latter, the systemic disease is an ANCA-associated vasculitis such as granulomatosis with polyangiitis, microscopic polyangiitis or eosinophilic granulomatosis with polyangiitis.

Nephrotic syndrome

a serious kidney disorderGlomerulosclerosisidiopathic nephrotic syndrome
Most types of RPGN are characterized by severe and rapid loss of kidney function featuring severe hematuria (blood in the urine), red blood cell casts in the urine, and proteinuria (protein in the urine), sometimes exceeding 3 g protein/24 h, a range associated with nephrotic syndrome.

Crescent

crescent mooncrescentsMoon
Regardless of the underlying cause, RPGN involves severe injury to the kidneys' glomeruli, with many of the glomeruli containing characteristic glomerular crescents (crescent-shaped scars).
The term crescent may also refer to objects with a shape reminiscent of the crescent shape, such as houses forming an arc, a type of solitaire game, Crescent Nebula, glomerular crescent (crescent shaped scar of the glomeruli of the kidney)., the Fertile Crescent (the fertile area of land between Mesopotamia and Egypt roughly forming a crescent shape), and the croissant (the French form of the word) for the crescent-shaped pastry.

IgA nephropathy

Berger's diseaseBerger diseaseglomerulonephritis, iga
These diseases include systemic lupus erythematosus, acute proliferative glomerulonephritis, Henoch–Schönlein purpura, and IgA nephropathy.

Anti-neutrophil cytoplasmic antibody

ANCAanti-neutrophil cytoplasmic antibodiesantineutrophil cytoplasmic antibodies
In the pathophysiology of rapidly progressive glomerulonephritis the antineutrophil cytoplasmic antibodies (ANCA) interact with antigens in cytoplasm of neutrophils. The presence of anti-Glomerular basement membrane (GBM) antibodies suggests type I RPGN; antinuclear antibodies (ANA) may support a diagnosis of systemic lupus erythematosus and type II RPGN; and type III and idiopathic RPGN are frequently associated with anti-neutrophil cytoplasmic antibodies (ANCA)-positive serum.
ANCAs are associated with small vessel vasculitides including granulomatosis with polyangiitis, microscopic polyangiitis, primary pauci-immune necrotizing crescentic glomerulonephritis (a type of renal-limited microscopic polyangiitis), eosinophilic granulomatosis with polyangiitis and drug induced vasculitides.

Microscopic polyangiitis

In most cases of the latter, the systemic disease is an ANCA-associated vasculitis such as granulomatosis with polyangiitis, microscopic polyangiitis or eosinophilic granulomatosis with polyangiitis.
Rapidly progressive glomerulonephritis may occur.

Syndrome

syndromesgenetic syndromessyndromic
Rapidly progressive glomerulonephritis (RPGN) is a syndrome of the kidney that is characterized by a rapid loss of kidney function, (usually a 50% decline in the glomerular filtration rate (GFR) within 3 months) with glomerular crescent formation seen in at least 50% or 75% of glomeruli seen on kidney biopsies.

Kidney

kidneysrenalkidney disorder
Rapidly progressive glomerulonephritis (RPGN) is a syndrome of the kidney that is characterized by a rapid loss of kidney function, (usually a 50% decline in the glomerular filtration rate (GFR) within 3 months) with glomerular crescent formation seen in at least 50% or 75% of glomeruli seen on kidney biopsies.

Renal function

glomerular filtration ratekidney functioncreatinine clearance
Rapidly progressive glomerulonephritis (RPGN) is a syndrome of the kidney that is characterized by a rapid loss of kidney function, (usually a 50% decline in the glomerular filtration rate (GFR) within 3 months) with glomerular crescent formation seen in at least 50% or 75% of glomeruli seen on kidney biopsies.

Acute kidney injury

acute renal failureacute kidney failureuremic poisoning
If left untreated, it rapidly progresses into acute kidney failure and death within months.

Goodpasture syndrome

Goodpasture's syndromeanti-glomerular basement membrane diseaseAnti-Glomerular Basement Membrane nephritis
In 50% of cases, RPGN is associated with an underlying disease such as Goodpasture syndrome, systemic lupus erythematosus or granulomatosis with polyangiitis; the remaining cases are idiopathic.

Systemic lupus erythematosus

lupusSLEsystemic lupus erythematosus (SLE)
In 50% of cases, RPGN is associated with an underlying disease such as Goodpasture syndrome, systemic lupus erythematosus or granulomatosis with polyangiitis; the remaining cases are idiopathic. These diseases include systemic lupus erythematosus, acute proliferative glomerulonephritis, Henoch–Schönlein purpura, and IgA nephropathy. The presence of anti-Glomerular basement membrane (GBM) antibodies suggests type I RPGN; antinuclear antibodies (ANA) may support a diagnosis of systemic lupus erythematosus and type II RPGN; and type III and idiopathic RPGN are frequently associated with anti-neutrophil cytoplasmic antibodies (ANCA)-positive serum.

Idiopathic disease

idiopathiccryptogenicunknown
In 50% of cases, RPGN is associated with an underlying disease such as Goodpasture syndrome, systemic lupus erythematosus or granulomatosis with polyangiitis; the remaining cases are idiopathic.

Glomerulus

glomeruliglomerularglomerular filtrate
Regardless of the underlying cause, RPGN involves severe injury to the kidneys' glomeruli, with many of the glomeruli containing characteristic glomerular crescents (crescent-shaped scars).

Scar

scarringscar tissuescars
Regardless of the underlying cause, RPGN involves severe injury to the kidneys' glomeruli, with many of the glomeruli containing characteristic glomerular crescents (crescent-shaped scars).

Hematuria

blood in the urinehaematuriabloody urine
Most types of RPGN are characterized by severe and rapid loss of kidney function featuring severe hematuria (blood in the urine), red blood cell casts in the urine, and proteinuria (protein in the urine), sometimes exceeding 3 g protein/24 h, a range associated with nephrotic syndrome.

Urinary cast

urinary castscastsRBC casts
Most types of RPGN are characterized by severe and rapid loss of kidney function featuring severe hematuria (blood in the urine), red blood cell casts in the urine, and proteinuria (protein in the urine), sometimes exceeding 3 g protein/24 h, a range associated with nephrotic syndrome.

Proteinuria

protein in the urineproteinIsolated proteinuria
Most types of RPGN are characterized by severe and rapid loss of kidney function featuring severe hematuria (blood in the urine), red blood cell casts in the urine, and proteinuria (protein in the urine), sometimes exceeding 3 g protein/24 h, a range associated with nephrotic syndrome.

Hypertension

high blood pressurehypertensivearterial hypertension
Some patients also experience hypertension (high blood pressure) and edema.

Edema

dropsyoedemaswelling
Some patients also experience hypertension (high blood pressure) and edema.

Oliguria

Decreased urine outputlow urine outputoliguric
Severe disease is characterized by pronounced oliguria or anuria, which portends a poor prognosis.

Anuria

No urine productionabsence of urine formationabsent
Severe disease is characterized by pronounced oliguria or anuria, which portends a poor prognosis.

Degranulation

degranulatedegranulatingmast cell degranulation
It is thought that ANCA causes an early degranulation giving way to release of lytic enzymes at site of injury.

Serology

serologicalserologicserologist
Serum analysis often aids in the diagnosis of a specific underlying disease.

Glomerular basement membrane

GBMbasement membraneglomerular basement membranes
The presence of anti-Glomerular basement membrane (GBM) antibodies suggests type I RPGN; antinuclear antibodies (ANA) may support a diagnosis of systemic lupus erythematosus and type II RPGN; and type III and idiopathic RPGN are frequently associated with anti-neutrophil cytoplasmic antibodies (ANCA)-positive serum.