Rapidly Progressive Crescentic Glomerulonephritis K018

Current RMA Instruments

Changes from previous Instruments

ICD Coding

• ICD-9-CM Codes: 583.4
• ICD-10-AM Codes: N01.9

Brief description

Rapidly progressive crescentic glomerulonephritis is a syndrome (i.e. not a single disease) affecting the glomeruli in the kidneys, which results in inflammation and rapid (and often severe) loss of renal function over a period of days to months. Histology (from renal biopsy) shows a characteristic pattern of extensive crescent formation around glomeruli (within Bowman's space).  The condition may develop in someone with a pre-exisiting kidney disease, such as Mesangial IgA nephropathy or lupus nephropathy.

Confirming the diagnosis

This condition typically manifests clinically with nephritic syndrome, which is a non-specific presentation (protein and blood in the urine, hypertension, increased blood urea and creatinine).  The condition can't be diagnosed on clinical grounds. Urinalysis and blood testing will be undertaken.  A renal biopsy is typically required to confirm the diagnosis.

The relevant medical specialist is a nephrologist.

Additional diagnoses that may be covered by SOP (more information required)

• Nephritic syndrome
• Progressive renal failure
• Glomerulonephritis

Conditions excluded from SOP

• Hypertensive renal disease
• Diabetic nephropathy
• Atherosclerotic nephropathy

Clinical onset

The usual presentation is with acute onset of macroscopic hematuria (visible blood in the urine), decreased urine output, hypertension, and edema (swelling, e.g. of the ankles).

Clinical worsening

The only SOP worsening factor is for inability to obtain appropriate clinical management.  The course of the condition is variable, but if untreated, the condition may progress rapidly.  Urgent diagnosis and appropriate medical therapy can minimise the extent of irreversible renal damage.