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Rapidly Progressive Crescentic Glomerulonephritis K018

Document
Last amended 
29 June 2015
Current RMA Instruments:
Reasonable Hypothesis SOP
81 of 2010
Balance of Probabilities SOP
82 of 2010
Changes from Previous Instruments:

SOP Bulletin 146

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

This is a complex form of kidney disease.  The SOP applies only when there is both the required histological evidence (from renal biopsy) and the correct clinical pattern of renal failure.  See comments below for further detail.

Is specific diagnostic evidence required to apply the SOP? – Yes.

This diagnosis is made by a specialist nephrologist on the basis of the histopathology on a renal biopsy as well as serial renal function testing. It cannot be made clinically without investigations.

Are there sub-factors that require specific information? – No.
Additional diagnoses that may be covered by SOP
  • Nephritic syndrome
  • Progressive renal failure
  • Glomerulonephritis
  • Mesangial IgA nephropathy (SOPs 63 & 64 of ‘01). A person who suffers ‘Mesangial IgA nephropathy’ may or may not progress to ‘rapidly progressive crescentic glomerulonephritis’. If there is progression to this pathology, the type will generally be the Immune Complex type.
  • Lupus nephropathy being a component manifestation of Systemic Lupus Erythematosus (SOPs 41 & 42 of ‘09. A person who suffers ‘Lupus nephropathy’ may or may not progress to ‘rapidly progressive crescentic glomerulonephritis’. If there is progression to this pathology, the type will generally be the Immune Complex type.
Conditions excluded from SOP
  • Hypertensive renal disease
  • Diabetic nephropathy
  • Atherosclerotic nephropathy
Unconfirmed diagnosis

If, after applying the above information, you are unable to confirm the diagnosis, you should then:

  1. seek medical officer advice about further investigation, or;
  2. generate a diagnostic report, or;
  3. re-encode the condition, if appropriate.

The following may be useful in establishing the diagnosis:

  • Report from nephrologist.
Comments

This is not a single disease entity but a group of different diseases.

This group of kidney diseases are characterised by a pathology involving the renal glomeruli associated with an acute clinical presentation with progressive decline in renal function over a short period of time (weeks or months).

The label ‘rapidly progressive glomerulonephritis’ refers to the clinical pattern of the disease and the label ‘crescentic glomerulonephritis’ refers to the histological appearance of the diseased tissue when viewed under a microscope. The ‘crescent’ is the abnormal presence of two layers of cells within the Bowman’s capsule of the glomerulus.

The clinical picture normally is that of a ‘nephritic syndrome’ with casts of protein and blood in the urine; and may also have hypertension and pitting oedema.

The decline in renal function is not trivial (50% in 3 months) nor is the involvement of the glomeruli by crescent formation which can involve 50-75% of the glomeruli. The SOP does not numerically specify a required threshold of glomeruli involvement but provides the adjectival qualifier of ‘extensive’.

Note that both the histological pattern of widespread (extensive) crescents must be present on renal histology AND there must be a rapidly progressive pattern of clinical decline in renal function. Note that there are patients who have abnormal renal function but not extensive numbers of crescents; and there are patients who have crescents on histology but not a rapidly progressive renal decline; neither of which circumstances would meet the requirements of this statement of principles.

The group of rapidly progressive crescentic glomerulonephritis includes:

  1. Anti-glomerular basement membrane type – Goodpasture’s disease.
  2. Immune complex type – Histology shows immune deposits from different immune sources such as Mesangial IgA, anti-streptococcal antibodies, antinuclear antibodies, anti-neutrophil cytoplasmic antibody.
  3. Pauci immune type – Histology shows few or no immune deposits
  4. Double antibody positive disease type – Has features of both Anti-glomerular basement membrane type and the Immune complex type.
  5. Idiopathic