Rapidly progressing glomerulonephritis

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Rapidly progressing glomerulonephritis
ICD-10	N00-N08 with .7 suffix
DiseasesDB	3165
eMedicine	med/881  med/890

Rapidly Progressing Glomerulonephritis (RPGN, also known as Crescentic Glomerulonephritis) is a form of acute Glomerulonephritis that results in severe injury to kidney glomerulus. The damage inflicted causes leakage of plasma proteins into the glomerulus which stimulate parietal epithelial cells lining Bowman's space along with infiltrating monocytes to form a crescent-shaped scar. As more and more glomeruli become disfunctional due to scarring, other glomeruli become larger (hypertrophy) to pick up the slack and maintain the glomerular filtration rate. This stresses the remaining glomeruli, causing them to become damaged, leading to a downwards spiral into acute renal failure.

Types of RPGN

Type I RPGN - Anti-GBM Disease

RPGN caused by auto-antibodies to glomerular basement membrane (GBM), possible the noncollagenous domain of Type IV collagen. If there is concurrent pulmonary alveolar disease, the disease is known as Goodpasture's syndrome.

Type II RPGN - Immune Complex Mediated Disease

RPGN caused by the glomerular deposition of massive amounts of immune complexes, usually generated by sever bouts of other diseases such as SLE, post-strep glomerulonephritis, MPGN, or IgA nephropathy.

Type III RPGN - Pauci-Immune Disease

Also known as non-immune deposit (NID) RPGN, is a type of RPGN that does not show glomerular deposition of antibodies or immune complexes. Instead, the glomeruli are damaged in an undefined manner, perhaps through the activation of PMNs in response to Anti-Neutrophil Cytoplasmic Antibodies (ANCA), releasing free radicals. This type of RPGN can be isolated, or associated with vascular diseases such as Microscopic polyangiitis, Wegener's granulomatosis, or Churg-Strauss syndrome.

Aetiology and Pathogenesis

• 1. may follow acute diffuse proliferative GN. This is more common in adults
• 2. Idiopathic (unknown cause)
• 3. RPGN may occur in association with systemic diseases e.g. SLE, PAN, Scleroadenoma Honoch Sholein purpura and Goodpasture's syndrome.

Clinical Picture

• 1. Severe Oliguria or Anuria
• 2. Proteinuria is mild or moderate
• 3. Haematuria
• 4. Hypertention and oedema

When the cause is Goodpasture's syndrome or vasculitic disease (such as Wegener's granulomatosis), the patient may also get lung and even upper airway involvement, with: cough, haemoptysis, dyspnoea, nasal bleeding/obstruction/sinusitis.

Fate

• 1. It is rapidly progressive and fatal due to acute renal failure or hypertension
• 2. Chronic glomerulonephritis