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IgA Nephropathy

Title               

Ñ    IgA Nephropathy

Definition

Ñ    Common glomerular disease

Characterised by:

Ñ    Ig A deposition

Ñ    Mesangial proliferation

Pathogenesis

Causes

Ñ    Not known

Ñ    Thought to be a genetic or acquired defect in immune regulation

Ñ    Increased mucosal IgA secretion in response to inhaled or ingested allergens

Ñ    IgA aggregates or complexes deposited in the mesangium

Pathological Process

Ñ    Deposition of IgA demonstrated by immunofluorescence

Ñ    IgA is deposited in the mesangium as confluent masses or discrete granules

Ñ    Proliferation of mesangial cells

Results in:

Ñ    Mesangial proliferative nephropathy

Clinical Features

History

Age

Ñ    2^nd or 3^rd decade of life

Sex

Ñ    Males > Females

E&M

Ñ    Fever

Ñ    Lethargy

RS

Ñ    Sore throat

KUS

Ñ    Loin pain

Ñ    Haematuria

Examination

KUS

Ñ    Usually no physical signs

Rarely:

Ñ    Hypertension

Ñ    Nephrotic syndrome

Ñ    Nephritic syndrome

Ñ    Acute renal failure

Ñ    Chronic renal failure

Investigations

Fluids

Urine

Ñ    Haematuria

Ñ    Proteinuria

Tissue Diagnosis

Renal biopsy

Ñ    Mesangial proliferation

Ñ    IgA deposits in the mesangium:

Demonstrated by immunofluorescence

Management

Control

Drugs

Ñ    No drugs of proven benefit

Support

Renal failure:

Ñ    Conservative treatment

Ñ    Dialysis

Ñ    Transplantation

Biological Agents

Ñ    Transplantation may be offered in end stage renal failure

Ñ    Mesangial deposits occur in allografts

Ñ    Loss of graft function as a result is not common

Prognosis

Ñ    Approximately 20 % go on to develop renal failure 20 years from diagnosis

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