IgA nephropathy K017

Current RMA Instruments

Changes from previous Instruments

 ICD Coding

• ICD-9-CM Codes: 583.9
• ICD-10-AM Codes: N05.9

Brief description

This is an autoimmune kidney disorder.  It involves the deposition of immunoglobulin A (plus or minus IgG and IgM) in the mesangium. The mesangium is a layer of tissue/cells in the glomeruli in the kidney.  A glomerulus is part of a filtration unit in the kidney, called a nephron, which is integral to the production of urine and which is the structural and functional unit of the kidney.  The condition results in glomerulonephritis, which is inflammation of the glomeruli/nephrons.   

Confirming the diagnosis

This diagnosis can only be made following a renal biopsy, with a pathologist providing a histological report (including immunofluorescence or immunoperoxidase studies). 

The relevant medical specialist is a nephrologist.

Additional diagnoses covered by SOP

• Berger’s disease
• Mesangial IgA glomerulonephritis

Conditions excluded from SOP

• Nephritic syndrome – Too non-specific but this clinical picture may arise from IgA nephropathy
• Nephrotic syndrome – Too non-specific but this clinical picture may arise from the IgA nephropathy
• Glomerulonephritis – too non-specific
• Henoch-Schonlein purpura^#

^# nonSOP condition

Clinical onset

Onset can be at any age, but is most common in the second or third decade of life.  The condition mostly presents with haematuria (blood in the urine), either visible, or detected on urine testing. Some patients present with nephrotic syndrome (swelling (oedema), elevated blood pressure and impaired kidney function).

Clinical worsening

Around half of patients have a benign and stable course with persistent low grade haematuria or proteinuria. The remainder typically progress slowly to end-stage renal failure over several decades. Markers of worsening disease include declining renal function, increasing urinary protein excretion and worsening hypertension.