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Fibromuscular Dysplasia G030

Document
Last amended 
10 November 2016
Current RMA Instruments
Reasonable Hypothesis SOP
79 of 2016
Balance of Probabilities SOP
80 of 2016
Changes from previous Instruments

SOP bulletin 193

ICD Coding
  • ICD-9-CM Codes: 447.3, 447.8
  • ICD-10-AM Codes: I77.3
Brief description

Fibromuscular dysplasia (FMD) is a rare disease of medium to small arteries that has similar effects to atherosclerosis.  It most commonly affects the renal and internal carotid arteries.

Confirming the diagnosis

The clinical presentation varies significantly with the arteries involved and the severity.  In patients suspected of having this condition confirmation requires radiological imaging of involved arteries in the form of computed tomography angiography (CTA), magnetic resonance angiography (MRA), duplex ultrasonography, or digital substraction angiography (DSA), showing characteristic changes of FMD.

The relevant medical specialist is a physician.

Additional diagnoses covered by the SOP
  • Nil
Conditions not covered by the SOP
  • atherosclerotic vascular disease
  • vasculitis
Clinical onset

The first clinical evidence of FMD may be: hypertension that is severe, resistant to treatment, or of early onset; a cervical or abdominal bruit (an abnormal sound heard via a stethoscope over an affected artery); pulsatile tinnitus; evidence of ischaemic vascular disease or a variety of other manifestations.  Advice on the timing of clinical onset should be sought from the treating physician if possible.

Clinical worsening

Appropriate treatment varies with the clinical presentation and may involve medical therapy or procedures/surgery to revascularise (reopen) occluded arteries.  The condition will commonly progress despite optimal treatment.  Specialist medical advice will generaly be required to establish whether there has been clinical worsening beyond the normal course of the disease.