Synopsis
Identify the characteristic non-atherosclerotic arteriopathy, assess renal and cerebrovascular complications, and coordinate individualised blood-pressure, antithrombotic and revascularisation decisions through a specialist multidisciplinary service.
- Fibromuscular dysplasia is an idiopathic, segmental, non-atherosclerotic and non-inflammatory disease of small and medium arteries that can cause stenosis, aneurysm, tortuosity and dissection.
- Renal and cervical arteries are the commonest clinically involved beds, and more than one territory is often affected; symptoms depend on which artery and complication are present.
- Think of renal FMD in a younger or middle-aged woman with early, abrupt or resistant hypertension, especially when imaging shows a mid-distal renal lesion rather than calcified ostial plaque.
Key red flags
Sudden unilateral head or neck pain, partial Horner syndrome, retinal or cerebral ischaemia, acute coronary syndrome without usual plaque risk, or abrupt flank pain can indicate a dissected affected artery.
Investigation priorities
Quantify the renal consequence, establish treatment safety and identify a competing intrinsic nephropathy.
Management branches
CTA, MRA or angiography reports renal or cervical beading or a compatible focal lesion.
- Ask radiology to confirm morphology and exclude ostial plaque, inflammatory wall thickening and artefact; record every involved bed, aneurysm, dissection and infarct.
- Assess blood pressure, kidney function, urine ACR and neurological history, then refer to a renal-vascular or FMD service for coordinated review.
Key medicines
ACE inhibitor or angiotensin-receptor blockerSelect a licensed agent and titrate gradually under NICE hypertension guidance and the local renal formulary.
Additional antihypertensive therapyUse licensed calcium-channel blocker, thiazide-like diuretic or other agents in stepped combinations tailored to physiology.