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Educational draft · awaiting clinical reviewUse Rapid for revision, not patient-care decisions. Check current national and local guidance and the BNF or BNFC before acting.

Fibromuscular dysplasia

Renal · UK scope · Sources checked 27 Aug 2026 · Clinical review pending

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Escalate

New focal neurology, thunderclap headache, painful Horner syndrome, acute neck or flank pain with organ ischaemia, suspected arterial dissection or aneurysmal subarachnoid haemorrhage, and severe hypertension with acute kidney, cardiac or neurological injury demand emergency stroke or vascular pathways and immediate specialist imaging.

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.
  • Multifocal disease produces alternating stenoses and dilatations, the classic string-of-beads appearance; focal or tubular disease requires more careful exclusion of atherosclerosis, vasculitis and syndromic arteriopathy.
  • Headache and pulsatile tinnitus are common but non-specific. TIA, stroke, cervical-artery dissection, subarachnoid haemorrhage and visceral infarction are uncommon, time-critical manifestations.
  • CTA or MRA establishes the arterial pattern. Duplex can support renal surveillance but may miss distal, branch or cervicocephalic disease and is highly operator-dependent.
  • After FMD is confirmed in one bed, a specialist service should decide a one-time survey for other arterial territories and intracranial aneurysm, balancing diagnostic yield, renal function, radiation and contrast.
  • Blood-pressure treatment is first-line for renal FMD; ACE inhibition or an ARB is often useful but needs creatinine and potassium monitoring, particularly if both kidneys or a solitary kidney are supplied through disease.
  • Balloon angioplasty without a planned stent is the usual renal revascularisation technique for selected haemodynamically significant FMD with resistant hypertension; stenting is generally reserved for procedural bailout.
  • There is no routine curative genetic test and family screening is not automatic. Care includes smoking cessation, pregnancy counselling, symptom education and long-term arterial follow-up tailored to phenotype.

Key red flags

Arterial dissection signal

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.

Aneurysmal complication

Thunderclap headache, meningism, collapse or acute abdominal pain raises concern for intracranial or visceral aneurysm rupture. An unruptured aneurysm may instead be discovered during multisite imaging.

Investigation priorities

01
Validated blood pressure, creatinine, potassium and urine ACRFirst step

Quantify the renal consequence, establish treatment safety and identify a competing intrinsic nephropathy.

Management branches

New diagnosisConfirm pattern and map consequences

CTA, MRA or angiography reports renal or cervical beading or a compatible focal lesion.

  1. Ask radiology to confirm morphology and exclude ostial plaque, inflammatory wall thickening and artefact; record every involved bed, aneurysm, dissection and infarct.
  2. Assess blood pressure, kidney function, urine ACR and neurological history, then refer to a renal-vascular or FMD service for coordinated review.
  3. Agree one-time multisite and intracranial-aneurysm assessment, prevention, pregnancy advice and a symptom-led surveillance schedule with the patient.

Key medicines

ACE inhibitor or angiotensin-receptor blockerSelect a licensed agent and titrate gradually under NICE hypertension guidance and the local renal formulary.Measure creatinine and potassium before and after initiation or titration; bilateral renal involvement, solitary-kidney disease, dehydration, pregnancy, hyperkalaemia and NSAIDs materially change safety.
Additional antihypertensive therapyUse licensed calcium-channel blocker, thiazide-like diuretic or other agents in stepped combinations tailored to physiology.Verify sustained resistance and adherence before adding drugs; review postural symptoms, oedema, electrolytes, kidney function, pregnancy potential and interaction with acute stroke or dissection management.
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Sources and review status4 sources · checked 27 Aug 2026 · clinical review pending+
Sources

Sources and review status

National guidance is shown before implementation-dependent detail. Typical adult dose examples remain subject to patient factors, contraindications and the live BNF or specialist protocol. Source check completed 27 Aug 2026; clinical approval remains outstanding.

  • UK Kidney Association fibromuscular dysplasia informationUK clinician resource on multisite phenotype, diagnosis and renal FMD management.
  • ESVS 2025 renal vascular disease guidelineCurrent specialist evidence and recommendations on imaging and renal FMD angioplasty.
  • NICE NG136 hypertension recommendationsUK confirmation, treatment sequencing and specialist advice for resistant hypertension.
  • NICE NG128 stroke and TIA recommendationsTime-critical UK pathway for neurological presentations that can complicate cervical FMD.

Editorial contact: Doctor’s Passport editorial team. Source-check date is separate from clinical approval. Report a content problem · Content changes

Authoring stateRapid draftClinical stateAwaiting reviewJurisdictionUnited Kingdom