Paediatrics: Takayasu’s arteritis (pulseless disease)

Takayasu’s arteritis (pulseless disease)

Rare chronic granulomatous panarteritis affecting aorta and large arteries.

Adolescent Asian (Japanese) girls and young women most susceptible.

Clinical features

• Subclinical prepulseless phase may last years: anorexia, fatigue, poor growth, unexplained fevers, and episodic arthritis (50%).

• Pulseless phase: diagnoses often made incidentally.

•   diminished peripheral pulses and aortic dilatation on CXR or hypertension and renal artery stenosis;

•   dramatic presentation with severe hypertensive encephalopathy and seizures; congestive cardiac failure; aortic valvulitis and aortic regurgitation; pulmonary stenosis;

•   syncope s to paroxysmal hypertension or paroxysmal tachycardias with facial flushing headaches, chest pain, dyspnoea, and palpitations. May be triggered by changes in posture or micturition (i.e. baroreceptor hypersensitivity).

Investigations

• FBC (normochromic normocytic anaemia, thrombocytosis); ESR and gamma globulins very elevated even in the prepulseless phase.

• Imaging: high resolution carotid US, angiography, or MR angiography show characteristic arterial dilatation, post-stenotic dilatation, aneurysm, thrombosis, and occlusion of the proximal branches of the aorta.

Treatment

• Manage hypertension: β-blockers and ACE inhibitors. Avoid vasodilators.

• Treat vasculitis: initially high dose steroids (prednisolone 1mg/kg/ day or equivalent) with MTX or AZA as steroid-sparing drugs. Cyclophosphamide for severe or resistant cases.

• Surgery: range from angioplasty to bypass grafting.

Prognosis

10yr survival 90%, although the majority (75%) have some impairment of daily living, and 50% are disabled. Prognosis depends on hypertension and aortic incompetence. Successful planned pregnancy is possible.