Home | | Paediatrics | Paediatrics: Takayasu’s arteritis (pulseless disease)

Chapter: Paediatrics: Bones and joints

Paediatrics: Takayasu’s arteritis (pulseless disease)

Rare chronic granulomatous panarteritis affecting aorta and large arteries.

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.

 

Study Material, Lecturing Notes, Assignment, Reference, Wiki description explanation, brief detail
Paediatrics: Bones and joints : Paediatrics: Takayasu’s arteritis (pulseless disease) |

Related Topics

Paediatrics: Bones and joints


Privacy Policy, Terms and Conditions, DMCA Policy and Compliant

Copyright © 2018-2023 BrainKart.com; All Rights Reserved. Developed by Therithal info, Chennai.