Dr Theresa Raju, Dr Suma Balan(paediatric Rheumatology), Dr C Jayakumar, Amrita Institute of Medical Sciences, Kochi
Thirteen year old female child with right eye scleritis,anterior uveitis and systemic hypertension presented with complaints of headache and multiple episodes of vomiting of 2 days duration and 2 episodes of generalized seizures with post ictal confusion.
Prolonged fever, myalgia, weight loss, orogenital ulcers were absent .No skin nodules or rashes.
Her Vitals and PICCLE were normal except for the BP which was 170/100mmhg
On evaluation venous doppler showed mild abdominal aortal ectasia at the renal level. She was admitted for further evaluation and management.
At admission, the child appeared drowsy with hypertension and discrepancy in upper and lower limb blood pressure of more than 10mm of hg. An urgent MRI with contrast was done for the same and revealed a posterior reversible encephalopathy.
Lab CBC normal but Plt:506000 and ESR: 70 and CRP:14. Hypertension was managed with arkamaine,nifedipine and atenolol.
The accelerated hypertension with high inflammatory markers with a past history of scleritis prompted an evaluation for systemic vasculitides.
Renal artery doppler was done which showed findings suggestive of right renal artery stenosis. In view of these, a provisional diagnosis of large vessel vasculitis due to Takayasu’s arteritis was considered. Diagnostic investigations towards the same diagnosis prompted further imaging with CT Aortagram which showed multiple aneurysms with bilateral renal artery stenosis, right small kidney and a thin arterial wall with ulceration in D7 part of descending thoracic aorta.
Child was initially managed in ICU with antiepileptics and antihypertensives. As the arteriographic findings were congruent with typical findings of Takayasus arteritis with active disease, she was administered Methylprednisiolone pulsefor 3 days along with cyclophosphamide IV with MESNA .
As Takayasus arteritis is known to be associated with Latent TB infections and past history of Quantiferon TB Gold positivity, repeat Mantoux and Quantiferon TB gold was done , both of which were positive, hence she was started on antitubercular therapy according to weight.
In a multidisciplinary meeting, stenting the renal artery stenosis was planned after control of disease activity. Methyl prednisolone was changed to oral prednisone at a dosage of 1mg/kg and She was asymptomatic prior to discharge and her CRP had normalised(0.84mg/L).
Takayasu’s Arteritis
It is a systemic inflammatory condition characterized damage to the large and medium arteries and their branches.
It occurs predominantly in aorta and its major branches ,particularly the renal arteries ,carotid arteries and subclavian arteries and leads to stenosis, occlusion or aneurysmal degeneration of large arteries .
Pathophysiology:It is characterized an inflammatory granulomatous vasculitis of medium and large arteries which leads to transmural fibrous thickening of the arterial walls, leading to multiple vascular obstructions and eventual ischemic changes.
Cell mediated immunity involving CD4+ and CD8+ T cells may play a key role in the pathophysiology of Takayasus arteritis as these cells supports the formation of granulomas and potenitially activate the activities of various proteases such as matrix metalloproteinase as well as other cells which promote chronic inflammation and fibrosis formation
It presents at first with constitutional symptoms such as fever ,malaise, weight loss, anorexia.
Other presentations such as hypertension, neurological manifestations and upper limb claudications occurs secondary to arterial insufficiency.
Severe debilitating upper or lower extremity ischemia often leads to diminished or absent pulses, hence the name pulseless disease or possibly a vascular bruit.
Other potential late findings include accelerated atherosclerosis or heart failure.
Treatment: Initial treatment of symptomatic pt begins with corticosteroids with Immunosuppressive medications started upfront .
If not responding , starting biological early is recommended
Transluminal angioplasty has been studied in the past. Arterial reconstruction is the other surgical option .
The type of reconstruction depends on where the lesion lis seen as well as the patients surgical anatomy.
In general, Dacron or polytetrafluroethylene would be used for major aortic reconstructions while autogenous saphenous vein would be used for extremity or isolated renal or mesentricrevascularization.
Surgical therapy is usually tolerated due to young age of most patients
Takayasu’s arteritis is a complex vascular disorder that has no cure. It is best managed with an interprofessional team that includes a vascular surgeon, radiologist, cardiologist, neurologist and nephrologist. While isolated lesions may be stented , large lesions may require a pass. Some patients may benefit from steroids and other immunosuppressive agents. These patients will need long term monitoring for adverse effects of the drugs. The prognosis is guarded.