From the Departments of Pediatrics, Cardiology* and Ophthalmology, ** Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India.

     Reprint requests: Dr. Lata Kumar, Professor and Head, Department. of Pediatrics, Postgraduate Institute of Medical Education and Research,  
Chandigarh 1600/2, India

Manuscript received: December 29, 1997; Initial review completed: March 4, 1998; Revision accepted: September 28,1998.

Takayasu's arteritis (TA) is a chronic, idiopathic, inflammatory disease primarily affecting large vessels, such as aorta and its branches(1). It mainly affects young females in the age group 10-30 years(2). Its onset in early childhood is uncommon(3-6) and there is paucity of Indian literature in the pediatric age group(3). We report here our experience of managing T A in young children over the last 5 years.

Subjects and Methods

We have managed six patients of TA over last 5 years. The diagnosis of this condition was made in these patients as per the ACR 1990 criteria (Table l) for. the classification of T A(7): All children in this report fulfilled the criteria for classification of T A. Autopsy findings in one patient who died were also consis­tent with the diagnosis of T A(7).

All patients were females with mean age of presentation being 9.8 years (Table ll). All six patients had severe hypertension. Two patients had congestive heart failure second­ary to hypertension. Two patients had typical hypotensive retinopathy. Four out of six patients had strongly Positive Mantoux test. This test could not be performed in Case 4 because of her severe illness and Case 2 had a non-reactive Mantoux test. Angiography was performed in all patients except one who was too sick to undergo this procedure.

Treatment included use of immuno­suppressants, antihypertensives and antituber­cular therapy (Table Ill). Four patients under­went surgical interventions as shown in Table Ill. All children were continued on low dose prednisolone (5-7.5 mg/day) and antihyper­tensive medication was tapered according to individual needs.

 Discussion

TA is a large vessel arteritis involving aorta and its major branches. Though the precise etiology is still unknown, there appears to be some association with an underlying tuber­culosis infection. Pantell and Goodman showed that the occurrence of tuberculosis in patients with TA was higher than in general population(8). Gupta had noticed positive Mantoux test in 45% cases(8). It is postulated that Mycobacterium tuberculosis initiates a hypersensitivity reaction in large vessles(9). Four out of six patients in this report also had a large positive Mantoux test and received antitubercular therapy. Autoimmune nature of this disease has been suspected by many workers because of its association with various autoimmune disorders. Presence of anti aorta autoantibodies have been reported by many workers. Levels of anti-endothelial cell antibodies and anticardiolipin antibodies have also been reported to be raised in these patients(10).

TA is 8-10 times more common in females than males(11). All our patients were young girls. The usual age of involvement is 10-30 years, but the disease is well known to occur in very young children as well. Golding et al. reported] 8 children less than 4 years of age with the youngest child being 4 years 0Id(4). The mean age of our patients was 9.8 years (range 6-12 years). There is paucity of pediatric literature on T A from our country.

The commonest clinical feature at the time of presentation is hypertension. All 6 of our patients had severe hypertension at admission. However, it should be noted that a pre­pulseless stage precedes the onset of hyper­tension in this condition. The clinical features during the prepulseless stage are non specific and include fever; night sweats, malaise, arthralgia, myalgia and skin rash., Second stage is of vascular insufficiency(12). Development of hypertension in these patients is multifactorial in ,origin. It may be due to renal artery involvement, loss of elasticity due to aortic involvement, cerebral ischemia and involvement of baroreceptors. Renal artery involvement was documented in 2 out of 6 patients in our series. Retinopathy in T A can be hypertensive or hypotensive, but classical Takayasu's retinopathy is hypotensive which results from neovascularisation secondary to arterial hypotension(l3). Cases 2 and 3 had ,typical Takayasu's retinopathy.

Angiography remains the gold standard for diagnosis of T A. Various workers have used gallium scanning, CT .scanning, ultra­sonography and MRI. Non invasive nature of these procedures may look more easy, but none is as accurate as angiography(10).

Medical treatment of Takayasu' s' arteritis includes use of immunosuppresants. The preferred drug is prednisolone which is given in high doses initially (i.e., 1-2 mg/kg/day) and then tapered off after the activity parameters (especially the ESR) have come down. Long term low dose prednisolone is usually continued indefinitely(14). Use of additional immunosuppresants (azathioprine/methotrexate) has been tried by some workers, but is largely empirical(11). Since Takayasu's arteritis is a rare disease, exact guidelines for use of these drugs are not available in litera­ture. Prednisolone was started in 5 of the 6 patients in this series and was successfully tapered to low dose daily therapy. Role of antitubercular therapy is in those patients who either have coexisting tuberculosis or when there is possibility that steroid therapy can result in exacerbatio nof old tuberculosis. Platelet inhibitors can be used in patients having risk of cerebral infarction or ischemic changes of other organs due to occlussive arterial lesions. In patients with evidence of increased blood coagulability, anticoagulants are to be used. Role of fibrinolytic therapy is questionable( 15).

Interventional procedures are indicated in Takayasu's arteritis when there is critical stenosis of renal vessels, 'extremity ischemia limiting activity, clinical features of cere­brovascular ischemia, moderate aortic regur­gitation and myocardial ischemia due to proven coronary artery stenosis(1). The aim of interventional procedures is to restore, adequate circulation to ischemic tissue before irreversible damage' occurs(8). Various types of interventional techniques which have been used are endarterectomy, resection of diseased segment with graft replacement, bypass of stenosed segment with patch angioplasty and percutaneous transluminal baloon angioplasty(16-18). Improvement has been documented in 85% of patients with hypertension who underwent renal angioplasty(17).

Angioplasty with stent insertion was done in 2 of our patients and in two patients only angioplasty was done. All these children have shown symptomatic improvement. However, one of our patients (Case No.3) who had a stent insertion, subsequently developed restenosis and would need re-intervention.

One of our patients (Case No.4) had an incidental finding of pulmonary capillary hemangiomatosis on autopsy(19). It is a rare vascular proliferative process found in children and young adults in which there is an overgrowth of thin walled capillary sized blood vessels in pleura, perilobular septa, peribronchial and periarterial connective tissue sheath and lung parenchyma(20). This has not previously reported in association with TA.

The prognosis is Takayasu's arteritis has improved significantly over the last two decades. With medical and surgical treatment five year survival is estimated to be 94%(7). Takayasu's arteritis is now considered to be a potentially treatable condition and the long term outlook, even in young children, is not as grim as it is used to be in the past.