Discussion:
Takayasu’s arteritis is a chronic idiopathic inflammatory condition, typically beginning in the second or third decade of life. Its progression involves the gradual development of fibrotic narrowing in the aorta and its major branches. This can lead to various complications such as narrowing, clot formation, dilation, or even the formation of aneurysms. Regions like Asia, Africa, and Latin America report the highest number of cases, with Asia (2.69 cases per million per year) specifically showing an incidence rate about 100 times higher than Europe and North America7. It primarily affects young female during their twenties and thirties, but has been reported in children as young as 24 months of age9.
According to Sharma et al.’s study, Indian and South Asian patients more frequently exhibit type II and III angiographic involvement compared to type I, which contrasts with the predominant type I involvement seen in Japan and Western countries. Japanese patients typically experience the disease starting in the ascending aorta and arch before progressing to the thoraco-abdominal aorta. In contrast, Indian patients commonly initially present with involvement of the abdominal aorta, which then progresses upward to affect the thoracic aorta10. It often presents with varied clinical manifestations. In our patient, the presenting symptoms developed lately were painful blurring of vision and orbital pain with an ocular examination finding of 2:4 arteriovenous ratio indicative of venous engorgement which align closely with recognized ophthalmic manifestations of Takayasu arteritis (TAK)11.
It has an insidious onset which manifest as arteritis early in the course resulting in segmental stenosis, occlusion, dilatation and/or aneurysm of the vessel7. Vessel wall thickening, narrowing, and complete blockage can lead to reduced blood flow and tissue damage, posing significant risks to individuals with Takayasu’s arteritis (TA). This condition can impair organ function due to ischemia, potentially threatening the lives of affected patients. In addition, involvement of the coronary arteries in TA is particularly concerning, as it is associated with a poor prognosis and higher mortality rates. Furthermore, performing coronary artery revascularization in patients with active TA is challenging and carries an elevated risk of major adverse cardiac events (MACE)12.
A case of type III TA reported by Del et al in a 25 year female with 7 years of prednisolone administration resulted in disease remission, control and also improves the diameter of abdominal aorta13.
EULAR guidelines suggested Prednisolone as the primary treatment choice with an initial dosage of 1 mg/kg/day (up to a maximum of 60 mg/day) maintaining for a month, followed by a gradual reduction in dose. In most cases, additional immunosuppressive therapy is necessary to reduce the risk of steroid-related side effects and manage disease progression. It’s crucial to note that discontinuing steroid treatment can often lead to relapses. Up to 70% of Takayasu arteritis patients may require vascular surgery or bypass grafting to address aspects like renovascular hypertension. Although it has a favorable outcome, subsequent revision surgery are often necessary. Angioplasty and stent placement are associated with higher restenosis rates. It is advisable to schedule elective procedures during periods of disease remission requiring Long-term follow-up8.
Similarly, tocilizumab (TCZ) stood better than traditional DMARDs in patient involving coronary arteries with TA in terms of reducing disease activity as a whole, improving lumen stenosis and reduction of glucocorticoid dose post- TCZ treatment for 6 months12.
Kwon et al revealed that the administration of statins on TAK patients with active disease substantially reduces the relapse rate following remission attainment in this population14.
In our case, four years after the onset of the disease, imaging studies revealed extensive vascular changes, including 90% stenosis of the left common carotid artery and complete occlusion of the left subclavian artery with distal reformation. The delayed diagnosis resulted in lifelong morbidity for the patient, with no procedure able to resolve the persistent fatigue experienced. However, she was counselled to undergo arterial bypass which would relief dizziness temporarily. As the disease progresses beyond the bypass’s effectiveness, the symptoms would reappear. Despite treatment initiation with high-dose steroids and immunosuppressants, the disease had progressed significantly, limiting treatment options to symptomatic relief and delay of further progression which profoundly impacted on her quality-adjusted life year. Study has shown that, it significantly impacts patients’ quality of life, with both physical and mental health scores lower than those of many other chronic diseases involving peripheral vascular disease15. This case shows the typical natural history of type 1 Takayasu arteritis and the dreadful morbidity the patient had to suffer.