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.