<div>Our case highlights an unfortunate occurrence where the patient had
already developed a cerebrovascular event at diagnosis, seen in up to
6-8% of patients at presentation and up to 20% in the course of the
illness.(7,8)</div><div>Also, important to note is the pattern of vascular involvement which can
be identified both clinically but more accurately radiologically (9).
Takayasu arteritis can affect several large arteries. The pattern of
vascular involvement appears to vary with geographical region; patients
in Japan and India have predominant aortic arch and abdominal aorta
lesions respectively on imaging. (10). Whereas patients with Takayasu
arteritis in Tunisia and Morocco in North Africa, tend to present with
mostly aortic arch and/or subclavian artery involvement as described by
several authors. (11–13) Conversely, in South Africa, two case series
including the largest case series in Africa on Takayasu arteritis with
two hundred and seventy two patients by Mwipatayi et al describe more
patients with hypertension as the presenting feature and predominant
abdominal aorta involvement compared to the aortic arch and its
branches.(14,15)</div><div>The cases in literature describing patients from East Africa show a
mixed pattern at initial presentation with diffuse aortic and retinal
involvement respectively in two cases in Tanzania. (16,17) Elsewhere in
Uganda, a case report describes autopsy findings of fibrosis in several
branches of the arch of aorta. (18) In Kenya, two cases have been
reported, one presenting as chronic headache with carotid artery
stenosis and thoracic aorta involvement with another describing femoral
involvement from biopsy of amputated limb in a set up with limited
angiography capabilities at the time. (19,20) Our patient had
predominantly subclavian and carotid artery involvement and no clear
aortic localization which is similar to a case series description in
Tunisia. (13) Inflammatory markers such as erythrocyte sedimentation
rate and C reactive protein are frequently elevated as was the case in
our patient but there are non-specific.</div><div>Generally, treatment is multidisciplinary with both medical and surgical
interventions needed. Systemic glucocorticoids form the backbone of
medical treatment of Takayasu arteritis patients. Additionally, disease
modifying antirheumatic drugs (DMARDS) such as azathioprine and
methotrexate are concomitantly used to allow for tapering of steroids
and minimize glucocorticoid associated side effects. Lastly, biologic
agents such as Tocilizumab and ant—Tumor Necrosis Factor inhibitors
can be used. Surgery on the other hand is usually indicated in critical
vessel stenosis or to repair arterial aneurysms.</div><div>Two major rheumatology organizations namely EULAR and ACR have published
guidelines on management of Takayasu arteritis. However, the quality of
evidence available to support these recommendations has remained low.
(21,22)</div><div>The European League Against Rheumatism (EULAR) 2018 guidelines offer
several recommendations to guide management of patients with Takayasu
Arteritis. Acutely, EULAR supports initiation of glucocorticoids plus
disease modifying antirheumatic drug as initial treatment with tapering
off of steroids as tolerated. In refractory disease or major relapse,
adjunctive therapy with Anti-IL6 agent tocilizumab and Anti- TNF agents
are recommended. More specifically, for minor relapses, a trial of
re-institution of higher glucocorticoid doses is advised before
initiating Tocilizumab or anti-TNF agents. Additionally, for recurrent
relapses adjunctive therapy with biologic agents is advised.
Antiplatelet agents are also not routinely recommended and should only
be considered on a case-by-case basis based on degree of stenosis and
other risk factors. Further, the EULAR guidelines recommend for vascular
surgical interventions to be done electively during stable remission
since interventions during flares are associated with poor patency rates
of repaired vessels. However, whenever critical stenosis causing
ischemia or dissecting aneurysms is present surgical intervention should
be pursued emergently. Finally, despite there being no research evidence
to support follow up process, initial close follow up of 1-3 monthly
visits in the first year followed by 3-6 monthly visits afterwards is
advised due to high relapse rates. If relapse-free remission is
achieved, then annual follow-ups can be scheduled thereafter. During
follow ups, clinical assessment including ESR and CRP measurements are
advised with imaging being ordered on a case-by-case basis. (21)</div><div>On the other hand, the American College of Rheumatology (ACR) guidelines
are largely similar to the EULAR guidelines except for a few subtle
differences. Some of the most notable ones are recommendation for use of
ant-TNF inhibitors over Tocilizumab as initial addition in refractory
disease and addition of aspirin therapy in patients with active disease
and critical cranial or vertebrobasilar involvement. Additionally,
despite recommending oral over intravenous glucocorticoids, the ACR
guidelines give leeway for use of intravenous pulse steroids in cases of
organ or life threatening disease like in our patient’s case. (22)</div><div>Despite advances in diagnostic and treatment of Takayasu arteritis, the
rate of complications prevails with reported rates as high as 50%. Some
of these complications include ischemic cerebrovascular incidences as
experienced by our patient, as well as aortic regurgitation and
associated heart failure, end stage renal disease in cases where renal
vasculature has been involved. As expected, patients with more extensive
disease at the time of diagnosis have higher complication rates. (23,24)
Similarly, patients with a more progressive course, i.e., with few or no
event free periods also experience higher rates of complications.(25)</div><div>Takayasu Arteritis has been described as a chronic condition with a
relapsing pattern and this contributes significantly to the morbidity of
the patients.(26) Recent studies have found that the male sex, presence
of ongoing inflammation with elevated CRP levels and those carotidynia
are more likely to experience relapses.(27,28)</div><div>Although limited data is available, it should be noted that Takayasu
Arteritis is associated with reduced patient reported quality of life
with patients with the disease also reported to have higher rates of
clinical depression and anxiety compared to the general population
particularly during active disease.(29) It is therefore important to
ensure the evaluation of patients includes these aspects to ensure all
concurrent morbidities are appropriately evaluated and managed.</div>