Rare, interesting and a bit curious is Takayasu’s. Not only can it be slowly progressive without proper general symptoms (fever, weight loss, arthralgias and what not; not even lab signs of inflammation), it can even be abortive and only leave a difference in pulses.
Our ~40 yo female patient was symptomatic about 4 years ago, when she developed aortic insufficiency, which got worse after about 1-2 years, so that finally she was operated on. In situ they discovered widespread inflammation of the aorta, so they decided to implant an aortic conduit with a biological valve. On histology they found some panarteritis centered about the vasa vasorum, so that a rheumatology workup including PET was started. This revealed some inflammation around the conduit (deemed normal) and some inflammation parameters which decreased over time. Nothing then for over 2 years. Well, she did develop some orthostatic vertigo. A few days ago she presented to the ER with left-sided TIA and we found an occluded CCA on the left, a highly stenotic brachiocephalic trunc on the right and an occluded subclavian artery.
She still doesn’t have an elevated ESR or CRP, no other lab abnormalities. PET yields a highly inflamed aorta together with some inflammation in the major supraaortic branches. The infection workup (TPHA and so on) was negative.
By all diagnostic criteria, this is Takayasu’s. So we discussed this patient in our session on Friday, using the following literature:
- A review from 2002 in the BMJ – nothing much has happened since then.
- Some other review of 2009 (!!! have to look this up)
- A short chapter in “Uncommon causes of stroke” – a wonderful book for every neurovascular nightstand.
Some take home messages:
- There is a prepulseless phase with proper vasculitis symptomatology, then a slowly progressive, often quite unremarkable progress, until first signs of vascular insufficiency declare the disease.
- It works from left to right in terms of aortic branches, then from top to bottom, arriving at the abdominal aorta and leg arteries only very late.
- Conventional angiography used to be the gold standard, but in my view CTA is pretty good. Do MRA if your neuroradiologist can do it.
- Histology is usually difficult to come by – PET is the first alternative.
- Rule out infection and rare other disease: lues, borreliosis, HIV, TB, sarcoid, Behcet.
- If in doubt: treat.
- Steroids are key. CYC probably isn’t. Use MTX or TNFalpha-blockers, maybe AZA or other standard immunosuppressants. On the horizon, anti-rejection-medication could be the thing.
- Stenting is allowed, if disease is properly controlled. Of course, it yields worse results than in degenerative artery disease, but what doesn’t?