Large Vessel Vasculitides: Takayasu

by Carlo Raj, MD

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    00:01 So under Takayasu—a young, Asian lady walking through the door.

    00:08 You each try checking for her pulses. Radial—nothing.

    00:13 Carotid—nothing.

    00:16 It’s known as pulseless disease. So what’s going on here with this large vessel issue? There’s going to be granulomatous intense inflammation.

    00:26 95% of the time, the blood vessels that are being affected, the large ones, are going to be the branches of the arch of aorta.

    00:36 So you’re going to be focusing upon asymmetric blood pressure, pulseless disease.

    00:44 Diagnostically, what are you going to do? Well, in many of these issues with the blood vessels, you’re going to find an elevated erythrocyte sedimentation rate.

    00:53 So that doesn’t tell you much apart from the fact that—okay, maybe your patient has vasculitides.

    00:59 On MRI/MRA, what are we looking for? On angiography or our imaging study? It’s those blood vessels coming of the—you’re going to focus upon the branches of the arch of aorta.

    01:11 So now, as I’ve told you, the patient—a young, Asian, less than 40 lady, doesn’t have pulses in the carotids and may not have pulses in the radial because of issues of vasculitis in maybe left subclavian, maybe of the carotid.

    01:30 Management: High-dose steroids, but even that could be unaffected or ineffective therefore, you might be thinking about repeat, repeat, repeat coronary artery bypass grafting or CABG’s.

    01:48 Going on to our next major large vessel disease.

    01:52 Here we have giant-cell arteritis AKA temporal arteritis.

    01:56 There’s this patient walking through the door. She’s complaining of pain, maybe in the temporal region and maybe in the jaw area.

    02:05 In addition, there might also be involvement of neck and shoulder issues or maybe hip. In other words, there is an association with polymyalgia rheumatica connective tissue disease and a high percentage, 50%, usually going to be an elderly lady, maybe about 67 or so—it doesn’t always have to be but could be and what you’re worried about with temporal arteritis or giant-cell arteritis is a particular branch off the temporal artery known as the ophthalmic branch.

    02:38 And if not careful, that ophthalmic branch with severe vasculitis, will then undergo or may lead into blindness, permanently, in that patient.

    02:52 What do you do for diagnosis? You’re going to do a biopsy, and in that biopsy, you’re then going to find a granuloma.

    03:00 You find granulomatous changes and giant cell and mononuclear infiltration of the vessel wall. That must be understood.

    03:11 Yeah, you also find a high ESR. I told you the elevated ESR is nonspecific.

    03:16 You must immediately begin the patient on corticosteroids, because if you don’t—please focus on the fact that the ophthalmic branch will undergo severe vasculitis, and the end organ here would be the eyes and, therefore, resulting in permanent blindness. Giant-cell arteritis.

    03:38 Let me show you a picture here. On the left, are the branches of the aorta. Now those arrows are pointing to areas of the blood vessel in which there’s no perfusion.

    03:50 If you take a look at the arch, there is blood passing through there and the areas that are less opaque and more lucent, in other words, the areas that look more black are the areas in which no blood is passing through.

    04:08 So off the arch, let’s say that it’s the carotid, well if there’s no blood passing through the carotid then the patient—or you, the sign, is no pulse.

    04:19 Welcome to Takayasu. On the left, narrowing.

    04:24 Treatment, please? Corticosteroids. If that doesn’t work, maybe perhaps you need to do some bypass surgeries.

    04:33 The picture on the right is the forehead of your patient.

    04:37 In this elderly lady, take a look at the hair here—greyish, things like that you want to observe, and specifically, you’re looking at branches of the temporal artery that has been affected in giant-cell arteritis or temporal arteritis undergoing inflammation.

    04:54 Remind me again. If left untreated, not given steroids, what may then happen? Correct. Permanent blindness; and the picture on the right depicting giant-cell arteritis.

    About the Lecture

    The lecture Large Vessel Vasculitides: Takayasu by Carlo Raj, MD is from the course Vasculitis. It contains the following chapters:

    • Large Vessel Vasculitides - Takayasu
    • Large Vessel Vasculitides - Giant-cell Arteritis

    Included Quiz Questions

    1. Arch of aorta.
    2. Ascending aorta.
    3. Descending aorta.
    4. Abdominal aorta.
    5. Thoracic aorta.
    1. It most commonly affects old women.
    2. It most commonly affects young females.
    3. Arch of aorta is most commonly affected.
    4. It is also known as pulseless disease.
    5. High dose of steroids is given for treatment.
    1. Temporal artery biopsy.
    2. MRI.
    3. CT-Scan.
    4. Complete blood profile.
    5. X-ray.

    Author of lecture Large Vessel Vasculitides: Takayasu

     Carlo Raj, MD

    Carlo Raj, MD

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