Moffitt Pearls 7.17.17 – Rheum Morning Report – Takayasu Arteritis

Thank you to Arielle for presenting the fascinating case of middle aged women with known Takayasu arteritis transferred from OSH for further evaluation of hypertensive emergency and abdominal pain found to have progressive abdominal ischemia thought to be progressive thrombosis. She required several surgical interventions and bowel removal given ischemia despite a minimally elevated lactate.

Key Pearls

  1. Takayasu Arteritis (TAK) is an uncommon chronic large-vessel vasculitis of unknown etiology. “Large vessel” implies the aorta and any of the vessels that directly branch off of the aorta.
  2. There are no diagnostic laboratory tests for TAK and as such imaging studies of the arterial tree by MRA or CTA are essential for helping in making the diagnosis.
  3. Large vessel vasculitis is associated with acute arterial thrombosis. Add this to the DDx of pathological processes that cause arterial thrombosis – APS, HIT, Chronic DIC and Hyperhomocysteinemia

Takayasu Arteritis

  • TAK is an uncommon chronic vasculitis of unknown etiology, which primarily affects the aorta and its primary branches. Women are affected in 80 to 90 percent of cases, with an age of onset that is usually between 10 and 40 years. It has a worldwide distribution, with the greatest prevalence in Asia.
  • Classification criteria have been developed for TAK as a means of categorizing patents for research studies. Patients are said to have TAK if at least three of the six criteria are present.
    • Age at disease onset ≤40 years
    • Claudication of the extremities
    • Decreased pulsation of one or both brachial arteries aka “pulseless disease
    • Difference of at least 10 mmHg in systolic blood pressure between the arms
    • Bruit over one or both subclavian arteries or the abdominal aorta
    • Arteriographic narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the proximal upper or lower extremities, not due to arteriosclerosis, fibromuscular dysplasia, or other causes
  • There are no diagnostic laboratory tests for TAK.
  • Imaging studies are essential for establishing the diagnosis of TAK and for determining the extent of vascular involvement. Patients with suspected TAK should undergo imaging of the arterial tree by magnetic resonance angiography (MRA) or computed tomography angiography (CTA) to evaluate the arterial lumen
  • Differential Diagnosis of TKA
    • Giant cell (temporal) arteritis (GCA) – often significant overlap (table below)
    • IgG4-related disease
    • Behçet’s syndrome
    • Infectious aortitis
    • Fibromuscular dysplasia
    • Other diseases which can feature large-vessel vasculitis/aortitis such as Cogan’s syndrome, relapsing polychondritis, and spondyloarthropathies

GCA vs. Takayasu Arteritis (adapted from UpToDate)

Finding GCA Takayasu
F:M ratio 3:2 7:1
Age at onset >50 yrs <40 yrs
Ethnic ancestry European Asian
Histopathology

Granulomatous inflammation

Primary vessels External carotid arteries Aorta and branches
Renovascular HTN Rare Common
HLA association HLA-DR4 HLA-Bw52
Course Self-limited Chronic
Response to corticosteroids Excellent Excellent
Surgical intervention needed Rare Common

Hypertensive Emergency (EM) – Management

  • Hypertensive emergency is present when severe hypertension is associated with acute end-organ damage
  • There is not a specific blood pressure number that describes hypertensive emergency
  • Immediate but careful reduction in blood pressure is indicated in hypertensive emergencies; an excessive hypotensive response may lead to ischemic complications
  • For most hypertensive emergencies, mean arterial pressure should be reduced gradually by about 10 to 20 percent in the first hour and by a further 5 to 15 percent over the next 23 hours
  • Medications used to treat hypertensive emergency
    • Vasodilators
      • Nicardipine
      • Nitrates
      • Hydralazine
    • Adrenergic inhibitors
      • Esmolol
      • Labetolol

Differential Diagnosis of Spontaneous Arterial Clot

  •  Anti-phospholipid syndrome
  • HIT
  • Hyperviscosity States
    • Waldenstrom’s macroglobulinemia
    • Leukostatis in s/o leukemia
    • Sickle Cell disease
  • Myeloproliferative d/o
    • PV, ET, CML
  • Paroxysmal Nocturnal Hemoglobinuria
  • Chronic DIC, TTP/HUS
  • Hyperhomocysteinemia
  • Large Vessel Vasculitis

** remember though **

  1. atherosclerosis & embolism are far and away the most common causes of arterial clots.
  2. All of the conditions above cause both arterial AND venous clotting – and venous clotting is MUCH more common than arterial clots, even in these conditions.

Here’s a great link to a summary from a Stanford Educational Resource:  http://errolozdalga.com/medicine/pages/ThrombosisEmbolismHypercoaguableStatesAnti.cr.3.15.11.html

Evernote: https://www.evernote.com/shard/s462/sh/a8c34676-7719-42ff-9906-e8e8f9367bf1/1d2841c0a3f2f5aba49dd9a82a0a1673

 

 

 

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