taka-whatsitnow ?!?

this morning we all joined hands and stumbled through a killer case featuring a 34-year-old woman presenting admitted for STEMI and found to have a 99% occluded LM attributed to an underlying diagnosis of TAKAYASU ARTERITIS!!!

what’s that you say? you don’t remember anything about TAKAYASU ARTERITIS? well you’re in luck, cause here are the basics about this rare vasculitis that can initially present as pretty much anything … sigh

Takayasu arteritis is a rare chronic granulomatous vasculitis mainly affecting the aorta and its major branches, and the coronary and pulmonary arteries.

It is more common in women than men and usually presents before age 40 years

Clinical presentation

  • heterogenous clinical presentation3
  • in patients < 40 years old, clinical findings considered red flags suggesting investigation for Takayasu arteritis include3
    • absent or weak peripheral pulses and/or arterial bruits (most common presenting signs)
    • carotidynia (occurs in 10%-30% at presentation)
    • arterial bruit
    • angina
    • limb claudication
    • discrepancy (> 10 mm Hg) in blood pressure between upper limbs
    • hypertension
    • an unexplained acute phase response (elevated erythrocyte sedimentation rate or C-reactive protein or both)

History

Chief concern (CC)

  • most common symptoms at diagnosis in 126 patients with Takayasu arteritis in retrospective cohort study in United States
    • fatigue in 54%
    • light-headedness in 49%
    • headache in 45%
    • upper limb claudication in 40%
    • chest pain in 39%
    • arthralgia or arthritis in 35%
    • weight loss > 5 kg (11 lbs) in 36%
    • myalgia in 19%
    • night sweats in 19%
    • lower limb claudication in 18%
    • abdominal pain in 16%
    • Reference – Mayo Clin Proc 2013 Aug;88(8):822
  • 30%-50% of patients lack constitutional symptoms at presentation3
  • other symptoms may include visual defects (blindness, amaurosis, diplopia)2,4

History of present illness (HPI)

Physical

Skin

  • cutaneous manifestations may vary geographically
    • reported frequency 2.8%-28%
    • inflammatory nodules and erythema nodosum-like lesions more common in Europe and North America
    • other manifestations include Raynaud phenomenon, pyoderma gangrenosum, necrotic or ulcerated nodules, livedo reticularis, and purpura
    • Reference – J Rheumatol 2013 May;40(5):734

Neck

  • may have carotid or subclavian bruits3
  • carotidynia3
    • occurs in 10%-30% at presentation
    • considered a red flag in patients < 40 years old

Cardiac

  • listen for aortic or mitral regurgitation2

Abdomen

  • may have abdominal bruit3

Extremities

Diagnosis

Making the diagnosis

  • consider in patients < 40 years old with certain clinical finding such as3
    • absent or weak peripheral pulses and/or arterial bruits (most common presenting signs)
    • carotidynia (occurs in 10%-30% at presentation)
    • arterial bruit
    • angina
    • limb claudication
    • discrepancy (> 10 mm Hg) in blood pressure between upper limbs
  • diagnosis generally confirmed by angiography or imaging with magnetic resonance angiography (MRA) or positron emission tomography (PET) (EULAR Category 3, Grade C)1,2,3,5
  • American College of Rheumatology (ACR) classification criteria and Chapel Hill Consensus Conference nomenclature may be used to differentiate vasculitides
    • 2012 Chapel Hill Consensus Conference criteria for defining Takayasu arteritis
    • American College of Rheumatology 1990 classification criteria for Takayasu arteritis
      • age at disease onset ≤ 40 years old
      • claudication of extremities, especially upper extremities
      • decreased brachial artery pulse in 1 or both brachial arteries
      • systolic blood pressure difference > 10 mm Hg between arms
      • bruit over subclavian arteries or abdominal aorta
      • abnormal arteriogram (narrowing or occlusion of entire aorta, its primary branches or large arteries in proximal upper or lower extremities, not due to arteriosclerosis, fibromuscular dysplasia or similar causes; changes usually focal or segmental)
      • Reference – Arthritis Rheum 1990 Aug;33(8):1129
  • European League Against Rheumatism/Paediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society (EULAR/PRINTO/PRES) criteria for diagnosis of childhood Takayasu arteritis
    • angiographic abnormalities of aorta or its main branches
    • aneurysm or dilatation of pulmonary arteries
    • one or more of the following
      • pulse deficit or claudication
      • 4 limbs blood pressure discrepancy
      • hypertension
      • bruits
      • acute phase reactant
    • Reference – Ann Rheum Dis 2010 May;69(5):798
  • non invasive disease monitoring using Birmingham Vasculitis Activity Score (BVAS) may be useful in patients with antineutrophil, cytoplasmic, antibody-associated vasculitides, but may be less sensitive in patients with large vessel vasculitides3
    • Birmingham Vasculitis Activity Score
      • based on derivation cohort without validation
      • 213 patients with vasculitis (51 with rheumatoid vasculitis, 28 with granulomatosis with polyangiitis, 22 with nonrenal granulomatosis with polyangiitis, 14 with polyarteritis nodosa, 11 with Takayasu arteritis, 11 with Behçet syndrome, 10 with giant cell arteritis, and 60 with other vasculitides [including Churg-Strauss syndrome, cutaneous vasculitis, unclassified vasculitis]) were assessed
      • Birmingham Vasculitis Activity Score (BVAS) categorizes abnormalities in 9 organ systems (total score range 0-63)
        • systemic symptoms
        • cutaneous symptoms
        • mucous membrane/eye symptoms
        • ear nose throat symptoms
        • chest symptoms
        • cardiovascular symptoms
        • abdominal symptoms
        • renal symptoms
        • nervous system symptoms
      • median BVAS score in patients with
        • 0 (range 0) in 107 patients with inactive disease
        • 7.5 (range 4-30) in 22 patients with active untreated disease
        • 10 (range 1-29) in 69 patients with active treated disease
        • 20.5 (range 9-30) immediately before death in 12 patients who died
      • all patients who died had initial BVAS > 8
      • Reference – QJM 1994 Nov;87(11):671
    • BVAS version 3 uses fewer items, but may have strong correlation to BVAS version 2 and other scoring systems (level 2 [mid-level] evidence)
      • based on validation cohort study
      • 313 patients (mean age 55 years, 52% female) presenting to outpatient clinic with probable or definite systemic vasculitis (median duration 3.8 years) were assessed
        • diagnoses included 155 patients with granulomatosis with polyangiitis, 28 with Churg-Strauss syndrome, 25 with Behçet syndrome, 15 with microscopic polyangiitis, 11 with cutaneous vasculitis, 10 with polyarteritis nodosa, 10 with Henoch-Schönlein purpura, 9 with Takayasu arteritis, 9 with cerebral vasculitis, 8 with rheumatoid vasculitis, and 6 with cryoglobulinemic vasculitis
        • patients were excluded for probable or definite giant cell arteritis
      • BVAS version 3 reduced number of items assessed to 56 from 66 in version 2 and 63 in original BVAS by omitting or merging items, but still included abnormalities in same 9 organ systems (as summarized above) correlating with vasculitis activity
      • 96.5% had recorded treatment decision
      • BVAS version 3 had strong correlation with
        • BVAS version 2 in analysis of 138 patients (correlation coefficient 0.94)
        • physician’s global assessment in analysis of 307 patients (correlation coefficient 0.91)
        • vasculitis activity index in analysis of 304 patients (correlation coefficient 0.88)
      • BVAS version 3 had moderate correlation with treatment decision in analysis of 302 patients (correlation coefficient 0.66)
      • BVAS version 3 had strong repeatability in analysis of 99 patients, reproducibility in analysis of 39 patients assessed twice within 9 days, and sensitivity to change in analysis of 39 patients evaluated at baseline and 3 months (after major treatment escalation)
      • Reference – Ann Rheum Dis 2009 Dec;68(12):1827
      • free online calculator for BVAS-3 provided by University of Oxford
  • National Institutes of Health (NIH) criteria for disease activity in Takayasu arteritis
    • criteria for active disease
      • systemic features, such as fever, musculoskeletal (no other cause identified)
      • elevated erythrocyte sedimentation rate
      • features of vascular ischemia or inflammation, such as claudication, diminished or absent pulsation, bruit, carotodynia, asymmetric blood pressure in either upper or lower limbs (or both)
      • typical angiographic features
    • active disease defined as new onset or worsening of ≥ 2 criteria
    • Reference – Ann Intern Med 1994 Jun 1;120(11):919

References

General references used

  1. Terao C, Yoshifuji H, Mimori T. Recent advances in Takayasu arteritis. Int J Rheum Dis. 2014 Mar;17(3):238-47
  2. Wen D, Du X, Ma CS. Takayasu arteritis: diagnosis, treatment and prognosis. Int Rev Immunol. 2012 Dec;31(6):462-73
  3. Mason JC. Takayasu arteritis–advances in diagnosis and management. Nat Rev Rheumatol. 2010 Jul;6(7):406-15
  4. Mukhtyar C, Guillevin L, Cid MC, et al; European Vasculitis Study Group. EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis. 2009 Mar;68(3):318-23
  5. Keser G, Direskeneli H, Aksu K. Management of Takayasu arteritis: a systematic review. Rheumatology (Oxford). 2014 May;53(5):793-801

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