Kidney nontumor

Vascular disease

Vasculitis

Takayasu arteritis


Editorial Board Member: Jonathan E. Zuckerman, M.D., Ph.D.
Editor-in-Chief: Debra L. Zynger, M.D.
Dale Davis, M.D., M.A.
Nicole K. Andeen, M.D.

Topic Completed: 6 May 2021

Minor changes: 6 May 2021

Copyright: 2019-2021, PathologyOutlines.com, Inc.

PubMed Search: Takayasu arteritis kidney pathology

Dale Davis, M.D., M.A.
Nicole K. Andeen, M.D.
Page views in 2020: 2,922
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Cite this page: Davis D, Andeen NK. Takayasu arteritis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/kidneynontumortakayasuarteritis.html. Accessed November 27th, 2021.
Definition / general
  • Rare large vessel vasculitis
  • Usually occurs in patients younger than 50 years old
  • May present with renovascular hypertension, anecdotal association with wide range of renal glomerulopathies
Essential features
  • Takayasu arteritis usually involves aorta and its major branches
  • Inflammation is granulomatous with lymphoplasmacytic infiltrate and varying numbers of giant cells
  • Renal disease is present in most cases, usually as nonspecific changes due to chronic hypertension and renal artery stenosis and other vascular changes (Clin Exp Rheumatol 2007;25:S10)
Terminology
  • Pulseless disease
ICD coding
  • ICD-10: M31.4 - aortic arch syndrome [Takayasu]
Epidemiology
  • Incidence varies: 0.9/million (U.S.); 4.7 - 33/million (Europe); 40/million (Japan) (Int J Nephrol Renovasc Dis 2018;11:225)
  • F:M also varies; overall is 8.2 - 9.7:1 but is 1.6 - 2:1 in Middle East and India (J Autoimmun 2014;48:79)
    • Women frequently have involvement of the thoracic aorta and its branches
    • Men are more likely to have involvement of abdominal aorta and its branches
  • Patients commonly 20 - 40 years old (Best Pract Res Clin Rheumatol 2010;24:871)
  • Renal artery involvement in Takayasu arteritis is higher in Asian populations and overall ranges from 11.5% to 62%
Sites
  • Aorta and its major branches
  • Kidney
Pathophysiology
  • Genetic predisposition plus environmental factors
  • Virus or bacteria via molecular mimicry mechanism leads to autoimmune response (Pathogens 2018;7:E73)
  • Inflammatory cells implicated include gamma delta, CD4 and CD8 T cells, natural killer cells, macrophages, dendritic cells and B cells (Am J Med Sci 2013;346:314)
  • Autoantibodies include antiaortic, antiendothelial and circulating immune complexes (Autoimmun Rev 2017;16:146)
  • Associated with HLA-B genes, especially HLA-B*52 and HLA-B*67 in certain populations and TNFα genes (Int J Nephrol Renovasc Dis 2018;11:225)
  • 2 stages of disease:
    • Early inflammatory stage with nonspecific symptoms and transmural inflammation
    • Later pulseless phase with hypertension, ischemia and occlusive changes including intimal fibrosis
Etiology
  • Precise etiology unknown; likely multifactorial with genetic component and potentially viral or bacterial instigator (Pathogens 2018;7:E73)
Diagrams / tables

Images hosted on other servers:
Pathogenesis

Pathogenesis

Clinical features
  • Usually subclinical or nonspecific symptoms at disease onset
  • Constitutional symptoms (night sweats, weight loss, fever)
  • Limb fatigue and pain, different or absent pulses in extremities, blood pressure difference or unobtainable blood pressure (J Autoimmun 2014;48:79)
Diagnosis
  • Chapel Hill Consensus Conference 2012: large vessel vasculitis of patients < 50 years old
  • 1990 American College of Rheumatology Classification Criteria for Takayasu arteritis: 3 or more of the following criteria yields a sensitivity of 90.5% and a specificity of 97.8% (Arthritis Rheum 1990;33:1129):
    • Age at disease onset < 40 years
    • Claudication of extremities
    • Decreased brachial artery pulse
    • Blood pressure difference > 10 mm Hg
    • Bruit over subclavian arteries or aorta
    • Arteriogram abnormality
Laboratory
Radiology description
Radiology images

Images hosted on other servers:
ECG gated double inversion recovery ECG gated double inversion recovery ECG gated double inversion recovery ECG gated double inversion recovery

ECG gated double inversion recovery


Fast gradient echo Fast gradient echo

Fast gradient echo

MR angiography MR angiography

MR angiography

Prognostic factors
Case reports
Treatment
Gross description
  • Vascular sclerosis, nonspecific scarring, ill defined white mass-like lesions
Gross images

Contributed by Nicole K. Andeen, M.D.
White lesion

White lesion

Microscopic (histologic) description
Microscopic (histologic) images

Contributed by Nicole K. Andeen, M.D.
Active vasculitis with giant cells Active vasculitis with giant cells

Active vasculitis with giant cells

Tubulointerstitial inflammation Tubulointerstitial inflammation

Tubulointerstitial inflammation


Jones

Jones

Elastic Elastic

Elastic

Positive stains
  • Elastin will show disruption of the elastic lamina
  • Use immune cell markers as needed
Negative stains
  • Fungal, viral, spirochete, acid fast stains to rule out infectious etiologies for granulomatous inflammation
Sample pathology report
  • Right kidney, nephrectomy:
    • Arteritis with granulomatous inflammation and multinucleated giant cells, active and chronic (see comment)
    • Comment: Differential diagnosis for the large vessel vasculitis includes Takayasu arteritis and giant cell arteritis. Other etiologies for the vasculitis include antineutrophil cytoplasmic antibody associated vasculitis, necrotizing sarcoid vasculitis, polyarteritis nodosa or possibly infection. No acid fast or fungal organisms are identified on special stains. Takayasu arteritis usually affects the aorta and its major branches and is more common in women under the age of 50.
Differential diagnosis
Board review style question #1
Which feature associated with Takayasu arteritis helps to distinguish it from other vascular injury processes?

  1. Granulomatous transmural vascular inflammation with giant cells
  2. Granulomatous vascular inflammation with giant cells of arterial media
  3. Positive for antineutrophilic cytoplasmic antibody
  4. Vascular changes limited to arterial intima
Board review style answer #1
A. Granulomatous transmural vascular inflammation with giant cells

Comment here

Reference: Takayasu arteritis
Board review style question #2

The differential diagnosis for this vascular finding is

  1. Antiglomerular basement membrane disease
  2. Diabetic nephropathy
  3. IgA nephropathy
  4. Vasculitis with giant cells, consider Takayasu arteritis
Board review style answer #2
D. Vasculitis with giant cells, consider Takayasu arteritis

Comment here

Reference: Takayasu arteritis
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