Kidney nontumor
Vasculitis
Takayasu arteritis

Senior Author: Nicole K. Andeen, M.D.
Editor-in-Chief: Debra Zynger, M.D.
Dale Davis, M.A.
Nicole K. Andeen, M.D.

Topic Completed: 27 March 2019

Revised: 3 June 2019

Copyright: 2019, PathologyOutlines.com, Inc.

PubMed Search: Takayasu arteritis kidney


Dale Davis, M.A.
Nicole K. Andeen, M.D.
Page views in 2019 to date: 1,260
Cite this page: Davis D, Andeen NK. Takayasu arteritis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/kidneynontumortakayasuarteritis.html. Accessed December 5th, 2019.
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 (US); 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 TA 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;346:314)
  • Auto-antibodies include anti-aortic, anti-endothelial 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 TNF-α genes (Int J Nephrol Renovasc Dis 2018;11:225)
  • Two 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

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)
Diagnostic criteria
  • 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


Fast gradient echo

MR angiography

Prognostic factors
Case reports
Treatment
  • Glucocorticoids and other immunosuppressive therapy including methotrexate, azathioprine and cyclophosphamide
  • New studies show success with biologic therapy: anti-TNF-α, Anti-IL-6R and monoclonal antibodies (Curr Opin Rheumatol 2014;26:7)
Gross description
  • Vascular sclerosis, nonspecific scarring, ill defined white mass-like lesions
Gross images

Contributed by Nicole K. Andeen, M.D.

White lesion

Microscopic (histologic) description
  • Granulomatous vasculitis with mixed inflammatory infiltrate, including giant cells, with necrosis, disruption of the elastic lamina and features of recanalization (Int J Nephrol Renovasc Dis 2018;11:225)
  • Varying renal vascular and parenchymal changes associated with the degree of chronic hypertension and renal ischemia: atherosclerosis, ischemic change, atrophy, infiltrating lymphocytes, tubular dilation and narrowing and glomerulosclerosis
  • Specific glomerular and tubulointerstitial changes are rare and often manifestations of chronic disease (Chin Med Sci J 2009;24:69)
  • Reported glomerular disease includes: mesangial proliferative glomerulonephritis, IgA nephropathy, membranous glomerulonephropathy, membranoproliferative glomerulonephropathy, crescentic glomerulonephropathy, renal amyloidosis and focal glomerulosclerosis (Clin Exp Rheumatol 2007;25:S10)
Microscopic (histologic) images

Contributed by Nicole K. Andeen, M.D.

H&E

Tubulointerstitial inflammation


Jones

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
  • Giant cell arteritis
    • Patient population usually > 50 years, M:F = 1:2
    • More common in Caucasian populations
    • Temporal artery most frequently involved
    • Usually presents with polymyalgia rheumatica and localized headache
    • Giant cell arteritis seen in medial layer rather than transmural (Ann Rheum Dis 2012;71:1329, Medicine (Baltimore) 2009;88:221)
  • Fibromuscular dysplasia
    • Younger female patients
    • Multiple arteries are involved including renal and carotid
    • Fibromuscular thickening of media with irregular thinning and loss of internal elastic lamina
    • Not a large vessel vasculitis
    • Aorta is usually not involved (J Am Soc Hypertens 2018;12:506)
  • Atherosclerosis
    • Usually older patients
    • Primarily intimal sclerosis, eccentric (Int J Cardiol 2013;168:3)
    • Not a large vessel vasculitis
  • Infectious vasculitis
    • Rickettsial, bacterial, fungal, viral organisms
    • Not typically a large vessel vasculitis
  • Granulomatous vasculitis due to sarcoidosis
  • Isolated granulomatous arteritis
Board review question #1
Which feature is not associated with Takayasu arteritis and helps distinguish it from other vascular injury processes?

  1. Granulomatous interstitial inflammation
  2. Granulomatous transmural vascular inflammation with giant cells
  3. Marked arterial stenosis and features of recanalization
  4. Vascular changes limited to arterial intima
Board review answer #1
Board review 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 answer #2
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