U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Takayasu arteritis

MedGen UID:
21458
Concept ID:
C0039263
Disease or Syndrome
Synonyms: AORTIC ARCH SYNDROME; PULSELESS DISEASE; Takayasu disease; YOUNG FEMALE ARTERITIS
SNOMED CT: Takayasu disease (359789008); Takayasu's disease (359789008); Acquired aortoarteritis (359789008); Aortic arch arteritis (359789008); Occlusive thromboarteriopathy (359789008); Primary arteritis (359789008); Pulseless disease (359789008); Idiopathic medial aortopathy AND arteriopathy (359789008); Reverse coarctation (359789008); Atypical coarctation (359789008); Takayasu's arteriopathy (359789008); Takayasu's arteritis (359789008); Martorell syndrome (359789008); Nonspecific arteritis (359789008); Sclerosing aortitis AND arteritis (359789008); Nonspecific aortoarteritis (359789008); Raeder-Harbitz syndrome (359789008); Young female arteritis (359789008)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0017991
OMIM®: 207600
Orphanet: ORPHA3287

Definition

A rare, congenital anomaly of the great arteries characterized by cranially situated aortic arch ascending into the neck above the clavicles. Most patients remain asymptomatic, some present with a murmur and a pulsatile neck mass, stridor, dyspnea, recurrent bronchitis, dysphagia or signs and symptoms of a stenosis/aneurism of the aortic arch. Other congenital heart anomalies are frequently associated, including abnormalities of arch laterality and branching, aortic coarctation or aneurysm. [from ORDO]

Clinical features

From HPO
Arteritis
MedGen UID:
13916
Concept ID:
C0003860
Pathologic Function
Arterial inflammation.
  • Abnormality of the cardiovascular system

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTakayasu arteritis
Follow this link to review classifications for Takayasu arteritis in Orphanet.

Professional guidelines

PubMed

Grayson PC, Ponte C, Suppiah R, Robson JC, Gribbons KB, Judge A, Craven A, Khalid S, Hutchings A, Danda D, Luqmani RA, Watts RA, Merkel PA; DCVAS Study Group
Ann Rheum Dis 2022 Dec;81(12):1654-1660. Epub 2022 Nov 9 doi: 10.1136/ard-2022-223482. PMID: 36351705
Kermani TA, Warrington KJ, Dua AB
Rheum Dis Clin North Am 2022 Aug;48(3):705-724. Epub 2022 Jul 5 doi: 10.1016/j.rdc.2022.03.006. PMID: 35953232
Maz M, Chung SA, Abril A, Langford CA, Gorelik M, Guyatt G, Archer AM, Conn DL, Full KA, Grayson PC, Ibarra MF, Imundo LF, Kim S, Merkel PA, Rhee RL, Seo P, Stone JH, Sule S, Sundel RP, Vitobaldi OI, Warner A, Byram K, Dua AB, Husainat N, James KE, Kalot MA, Lin YC, Springer JM, Turgunbaev M, Villa-Forte A, Turner AS, Mustafa RA
Arthritis Rheumatol 2021 Aug;73(8):1349-1365. Epub 2021 Jul 8 doi: 10.1002/art.41774. PMID: 34235884

Recent clinical studies

Etiology

Esatoglu SN, Hatemi G
Curr Opin Rheumatol 2022 Jan 1;34(1):18-24. doi: 10.1097/BOR.0000000000000852. PMID: 34698679
Marquez J, Flores D, Candia L, Espinoza LR
Curr Rheumatol Rep 2003 Apr;5(2):128-35. doi: 10.1007/s11926-003-0040-6. PMID: 12628043
Kobayashi Y, Numano F
Intern Med 2002 Jan;41(1):44-6. doi: 10.2169/internalmedicine.41.44. PMID: 11838591
Fraga A, Medina F
Curr Rheumatol Rep 2002 Feb;4(1):30-8. doi: 10.1007/s11926-002-0021-1. PMID: 11798980
Bengtsson BA, Andersson R
Curr Opin Rheumatol 1991 Feb;3(1):15-22. doi: 10.1097/00002281-199102000-00004. PMID: 1675113

Diagnosis

Ezzeddine FM, Hurst PD, Bookwalter CA
Mayo Clin Proc 2023 Jul;98(7):1033-1034. doi: 10.1016/j.mayocp.2023.03.001. PMID: 37419571
Katekaru-Tokeshi DI, Jiménez-Santos M
Rev Esp Cardiol (Engl Ed) 2022 Jun;75(6):526. Epub 2022 Feb 9 doi: 10.1016/j.rec.2021.11.021. PMID: 35151598
Tombetti E, Mason JC
Rheumatology (Oxford) 2019 Feb 1;58(2):206-219. doi: 10.1093/rheumatology/key040. PMID: 29635396
Keser G, Aksu K, Direskeneli H
Turk J Med Sci 2018 Aug 16;48(4):681-697. doi: 10.3906/sag-1804-136. PMID: 30114347
Numano F, Okawara M, Inomata H, Kobayashi Y
Lancet 2000 Sep 16;356(9234):1023-5. doi: 10.1016/S0140-6736(00)02701-X. PMID: 11041416

Therapy

Monach PA
Rheum Dis Clin North Am 2023 Aug;49(3):713-729. Epub 2023 Apr 15 doi: 10.1016/j.rdc.2023.03.014. PMID: 37331742
Somashekar A, Leung YT
Postgrad Med 2023 Jan;135(sup1):14-21. Epub 2023 Jan 2 doi: 10.1080/00325481.2022.2159723. PMID: 36588528
Torp CK, Brüner M, Keller KK, Brouwer E, Hauge EM, McGonagle D, Kragstrup TW
Autoimmun Rev 2021 Jun;20(6):102829. Epub 2021 Apr 16 doi: 10.1016/j.autrev.2021.102829. PMID: 33872767
Hellmich B, Agueda A, Monti S, Buttgereit F, de Boysson H, Brouwer E, Cassie R, Cid MC, Dasgupta B, Dejaco C, Hatemi G, Hollinger N, Mahr A, Mollan SP, Mukhtyar C, Ponte C, Salvarani C, Sivakumar R, Tian X, Tomasson G, Turesson C, Schmidt W, Villiger PM, Watts R, Young C, Luqmani RA
Ann Rheum Dis 2020 Jan;79(1):19-30. Epub 2019 Jul 3 doi: 10.1136/annrheumdis-2019-215672. PMID: 31270110
Keser G, Aksu K, Direskeneli H
Turk J Med Sci 2018 Aug 16;48(4):681-697. doi: 10.3906/sag-1804-136. PMID: 30114347

Prognosis

Ellacuriaga San Martin J, Geiger PA
Eur J Vasc Endovasc Surg 2020 Mar;59(3):479. Epub 2019 Dec 19 doi: 10.1016/j.ejvs.2019.11.032. PMID: 31865032
Koter SH, Tiesenhausen K
Eur J Vasc Endovasc Surg 2015 Mar;49(3):276. Epub 2014 Oct 11 doi: 10.1016/j.ejvs.2014.09.003. PMID: 25308759
Fraga A, Medina F
Curr Rheumatol Rep 2002 Feb;4(1):30-8. doi: 10.1007/s11926-002-0021-1. PMID: 11798980
Kerr GS
Rheum Dis Clin North Am 1995 Nov;21(4):1041-58. PMID: 8592736
Bengtsson BA, Andersson R
Curr Opin Rheumatol 1991 Feb;3(1):15-22. doi: 10.1097/00002281-199102000-00004. PMID: 1675113

Clinical prediction guides

Pham V, Hemptinne Q, Grinda JM, Duboc D, Varenne O, Picard F
Arch Cardiovasc Dis 2020 Jan;113(1):59-69. Epub 2019 Dec 19 doi: 10.1016/j.acvd.2019.10.008. PMID: 31866173
Keser G, Aksu K, Direskeneli H
Turk J Med Sci 2018 Aug 16;48(4):681-697. doi: 10.3906/sag-1804-136. PMID: 30114347
Lally L, Spiera RF
Rheum Dis Clin North Am 2015 May;41(2):315-31. Epub 2015 Feb 27 doi: 10.1016/j.rdc.2015.01.004. PMID: 25836645
Koter SH, Tiesenhausen K
Eur J Vasc Endovasc Surg 2015 Mar;49(3):276. Epub 2014 Oct 11 doi: 10.1016/j.ejvs.2014.09.003. PMID: 25308759
Blockmans D
Ann N Y Acad Sci 2011 Jun;1228:64-70. doi: 10.1111/j.1749-6632.2011.06021.x. PMID: 21718324

Recent systematic reviews

Dejaco C, Ramiro S, Bond M, Bosch P, Ponte C, Mackie SL, Bley TA, Blockmans D, Brolin S, Bolek EC, Cassie R, Cid MC, Molina-Collada J, Dasgupta B, Nielsen BD, De Miguel E, Direskeneli H, Duftner C, Hočevar A, Molto A, Schäfer VS, Seitz L, Slart RHJA, Schmidt WA
Ann Rheum Dis 2024 May 15;83(6):741-751. doi: 10.1136/ard-2023-224543. PMID: 37550004
de Souza AWS, Sato EI, Brance ML, Fernández-Ávila DG, Scolnik M, Magri SJ, Ugarte-Gil MF, Flores-Suárez LF, Saldarriaga-Rivera LM, Babini A, Zamora NV, Acosta Felquer ML, Vergara F, Carlevaris L, Scarafia S, Soriano Guppy ER, Unizony S
J Clin Rheumatol 2023 Oct 1;29(7):316-325. Epub 2023 Aug 9 doi: 10.1097/RHU.0000000000002004. PMID: 37553869
Zeng L, Yang T, Yang K, Yu G, Li J, Xiang W, Chen H
Front Immunol 2022;13:896476. Epub 2022 Aug 1 doi: 10.3389/fimmu.2022.896476. PMID: 35979355Free PMC Article
Maz M, Chung SA, Abril A, Langford CA, Gorelik M, Guyatt G, Archer AM, Conn DL, Full KA, Grayson PC, Ibarra MF, Imundo LF, Kim S, Merkel PA, Rhee RL, Seo P, Stone JH, Sule S, Sundel RP, Vitobaldi OI, Warner A, Byram K, Dua AB, Husainat N, James KE, Kalot MA, Lin YC, Springer JM, Turgunbaev M, Villa-Forte A, Turner AS, Mustafa RA
Arthritis Rheumatol 2021 Aug;73(8):1349-1365. Epub 2021 Jul 8 doi: 10.1002/art.41774. PMID: 34235884
Torp CK, Brüner M, Keller KK, Brouwer E, Hauge EM, McGonagle D, Kragstrup TW
Autoimmun Rev 2021 Jun;20(6):102829. Epub 2021 Apr 16 doi: 10.1016/j.autrev.2021.102829. PMID: 33872767

Supplemental Content

Table of contents

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...