U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Arteritis

MedGen UID:
13916
Concept ID:
C0003860
Pathologic Function
Synonyms: Arterial Inflammation; Arteritides; Inflammation, Arterial
SNOMED CT: Inflammation of artery (52089001); Arteritis (52089001)
 
HPO: HP:0012089
Monarch Initiative: MONDO:0043494

Definition

Arterial inflammation. [from HPO]

Conditions with this feature

Takayasu arteritis
MedGen UID:
21458
Concept ID:
C0039263
Disease or Syndrome
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.
Granulocytopenia with immunoglobulin abnormality
MedGen UID:
383874
Concept ID:
C1856263
Disease or Syndrome
Immunodeficiency-59 and hypoglycemia (IMD59) is an autosomal recessive primary immunologic disorder characterized by combined immunodeficiency and recurrent septic infections of the respiratory tract, skin, and mucous membranes, as well as disturbed glucose metabolism. Granulocytopenia and B-cell and dendritic cell deficiency are present (Haapaniemi et al., 2017).
VEXAS syndrome
MedGen UID:
1765785
Concept ID:
C5435753
Disease or Syndrome
VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome) is an adult-onset inflammatory disease that primarily affects males and is caused by somatic, not germline, mutations. The disorder is characterized by adult onset of rheumatologic symptoms at a mean age of 64 years. Features include recurrent fevers, pulmonary and dermatologic inflammatory manifestations, vasculitis, deep vein thrombosis, arthralgias, and ear and nose chondritis. Laboratory studies indicate hematologic abnormalities, including macrocytic anemia, as well as increased levels of acute-phase reactants; about half of patients have positive autoantibodies. Bone marrow biopsy shows degenerative vacuolization restricted to myeloid and erythroid precursor cells, as well as variable hematopoietic dyspoiesis and dysplasias. The condition does not respond to rheumatologic medications and the features may result in premature death (summary by Beck et al., 2020).

Professional guidelines

PubMed

Somashekar A, Leung YT
Postgrad Med 2023 Jan;135(sup1):14-21. Epub 2023 Jan 2 doi: 10.1080/00325481.2022.2159723. PMID: 36588528
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
McCrindle BW, Rowley AH, Newburger JW, Burns JC, Bolger AF, Gewitz M, Baker AL, Jackson MA, Takahashi M, Shah PB, Kobayashi T, Wu MH, Saji TT, Pahl E; American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young; Council on Cardiovascular and Stroke Nursing; Council on Cardiovascular Surgery and Anesthesia; and Council on Epidemiology and Prevention
Circulation 2017 Apr 25;135(17):e927-e999. Epub 2017 Mar 29 doi: 10.1161/CIR.0000000000000484. PMID: 28356445

Recent clinical studies

Etiology

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
Esatoglu SN, Hatemi G
Curr Opin Rheumatol 2022 Jan 1;34(1):18-24. doi: 10.1097/BOR.0000000000000852. PMID: 34698679
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
Saadoun D, Vautier M, Cacoub P
Circulation 2021 Jan 19;143(3):267-282. doi: 10.1161/CIRCULATIONAHA.120.046657. PMID: 33464968
Hayreh SS
Prog Retin Eye Res 2009 Jan;28(1):34-62. Epub 2008 Nov 27 doi: 10.1016/j.preteyeres.2008.11.002. PMID: 19063989

Diagnosis

Pepper K
Postgrad Med 2023 Jan;135(sup1):22-32. Epub 2023 Apr 6 doi: 10.1080/00325481.2023.2190288. PMID: 37021621
Somashekar A, Leung YT
Postgrad Med 2023 Jan;135(sup1):14-21. Epub 2023 Jan 2 doi: 10.1080/00325481.2022.2159723. PMID: 36588528
Rebello A, Joshi P
N Engl J Med 2022 Oct 13;387(15):e36. Epub 2022 Oct 8 doi: 10.1056/NEJMicm2202567. PMID: 36214594
Ling ML, Yosar J, Lee BW, Shah SA, Jiang IW, Finniss A, Allende A, Francis IC
Clin Exp Optom 2020 Sep;103(5):572-582. Epub 2019 Oct 29 doi: 10.1111/cxo.12975. PMID: 31663193
Younger DS
Neurol Clin 2019 May;37(2):335-344. Epub 2019 Mar 16 doi: 10.1016/j.ncl.2019.01.008. PMID: 30952412

Therapy

Espígol-Frigolé G, Dejaco C, Mackie SL, Salvarani C, Matteson EL, Cid MC
Lancet 2023 Oct 21;402(10411):1459-1472. Epub 2023 Oct 10 doi: 10.1016/S0140-6736(23)01310-7. PMID: 37832573
Pepper K
Postgrad Med 2023 Jan;135(sup1):22-32. Epub 2023 Apr 6 doi: 10.1080/00325481.2023.2190288. PMID: 37021621
Somashekar A, Leung YT
Postgrad Med 2023 Jan;135(sup1):14-21. Epub 2023 Jan 2 doi: 10.1080/00325481.2022.2159723. PMID: 36588528
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
Ciofalo A, Gulotta G, Iannella G, Pasquariello B, Manno A, Angeletti D, Pace A, Greco A, Altissimi G, de Vincentiis M, Magliulo G
Curr Rheumatol Rev 2019;15(4):259-268. doi: 10.2174/1573397115666190227194014. PMID: 30827250

Prognosis

Colombo MG, Wetzel AJ, Haumann H, Dally S, Kirtschig G, Joos S
Dtsch Arztebl Int 2022 Jun 17;119(24):411-417. doi: 10.3238/arztebl.m2022.0218. PMID: 35635433Free PMC Article
Rosenberg H, Halman S, Yadav K
CMAJ 2021 Nov 22;193(46):E1770. doi: 10.1503/cmaj.210541. PMID: 34810164Free PMC Article
Iorga ER, Costin D
Rom J Ophthalmol 2020 Oct-Dec;64(4):323-332. doi: 10.22336/rjo.2020.54. PMID: 33367170Free PMC Article
Newburger JW
Congenit Heart Dis 2017 Sep;12(5):633-635. Epub 2017 Jun 5 doi: 10.1111/chd.12498. PMID: 28580712
Salvarani C, Cantini F, Hunder GG
Lancet 2008 Jul 19;372(9634):234-45. doi: 10.1016/S0140-6736(08)61077-6. PMID: 18640460

Clinical prediction guides

Misra DP, Singh K, Rathore U, Kavadichanda CG, Ora M, Jain N, Agarwal V
Best Pract Res Clin Rheumatol 2023 Mar;37(1):101826. Epub 2023 May 26 doi: 10.1016/j.berh.2023.101826. PMID: 37246052
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
McCrindle BW, Rowley AH, Newburger JW, Burns JC, Bolger AF, Gewitz M, Baker AL, Jackson MA, Takahashi M, Shah PB, Kobayashi T, Wu MH, Saji TT, Pahl E; American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young; Council on Cardiovascular and Stroke Nursing; Council on Cardiovascular Surgery and Anesthesia; and Council on Epidemiology and Prevention
Circulation 2017 Apr 25;135(17):e927-e999. Epub 2017 Mar 29 doi: 10.1161/CIR.0000000000000484. PMID: 28356445
Schmidt WA
Clin Exp Rheumatol 2014 Jan-Feb;32(1 Suppl 80):S71-7. Epub 2014 Feb 17 PMID: 24529335

Recent systematic reviews

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
Dejaco C, Ramiro S, Duftner C, Besson FL, Bley TA, Blockmans D, Brouwer E, Cimmino MA, Clark E, Dasgupta B, Diamantopoulos AP, Direskeneli H, Iagnocco A, Klink T, Neill L, Ponte C, Salvarani C, Slart RHJA, Whitlock M, Schmidt WA
Ann Rheum Dis 2018 May;77(5):636-643. Epub 2018 Jan 22 doi: 10.1136/annrheumdis-2017-212649. PMID: 29358285
Buttgereit F, Dejaco C, Matteson EL, Dasgupta B
JAMA 2016 Jun 14;315(22):2442-58. doi: 10.1001/jama.2016.5444. PMID: 27299619

Supplemental Content

Table of contents

    Clinical resources

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

    Consumer resources

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...