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Clinical Manifestations, Differential Diagnosis, and Laboratory Markers

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Large and Medium Size Vessel and Single Organ Vasculitis

Part of the book series: Rare Diseases of the Immune System ((RDIS))

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Abstract

As a large-vessel arteritis, Takayasu’s arteritis (TAK) predominantly affects aorta and its major branches. Arterial stenosis, occlusion, and aneurysms lead to various signs and symptoms such as consitutional features, extremity pain, claudication, light-headedness, bruits, absent or diminished pulses, and loss of blood pressure. As acute-phase reactants, ESR and C-reactive protein are frequently advocated for disease assessment of TAK. Recently, a member of pentraxin family, PTX-3 was suggested to be a discriminative marker for active disease in TAK, with controversial results. Giant-cell arteritis, accelerated atherosclerosis, and various non-inflammatory vascular disorders have clinical similarities with TAK and should be investigated in the differential diagnosis.

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References

  1. Numano F. The story of Takayasu arteritis. Rheumatology (Oxford). 2002;41:103–6.

    CAS  Google Scholar 

  2. Kerr GS, Hallahan CW, Giordano J, Leavitt RY, Fauci AS, et al. Takayasu arteritis. Ann Intern Med. 1994;120(11):919–29.

    CAS  PubMed  Google Scholar 

  3. Bıcakcıgil M, Aksu K, Kamalı S, Ozbalkan Z, Ates A, et al. Takayasu’s arteritis in Turkey—clinical and angiographic features of 248 patients. Clin Exp Rheumatol. 2009;27:S59–64.

    PubMed  Google Scholar 

  4. Alibaz-Oner F, Aydin SZ, Direskeneli H. Advances in the diagnosis, assessment and outcome of Takayasu’s arteritis. Clin Rheumatol. 2013;32(5):541–6.

    PubMed  Google Scholar 

  5. Li J, Li H, Sun F, Chen Z, Yang Y, et al. Clinical characteristics of heart involvement in Chinese patients with Takayasu arteritis. J Rheumatol. 2017;44(12):1867–74.

    PubMed  Google Scholar 

  6. Alibaz-Oner F, On behalf of Turkish Vasculitis Study Group. Vascular damage is less present in an early inception cohort in Takayasu’s arteritis [abstract]. Arthritis Rheumatol. 2019;71(Suppl 10).

    Google Scholar 

  7. Langford C. Takayasu arteritis. In: Hochberg MC, editor. Rheumatology. 7th ed. Philadelphia: Elsevier; 2019. p. 1378–83.

    Google Scholar 

  8. Park MC, Lee SW, Park YB, Chung N, Lee SK. Clinical characteristics and outcomes of Takayasu’s arteritis: analysis of 108 patients using standardized criteria for diagnosis, activity assessment, and angiographic classification. Scand J Rheumatol. 2005;34:284–92.

    PubMed  Google Scholar 

  9. Vanoli M, Daina E, Salvarani C, Sabbadini M, Rossi C, et al. Takayasu’s arteritis: a study of 104 Italian patients. Arthritis Rheum. 2005;53:100–7.

    CAS  PubMed  Google Scholar 

  10. Park YB, Hong SK, Choi KJ, Sohn DW, Oh BH, et al. Takayasu arteritis in Korea. Clinical and angiographic features. Heart Vessels Suppl. 1992;7:55–9.

    CAS  PubMed  Google Scholar 

  11. He Y, Lv N, Dang A, Cheng N. Pulmonary artery involvement in patients with Takayasu arteritis. J Rheumatol. 2019. pii: jrheum.190045.

    Google Scholar 

  12. Zhang Y, Yang K, Meng X, Tian T, Fan P, et al. Cardiac valve involvement in Takayasu arteritis is common: a retrospective study of 1,069 patients over 25 years. Am J Med Sci. 2018;356(4):357–64.

    PubMed  Google Scholar 

  13. Hotchi M. Pathological studies on Takayasu arteritis. Heart Vessels Suppl. 1992;7:11–7.

    CAS  PubMed  Google Scholar 

  14. Sharma BK, Jain S, Radotra BD. An autopsy study of Takayasu arteritis in India. Int J Cardiol. 1998;66(Suppl 1):S85–91.

    PubMed  Google Scholar 

  15. Kalfa M, Emmungil H, Musayev O, Gündüz ÖS, Yılmaz Z, et al. Frequency of pulmonary hypertension in transthoracic echocardiography screening is not increased in Takayasu arteritis: experience from a single center in Turkey. Eur J Rheumatol. 2018;5(4):249–53.

    PubMed  PubMed Central  Google Scholar 

  16. Esen F, Ergelen R, Alibaz-Öner F, Çelik G, Direskeneli H, Kazokoğlu H. Ocular findings and blood flow in patients with Takayasu arteritis: a cross-sectional study. Br J Ophthalmol. 2019;103(7):928–32.

    PubMed  Google Scholar 

  17. Francès C, Boisnic S, Blétry O, Dallot A, Thomas D, et al. Cutaneous manifestations of Takayasu arteritis. A retrospective study of 80 cases. Dermatologica. 1990;181(4):266–72.

    PubMed  Google Scholar 

  18. Pascual-López M, Hernández-Núñez A, Aragüés-Montañés M, Daudén E, Fraga J, García-Díez A. Takayasu’s disease with cutaneous involvement. Dermatology. 2004;208(1):10–5.

    PubMed  Google Scholar 

  19. Lupi-Herrera E, Sanchez-Torres G, Marchushamer J, et al. Takayasu’s arteritis: clinical study of 107 cases. Am Heart J. 1977;93:94–103.

    CAS  PubMed  Google Scholar 

  20. Riviere E, Arnaud L, Ebbo M, Allanore Y, Claudepierre P, Dernis E, et al. Takayasu arteritis and spondyloarthritis: coincidence or association? A study of 14 cases. J Rheumatol. 2017;44:1011–7.

    PubMed  Google Scholar 

  21. Kilic L, Kalyoncu U, Karadag O, Akdogan A, Dogan I, et al. Inflammatory bowel diseases and Takayasu’s arteritis: coincidence or association? Int J Rheum Dis. 2016;19(8):814–8.

    CAS  PubMed  Google Scholar 

  22. Kanıtez NA, Toz B, Güllüoğlu M, Erer B, Esen BA, et al. Microscopic colitis in patients with Takayasu’s arteritis: a potential association between the two disease entities. Clin Rheumatol. 2016;35(10):2495.

    PubMed  Google Scholar 

  23. Güzel Esen S, Armagan B, Atas N, Ucar M, Varan Ö, et al. Increased incidence of spondyloarthropathies in patients with Takayasu arteritis: a systematic clinical survey. Joint Bone Spine. 2019;86(4):497–501.

    PubMed  Google Scholar 

  24. Grayson PC, Tomasson G, Cuthbertson D, Carette S, Hoffman GS, Khalidi NA, et al. Vasculitis Clinical Research Consortium. Association of vascular physical examination findings and arteriographic lesions in large vessel vasculitis. J Rheumatol. 2012;39(2):303–9.

    PubMed  Google Scholar 

  25. Michailidou D, Rosenblum JS, Rimland CA, Marko J, Ahlman MA, Grayson PC. Clinical symptoms and associated vascular imaging findings in Takayasu’s arteritis compared to giant cell arteritis. Ann Rheum Dis. 2020;79(2):262–7. https://doi.org/10.1136/annrheumdis-2019-216145.

    Article  CAS  PubMed  Google Scholar 

  26. Salvarani C, Cantini F, Boiardi L, Hunder GG. Laboratory investigations useful in giant cell arteritis and Takayasu’s arteritis. Clin Exp Rheumatol. 2003;21(Suppl 32):S23–8.

    CAS  PubMed  Google Scholar 

  27. Mason JC. Takayasu arteritis-advances in diagnosis and management. Nat Rev Rheumatol. 2010;6(7):406–15.

    PubMed  Google Scholar 

  28. Hoffman GS, Ahmed AE. Surrogate markers of disease activity in patients with Takayasu arteritis. A preliminary report from The International Network for the Study of the Systemic Vasculitides (INSSYS). Int J Cardiol. 1998;66(Suppl 1):S191–5.

    PubMed  Google Scholar 

  29. Maksimowicz-Mckinnon K, Clark TM, Hoffman GS. Limitations of therapy and a guarded prognosis in an American cohort of Takayasu arteritis patients. Arthritis Rheum. 2007;56:1000–9.

    PubMed  Google Scholar 

  30. Dhingra R, Talwar KK, Chopra P, Kumar R. An enzyme linked immunosorbent assay for detection of anti-aorta antibodies in Takayasu arteritis patients. Int J Cardiol. 1993;40:237–42.

    CAS  PubMed  Google Scholar 

  31. Lee SK. Anti-endothelial cell antibodies and antiphospholipid antibodies in Takayasu’s arteritis: correlations of their titers and isotype distributions with disease activity. Clin Exp Rheumatol. 2006;24(Suppl 41):S10–6.

    PubMed  Google Scholar 

  32. Chauhan SK, Tripathy NK, Nityanand S. Antigenic targets and pathogenicity of antiaortic endothelial cell antibodies in Takayasu arteritis. Arthritis Rheum. 2006;54:2326–33.

    CAS  PubMed  Google Scholar 

  33. Noris M, Daina E, Gamba S, Bonazzola S, Remuzzi G. Interleukin-6 and RANTES in Takayasu arteritis: a guide for therapeutic decisions? Circulation. 1999;100:55–60.

    CAS  PubMed  Google Scholar 

  34. Park MC, Lee SW, Park YB, Lee SK. Serum cytokine profiles and their correlations with disease activity in Takayasu’s arteritis. Rheumatology. 2006;45:545–8.

    CAS  PubMed  Google Scholar 

  35. Tripathy N, Sinha N, Nityanand S. Interleukin-8 in Takayasu’s arteritis: plasma levels and relationship with disease activity. Clin Exp Rheumatol. 2004;22:S27–30.

    CAS  PubMed  Google Scholar 

  36. Nishino Y, Tamai M, Kawakami A, et al. Serum levels of BAFF for assessing the disease activity of Takayasu arteritis. Clin Exp Rheumatol. 2010;28:14–7.

    PubMed  Google Scholar 

  37. Alibaz-Oner F, Yentur SP, Saruhan-Direskeneli G, Direskeneli H. Serum cytokine profiles in Takayasu’s arteritis: a search for a biomarker. Clin Exp Rheumatol. 2015;33(Suppl 89):32–5.

    Google Scholar 

  38. Garlanda C, Bottazzi B, Bastone A, Mantovani A. Pentraxins at the crossroads between innate immunity, inflammation, matrix deposition, and female fertility. Annu Rev Immunol. 2005;23:337–66.

    CAS  PubMed  Google Scholar 

  39. Ishihara T, Haraguchi G, Kamiishi T, Tezuka D, Inagaki H, Isobe M. Sensitive assessment of activity of Takayasu’s arteritis by pentraxin3, a new biomarker. J Am Coll Cardiol. 2011;57(16):1712–3. No abstract available.

    PubMed  Google Scholar 

  40. Dagna L, Salvo F, Tiraboschi M, Bozzolo EP, Franchini S, et al. Pentraxin-3 as a marker of disease activity in Takayasu arteritis. Ann Intern Med. 2011;155(7):425–33.

    PubMed  Google Scholar 

  41. Alibaz-Oner F, Aksu K, Yentur SP, Keser G, Saruhan-Direskeneli G, Direskeneli H. Plasma pentraxin-3 levels in patients with Takayasu’s arteritis during routine follow-up. Clin Exp Rheumatol. 2016;34(Suppl 97):S73–6.

    PubMed  Google Scholar 

  42. Tombetti E, Di Chio MC, Sartorelli S, Papa M, Salerno A, et al. Systemic pentraxin-3 levels reflect vascular enhancement and progression in Takayasu arteritis. Arthritis Res Ther. 2014;16:479.

    PubMed  PubMed Central  Google Scholar 

  43. Arent WP, Michel BA, Bloch DA, Hunder GG, Calabrese LH, et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum. 1990;33:1129–34.

    Google Scholar 

  44. Nazareth R, Mason J. Takayasu arteritis: severe consequences of delayed diagnosis. QJM. 2010;104:797–800.

    PubMed  Google Scholar 

  45. Arnaud L, Haroche J, Mathian A, Gorochov G, Amoura Z. Pathogenesis of Takayasu’s arteritis: a 2011 update. Autoimmun Rev. 2011;11:61–7.

    CAS  PubMed  Google Scholar 

  46. Maksimowicz-McKinnon K, Clark TM, Hoffman GS. Takayasu arteritis and giant cell arteritis: a spectrum within the same disease? Medicine. 2009;88:221–6.

    PubMed  Google Scholar 

  47. Furuta S, Cousins C, Chaudhry A, Jayne D. Clinical features and radiological findings in large vessel vasculitis: are Takayasu arteritis and giant cell arteritis 2 different diseases or a single entity? J Rheumatol. 2015;42:300–8.

    PubMed  Google Scholar 

  48. Grayson PC, Maksimowicz-McKinnon K, Clark TM, Tomasson G, Cuthbertson D, et al. Distribution of arterial lesions in Takayasu’s arteritis and giant cell arteritis. Ann Rheum Dis. 2012;71:1329–34.

    PubMed  PubMed Central  Google Scholar 

  49. Keser G, Aksu K, Direskeneli H. Takayasu arteritis: an update. Turk J Med Sci. 2018;48(4):681–97.

    CAS  PubMed  Google Scholar 

  50. Tavora F, Burke A. Review of isolated ascending aortitis: differential diagnosis, including syphilitic, Takayasu’s and giant cell aortitis. Pathology (Phila). 2006;38(4):302–8.

    Google Scholar 

  51. Tsui KL, Lee KW, Chan WK, Chan HK, Hon SF, et al. Behçet’s aortitis and aortic regurgitation: a report of two cases. J Am Soc Echocardiogr. 2004;17(1):83–6.

    PubMed  Google Scholar 

  52. Sahutoglu T, Artim Esen B, Aksoy M, Kurtoglu M, Poyanli A, Gul A. Clinical course of abdominal aortic aneurysms in Behçet disease: a retrospective analysis. Rheumatol Int. 2019;39(6):1061–7.

    CAS  PubMed  Google Scholar 

  53. Stone JH, Khosroshahi A, Deshpande V, Stone JR. IgG4-related systemic diseaseaccounts for a significant proportion of thoracic lymphoplasmacytic aortitis cases. Arthritis Care Res. 2010;62(3):316–22.

    Google Scholar 

  54. Kaneko S, Yamashita H, Sugimori Y, Takahashi Y, Kaneko H, et al. Rheumatoid arthritis-associated aortitis: a case report and literature review. Springerplus. 2014;3:509.

    PubMed  PubMed Central  Google Scholar 

  55. Brinster DR, Grizzard JD, Dash A. Lupus aortitis leading to aneurysmal dilatation in the aortic root and ascending aorta. Heart Surg Forum. 2009;12(2):E105–8.

    PubMed  Google Scholar 

  56. Sokalski D, Spring TC, Roberts W. Large artery inflammation in systemic lupus erythematosus. Lupus. 2013;22(9):953–6.

    CAS  PubMed  Google Scholar 

  57. Loricera J, Blanco R, Hernández JL, Carril JM, Martínez-Rodríguez I, et al. Non-infectious aortitis: a report of 32 cases from a single tertiary centre in a 4-year period and literature review. Clin Exp Rheumatol. 2015;33(2 Suppl 89):S-19–31. Epub 2014 Dec 1.

    Google Scholar 

  58. Chirinos JA, Tamariz LJ, Lopes G, Del Carpio F, Zhang X, et al. Large vessel involvement in ANCA-associated vasculitides: report of a case and review of the literature. Clin Rheumatol. 2004;23(2):152–9.

    PubMed  Google Scholar 

  59. Grewal GS, Leipsic J, Klinkhoff AV. Abdominal aortitis in HLA-B27+ spondyloarthritis: case report with 5-year follow-up and literature review. Semin Arthritis Rheum. 2014;44(3):305–8.

    PubMed  Google Scholar 

  60. Tufan A, Tezcan ME, Kaya A, Mercan R, Oner Y, Ozturk MA. Aortitis in a patient with psoriatic arthritis. Mod Rheumatol. 2012;22(5):774–7.

    PubMed  Google Scholar 

  61. Chapelon-Abric C, Saadoun D, Marie I, Comarmond C, DesboisAC, et al. Sarcoidosis with Takayasu arteritis: a model of overlapping granulomatosis. A report of seven cases and literature review. Int J Rheum Dis. 2018;21(3):740–5.

    PubMed  Google Scholar 

  62. Gasparovic H, Djuric Z, Bosnic D, Petricevic M, Brida M, et al. Aortic root vasculitis associated with Cogan’s syndrome. Ann Thorac Surg. 2011;92(1):340–1.

    PubMed  Google Scholar 

  63. Erdogan M, Esatoglu SN, Hatemi G, Hamuryudan V. Aortic involvement in relapsing polychondritis: case-based review. Rheumatol Int. 2019. https://doi.org/10.1007/s00296-019-04468-5.

  64. Delay C, Schwein A, Lejay A, Gaertner S, Aleil B, et al. Aortitis and aortic occlusion in Crohn disease. Ann Vasc Surg. 2015;29(2):365.e5–9.

    Google Scholar 

  65. Kudo T, Aoyagi Y, Fujii T, Ohtsuka Y, Nagata S, Shimizu T. Ulcerative colitis and aortitis syndrome. Pediatr Int. 2010;52(1):e43–5.

    CAS  PubMed  Google Scholar 

  66. Talarico R, Boiardi L, Pipitone N, d’Ascanio A, Stagnaro C, et al. Isolated aortitis versus giant cell arteritis: are they really two sides of the same coin? Clin Exp Rheumatol. 2014;32(3 Suppl 82):S55–8.

    PubMed  Google Scholar 

  67. Mertz LE, Conn DL. Vasculitis associated with malignancy. Curr Opin Rheumatol. 1992;4(1):39–46.

    CAS  PubMed  Google Scholar 

  68. Dijkema EJ, Leiner T, Grotenhuis HB. Diagnosis, imaging and clinical management of aortic coarctation. Heart. 2017;103(15):1148–55.

    PubMed  Google Scholar 

  69. Delis KT, Gloviczki P. Middle aortic syndrome: from presentation to contemporary open surgical and endovascular treatment. Perspect Vasc Surg Endovasc Ther. 2005;17(3):187–203.

    PubMed  Google Scholar 

  70. Olin JW, Sealove BA. Diagnosis management, and future developments offibromuscular dysplasia. J Vasc Surg. 2011;53(3):826–36.e1.

    PubMed  Google Scholar 

  71. Slovut DP, Olin JW. Fibromuscular dysplasia. N Engl J Med. 2004;350:1862–71.

    CAS  PubMed  Google Scholar 

  72. Keser G, Aksu K. Diagnosis and differential diagnosis of large-vessel vasculitides. Rheumatol Int. 2019;39(2):169–85.

    PubMed  Google Scholar 

  73. Shenouda M, Riga C, Naji Y, et al. Segmental arterial mediolysis: a systematic review of 85 cases. Ann Vasc Surg. 2014;28:269–77.

    PubMed  Google Scholar 

  74. Slavin RE, Saeki K, Bhagavan B, Maas AE. Segmental arterial mediolysis: a precursor to fibromuscular dysplasia? Mod Pathol. 1995;8:287–94.

    CAS  PubMed  Google Scholar 

  75. Baker-LePain JC, Stone DH, Mattis AN, Nakamura MC, Fye KH. Clinical diagnosis of segmental arterial mediolysis: differentiation from vasculitis and other mimics. Arthritis Care Res (Hoboken). 2010;62(11):1655–60.

    Google Scholar 

  76. Skeik N, Olson SL, Hari G, Pavia ML. Segmental arterial mediolysis (SAM): systematic review and analysis of 143 cases. Vasc Med. 2019;24(6):549–63.

    PubMed  Google Scholar 

  77. Khasnis A. Mimics of primary systemic vasculitides. Int J Clin Rheumatol. 2009;4(5):597–609.

    Google Scholar 

  78. Milewicz DM, Dietz HC, Miller DC. Treatment of aortic disease in patients with Marfan syndrome. Circulation. 2005;111(11):e150–7.

    PubMed  Google Scholar 

  79. Pepin M, Schwarze U, Superti-Furga A, Byers PH. Clinical and genetic features of Ehlers–Danlos syndrome type IV, the vascular type. N Engl J Med. 2000;342(10):673–80.

    CAS  PubMed  Google Scholar 

  80. Loeys BL, Schwarze U, Holm T, Callewaert BL, Thomas GH, et al. Aneurysm syndromes caused by mutations in the TGF-βreceptor. N Engl J Med. 2006;355(8):788–98.

    CAS  PubMed  Google Scholar 

  81. Williams VC, Lucas J, Babcock MA, Gutmann DH, Korf B, Maria BL. Neurofibromatosis type 1 revisited. Pediatrics. 2009;123(1):124–33.

    PubMed  Google Scholar 

  82. Borofsky S, Levy LM. Neurofibromatosis: types 1 and 2. Am J Neuroradiol. 2013;34(12):2250–1.

    CAS  PubMed  Google Scholar 

  83. Estrada-Veras JI, O’Brien KJ, Boyd LC, Dave RH, Durham BH, et al. The clinical spectrum of Erdheim–Chester disease: an observational cohort study. Blood Adv. 2017;1(6):357–66.

    CAS  PubMed  PubMed Central  Google Scholar 

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Authors and Affiliations

  1. Division of Rheumatology, Department of Internal Medicine, Marmara University, School of Medicine, Istanbul, Turkey

    Fatma Alibaz-Oner & Haner Direskeneli

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  1. Fatma Alibaz-Oner
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  1. Rheumatology Unit, Azienda Unita’ Sanitaria Locale IRCCS di Reggio Emilia, Reggio Emilia, Italy

    Carlo Salvarani

  2. Rheumatology Unit, Azienda Unita’ Sanitaria Locale IRCCS di Reggio Emilia, Reggio Emilia, Italy

    Luigi Boiardi

  3. Rheumatology Unit, Azienda Unita’ Sanitaria Locale IRCCS di Reggio Emilia, Reggio Emilia, Italy

    Francesco Muratore

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Alibaz-Oner, F., Direskeneli, H. (2021). Clinical Manifestations, Differential Diagnosis, and Laboratory Markers. In: Salvarani, C., Boiardi, L., Muratore, F. (eds) Large and Medium Size Vessel and Single Organ Vasculitis. Rare Diseases of the Immune System. Springer, Cham. https://doi.org/10.1007/978-3-030-67175-4_9

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