Advertisement
Canadian Journal of Cardiology

Diagnostic and Therapeutic Challenges of Vasculitis

Published:February 11, 2022DOI:https://doi.org/10.1016/j.cjca.2022.02.007

      Abstract

      Systemic vasculitis can be a challenge to differentiate from other forms of vasculopathy. Because treatment for systemic vasculitis is disparate from that for other forms of vasculopathy, clinicians should strive for high diagnostic certainty. This review article aims to highlight the clinical, radiographic, and histologic clues to distinguish systemic vasculitis from mimics. Vasculitis should be considered in patients with preexisting conditions including autoimmune connective tissue diseases, multisystem manifestations, unexplained ischemic events, unusual radiographic findings, or signs of systemic inflammation. A multidisciplinary approach can be used among rheumatologists, vascular cardiologists, radiologists, and vascular interventionalists to raise diagnostic certainty in cases with large- and/or medium-vessel involvement. Recognition of cardiac manifestations, including myocarditis seen in forms of small-vessel vasculitis (eg, eosinophilic granulomatosis with polyangiitis) or coronary arteritis seen in forms of medium-vessel vasculitis (eg, polyarteritis nodosa and Kawasaki disease) is important owing to the associated mortality. Clinical phenotype, radiographic features, laboratory tests, and histology can help to differentiate vasculitis from noninflammatory vasculopathies and define the etiology of the vasculitis to help guide appropriate treatment. Various modalities of imaging can give clues to aid in diagnosis of vasculitis and can be considered in the context of physician preference and patient comorbidity. While conventional angiography can give important details regarding luminal anatomy and pressure gradients in medium- and large-vessel vasculitis, noninvasive imaging modalities such as computed tomographic angiography, magnetic resonance angiography, color Doppler ultrasound, and positron emission tomography/computed tomography are commonly used for both diagnosis and follow-up. Treatment for systemic vasculitis should be coordinated with an experienced rheumatologist.

      Résumé

      Il peut être difficile de distinguer la vascularite systémique des autres formes de vasculopathie. Le traitement de la vascularite systémique étant différent de celui des autres formes de vasculopathie, les cliniciens doivent s’efforcer de confirmer le plus possible le diagnostic. La présente synthèse vise à mettre en évidence les indices cliniques, radiographiques et histologiques qui permettent de distinguer la vascularite systémique des autres affections qui lui ressemblent. Une vascularite doit être envisagée chez les personnes atteintes d’affections préexistantes, comme une maladie auto-immune du tissu conjonctif, des manifestations multisystémiques, des événements ischémiques inexpliqués, des observations anormales à la radiographie ou des signes d’inflammation généralisée. Une approche pluridisciplinaire peut être adoptée par les rhumatologues, les cardiologues vasculaires, les radiologues ainsi que les médecins interventionnels spécialisés dans la prise en charge des affections vasculaires afin d’accroître la certitude du diagnostic en cas d’atteinte de vaisseaux sanguins de gros ou de moyen calibre. Étant donné la mortalité qui leur est associée, il est important de déceler les manifestations cardiaques, y compris la myocardite accompagnant certaines formes de vascularite des vaisseaux de petit calibre (p. ex. granulomatose éosinophile avec polyangéite) et la coronarite accompagnant certaines formes de vascularite des vaisseaux de moyen calibre (p. ex., polyartérite noueuse et maladie de Kawasaki). Le phénotype clinique et les caractéristiques radiographiques de la maladie ainsi que les épreuves de laboratoire et l’analyse histologique peuvent aider à différencier la vascularite des vasculopathies non inflammatoires et à déterminer la cause de la vascularite afin d’aiguiller vers le traitement approprié. Diverses techniques d’imagerie peuvent fournir des indices pour faciliter le diagnostic de vascularite et être envisagées selon les préférences du médecin et des affections concomitantes du patient. Bien qu’une angiographie classique puisse donner de précieuses indications au sujet de l’anatomie luminale et des gradients de pression dans les vaisseaux de moyen et de gros calibre atteints par la vascularite, les techniques d’imagerie non invasives comme l’angiographie par tomodensitométrie, l’angiographie par résonance magnétique, l’échographie Doppler couleur ainsi que la tomographie par émission de positons et la tomodensitométrie sont couramment utilisées pour le diagnostic et le suivi. Le traitement de la vascularite systémique doit être coordonné avec un rhumatologue d’expérience.
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Canadian Journal of Cardiology
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Jennette J.C.
        • Falk R.J.
        • Bacon P.A.
        • et al.
        2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides.
        Arthritis Rheum. 2013; 65: 1-11
        • Xie Y.
        • Seidman M.A.
        Inflammatory comments in coronary artery disease: when to suspect polyarteritis nodosa or other primary systemic vasculitis.
        Can J Cardiol. 2021; 37: 830-832
        • Gonzalez-Gay M.A.
        • Vazquez-Rodriguez T.R.
        • Lopez-Diaz M.J.
        • et al.
        Epidemiology of giant cell arteritis and polymyalgia rheumatica.
        Arthritis Rheum. 2009; 61: 1454-1461
        • Kerr G.S.
        • Hallahan C.W.
        • Giordano J.
        • et al.
        Takayasu arteritis.
        Ann Intern Med. 1994; 120: 919-929
        • Rutter M.
        • Bowley J.
        • Lanyon P.C.
        • Grainge M.J.
        • Pearce F.A.
        A systematic review and meta-analysis of the incidence rate of Takayasu arteritis.
        Rheumatology (Oxford). 2021; 60: 4982-4990
        • Gonzalez-Gay M.A.
        • Barros S.
        • Lopez-Diaz M.J.
        • et al.
        Giant cell arteritis: disease patterns of clinical presentation in a series of 240 patients.
        Medicine. 2005; 84: 269-276
        • Kim H.
        • Barra L.
        Ischemic complications in Takayasu’s arteritis: a meta-analysis.
        Semin Arthritis Rheum. 2018; 47: 900-906
        • Lei C.
        • Huang Y.
        • Yuan S.
        • et al.
        Takayasu arteritis with coronary artery involvement: differences between pediatric and adult patients.
        Can J Cardiol. 2020; 36: 535-542
        • Bulanov N.
        • Smitienko I.
        • Smagina A.
        • Novikov P.
        • Moiseev S.
        Myocardial infarction due to Takayasu arteritis in adults: look out for multivascular involvement and signs of systemic inflammation.
        Can J Cardiol. 2020; 36: 969.e11
        • Li K.J.
        • Semenov D.
        • Turk M.
        • Pope J.
        A meta-analysis of the epidemiology of giant cell arteritis across time and space.
        Arthritis Res Ther. 2021; 23: 82
        • Zhang Y.-J.
        • Chen Y.
        • Zhao X.
        • et al.
        coronary arteritis and periaortitis in igg4-Related Disease.
        Can J Cardiol. 2020; 36 (589.e5-589.e7)
        • Khosroshahi A.
        • Deshpande V.
        • Stone J.H.
        The clinical and pathological features of IgG4-related disease.
        Curr Rheumatol Rep. 2011; 13: 473-481
        • Rojo-Leyva F.
        • Ratliff N.B.
        • Cosgrove D.M.
        • Hoffman G.S.
        Study of 52 patients with idiopathic aortitis from a cohort of 1,204 surgical cases.
        Arthritis Rheum. 2000; 43: 901-907
        • Clifford A.H.
        • Arafat A.
        • Idrees J.J.
        • et al.
        Outcomes among 196 patients with noninfectious proximal aortitis.
        Arthritis Rheumatol. 2019; 71: 2112-2120
        • Banerjee S.
        • Bagheri M.
        • Sandfort V.
        • et al.
        Vascular calcification in patients with large-vessel vasculitis compared to patients with hyperlipidemia.
        Semin Arthritis Rheum. 2019; 48: 1068-1073
        • Alavi A.
        • Werner T.J.
        • Raynor W.
        • Høilund-Carlsen P.F.
        • Revheim M.-E.
        Critical review of PET imaging for detection and characterization of the atherosclerotic plaques with emphasis on limitations of FDG-PET compared to NaF-PET in this setting.
        Am J Nucl Med Mol Imaging. 2021; 11: 337-351
        • Hernández-Rodríguez J.
        • Alba M.A.
        • Prieto-González S.
        • Cid M.C.
        Diagnosis and classification of polyarteritis nodosa.
        J Autoimmun. 2014; 48-49: 84-89
        • Pagnoux C.
        • Seror R.
        • Henegar C.
        • et al.
        Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group database.
        Arthritis Rheum. 2010; 62: 616-626
        • Butt W.
        Kawasaki Disease: Complex long-term issues for pediatric and adult cardiologists.
        Can J Cardiol. 2020; 36: 1566-1568
        • Newburger J.W.
        • Takahashi M.
        • Gerber M.A.
        • et al.
        Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association.
        Pediatrics. 2004; 114: 1708-1733
        • McCrindle B.W.
        • Rowley A.H.
        • Newburger J.W.
        • et al.
        Diagnosis, treatment, and long-term management of Kawasaki disease: a scientific statement for health professionals from the American Heart Association.
        Circulation. 2017; 135: e927-e999
        • Audemard-Verger A.
        • Terrier B.
        • Dechartres A.
        • et al.
        Characteristics and management of IgA vasculitis (Henoch-Schönlein) in adults: data from 260 patients included in a French multicenter retrospective survey.
        Arthritis Rheumatol. 2017; 69: 1862-1870
        • Seyahi E.
        Behçet’s disease: How to diagnose and treat vascular involvement.
        Best Pract Res Clin Rheumatol. 2016; 30: 279-295
        • Lamprecht P.
        • Gause A.
        • Gross W.L.
        Cryoglobulinemic vasculitis.
        Arthritis Rheum. 1999; 42: 2507-2516
        • Silva F.
        • Pinto C.
        • Barbosa A.
        • et al.
        New insights in cryoglobulinemic vasculitis.
        J Autoimmun. 2019; 105: 102313
        • Sartorelli S.
        • Chassagnon G.
        • Cohen P.
        • et al.
        Revisiting characteristics, treatment and outcome of cardiomyopathy in eosinophilic granulomatosis with polyangiitis (Churg-Strauss).
        Rheumatology (Oxford). 2021; (keab514)
        • Khanna S.
        • Garikapati K.
        • Goh D.S.L.
        • et al.
        Coronary artery vasculitis: a review of current literature.
        BMC Cardiovasc Disord. 2021; 21: 7
        • Blockmans D.
        • de Ceuninck L.
        • Vanderschueren S.
        • et al.
        Repetitive 18F-fluorodeoxyglucose positron emission tomography in giant cell arteritis: a prospective study of 35 patients.
        Arthritis Rheum. 2006; 55: 131-137
        • Silveira L.H.
        Cardiovascular manifestations of systemic vasculitides.
        Curr Rheumatol Rep. 2020; 22: 72
        • Printz B.F.
        • Sleeper L.A.
        • Newburger J.W.
        • et al.
        Noncoronary cardiac abnormalities are associated with coronary artery dilation and with laboratory inflammatory markers in acute Kawasaki disease.
        J Am Coll Cardiol. 2011; 57: 86-92
        • Moran A.M.
        • Newburger J.W.
        • Sanders S.P.
        • et al.
        Abnormal myocardial mechanics in Kawasaki disease: rapid response to gamma-globulin.
        Am Heart J. 2000; 1392: 217-223
        • Dominguez S.R.
        • Anderson M.S.
        • El-Adawy M.
        • Glodé M.P.
        Preventing coronary artery abnormalities: a need for earlier diagnosis and treatment of Kawasaki disease.
        Pediatr Infect Dis J. 2012; 31: 1217-1220
        • Geri G.
        • Wechsler B.
        • Thi Huong D.L.
        • et al.
        Spectrum of cardiac lesions in Behçet disease: a series of 52 patients and review of the literature.
        Medicine (Baltimore). 2012; 91: 25-34
        • Kechida M.
        • Salah S.
        • Kahloun R.
        • et al.
        Cardiac and vascular complications of Behçet disease in the Tunisian context: clinical characteristics and predictive factors.
        Adv Rheumatol. 2018; 58: 32
        • Comarmond C.
        • Pagnoux C.
        • Khellaf M.
        • et al.
        Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort.
        Arthritis Rheum. 2013; 65: 270-281
        • Pugnet G.
        • Gouya H.
        • Puéchal X.
        • et al.
        Cardiac involvement in granulomatosis with polyangiitis: a magnetic resonance imaging study of 31 consecutive patients.
        Rheumatology (Oxford). 2017; 56: 947-956
        • Guillevin L.
        • Durand-Gasselin B.
        • Cevallos R.
        • et al.
        Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients.
        Arthritis Rheum. 1999; 42: 421-430
        • Shuai Z.W.
        • Lv Y.F.
        • Zhang M.M.
        • Hu Z.Y.
        Clinical analysis of patients with myeloperoxidase antineutrophil cytoplasmic antibody-associated vasculitis.
        Genet Mol Res. 2015; 14: 5296-5303
        • Klein-Weigel P.F.
        • Richter J.G.
        Thromboangiitis obliterans (Buerger’s disease).
        Vasa. 2014; 43: 337-346
        • Olin J.W.
        Thromboangiitis obliterans (Buerger’s disease).
        N Engl J Med. 2000; 343: 864-869
        • Asano Y.
        • Sato S.
        Vasculopathy in scleroderma.
        Semin Immunopathol. 2015; 37: 489-500
        • Cerinic M.M.
        • Valentini G.
        • Sorano G.G.
        • et al.
        Blood coagulation, fibrinolysis, and markers of endothelial dysfunction in systemic sclerosis.
        Semin Arthritis Rheum. 2003; 32: 285-295
        • Capsoni F.
        • Poletto G.
        • Giorgetti P.L.
        Fibromuscular dysplasia: a rare disease that can mimic vasculitis.
        Rheumatol Int. 2012; 32: 4027-4029
        • Gornik H.L.
        • Persu A.
        • Adlam D.
        • et al.
        First International Consensus on the diagnosis and management of fibromuscular dysplasia.
        Vasc Med. 2019; 24: 164-189
        • Skeik N.
        • Olson S.L.
        • Hari G.
        • Pavia M.L.
        Segmental arterial mediolysis (SAM): systematic review and analysis of 143 cases.
        Vasc Med. 2019; 24: 549-563
        • Molloy E.S.
        • Langford C.A.
        Vasculitis mimics.
        Curr Opin Rheumatol. 2008; 20: 29-34
        • Grayson P.C.
        • Alehashemi S.
        • Bagheri A.A.
        • et al.
        18F-Fluorodeoxyglucose–positron emission tomography as an imaging biomarker in a prospective, longitudinal cohort of patients with large vessel vasculitis.
        Arthritis RHEUMATOL. 2018; 70: 439-449
        • Dua A.B.
        • Husainat N.M.
        • Kalot M.A.
        • et al.
        Giant cell arteritis: a systematic review and meta-analysis of test accuracy and benefits and harms of common treatments.
        ACR Open Rheumatol. 2021; 3: 429-441
        • Chowdhary V.R.
        • Crowson C.S.
        • Bhagra A.S.
        • Warrington K.J.
        • Vrtiska T.J.
        CT angiographic imaging characteristics of thoracic idiopathic aortitis.
        J Cardiovasc Comput Tomogr. 2013; 7: 297-302
        • Chung S.A.
        • Gorelik M.
        • Langford C.A.
        • et al.
        2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of polyarteritis nodosa.
        Arthritis Rheumatol. 2021; 73: 1384-1393
        • Blockmans D.
        • Luqmani R.
        • Spaggiari L.
        • Salvarani C.
        Magnetic resonance angiography versus 18F-fluorodeoxyglucose positron emission tomography in large vessel vasculitis.
        Autoimmun Rev. 2019; 18: 102405
        • d’Souza N.M.
        • Morgan M.L.
        • Almarzouqi S.J.
        • Lee A.G.
        Magnetic resonance imaging findings in giant cell arteritis.
        Eye (Lond). 2016; 30: 758-762
        • Dejaco C.
        • Ramiro S.
        • Duftner C.
        • et al.
        EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice.
        Ann Rheum Dis. 2018; 77: 636-643
        • Luqmani R.
        • Lee E.
        • Singh S.
        • et al.
        The role of ultrasound compared to biopsy of temporal arteries in the diagnosis and treatment of giant cell arteritis (TABUL): a diagnostic accuracy and cost-effectiveness study.
        Health Technol Assess. 2016; 20: 1-238
        • Maz M.
        • Chung S.A.
        • Abril A.
        • et al.
        2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of giant cell arteritis and Takayasu arteritis.
        Arthritis Rheumatol. 2021; 73: 1349-1365
        • Cetinkaya A.
        • Kersten R.C.
        • Brannan P.A.
        • Thiagarajah C.
        • Kulwin D.R.
        Intraoperative predictability of temporal artery biopsy results.
        Ophthalmic Plast Reconstr Surg. 2008; 24 (discussion 377): 372-376
        • Hedges 3rd, T.R.
        • Gieger G.L.
        • Albert D.M.
        The clinical value of negative temporal artery biopsy specimens.
        Arc Ophthalmol. 1983; 101: 1251-1254
        • Yuksel V.
        • Guclu O.
        • Tastekin E.
        • et al.
        Clinical correlation of biopsy results in patients with temporal arteritis.
        Rev Assoc Med Bras (1992). 2017; 63: 953-956
        • Goslin B.J.
        • Chung M.H.
        Temporal artery biopsy as a means of diagnosing giant cell arteritis: is there over-utilization?.
        Am Surg. 2011; 77: 1158-1160
        • Durling B.
        • Toren A.
        • Patel V.
        • et al.
        Incidence of discordant temporal artery biopsy in the diagnosis of giant cell arteritis.
        Can J Ophthalmol. 2014; 49: 157-161
        • Allison M.C.
        • Gallagher P.J.
        Temporal artery biopsy and corticosteroid treatment.
        Ann Rheum Dis. 1984; 43: 416-417
        • Lie J.T.
        • Brown A.L.
        • Carter E.T.
        Spectrum of aging changes in temporal arteries. Its significance, in interpretation of biopsy of temporal artery.
        Arch Pathol. 1970; 90: 278-285
        • Dimachkie M.D.
        • Fraga G.R.
        • Moura N.S.
        • Springer J.M.
        A rare case of adenosine deaminase 2 deficiency presenting with temporal arteritis.
        J Clin Rheumatol. 2021; 27: e251-e252
        • Esteban M.J.
        • Font C.
        • Hernández-Rodríguez J.
        • et al.
        Small-vessel vasculitis surrounding a spared temporal artery: clinical and pathological findings in a series of twenty-eight patients.
        Arthritis Rheum. 2001; 44: 1387-1395
        • Ghinai R.A.
        • Mahmood S.
        • Mukonoweshuro P.
        • et al.
        Diagnosing light chain amyloidosis on temporal artery biopsies for suspected giant cell arteritis.
        J Neuroophthalmol. 2017; 37: 34-39
        • Neri A.
        • Rubino P.
        • Macaluso C.
        • Gandolfi S.A.
        Light-chain amyloidosis mimicking giant cell arteritis in a bilateral anterior ischemic optic neuropathy case.
        BMC Ophthalmol. 2013; 13: 82
        • Miloslavsky E.M.
        • Naden R.P.
        • Bijlsma J.W.J.
        • et al.
        Development of a glucocorticoid toxicity index (GTI) using multicriteria decision analysis.
        Ann Rheum Dis. 2017; 76: 543-546
        • Titeca-Beauport D.
        • Francois A.
        • Lobbedez T.
        • et al.
        Early predictors of one-year mortality in patients over 65 presenting with ANCA-associated renal vasculitis: a retrospective, multicentre study.
        BMC Nephrol. 2018; 19: 317
        • Chung S.A.
        • Langford C.A.
        • Maz M.
        • et al.
        2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of antineutrophil cytoplasmic antibody–associated vasculitis.
        Arthritis Rheumatol. 2021; 73: 1366-1383