Download PDF
Semin Respir Crit Care Med 2004; 25(5): 569-579
DOI: 10.1055/s-2004-836148
Copyright © 2004 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA.

Large-Vessel Vasculitis

Kathleen Maksimowicz-McKinnon1 , Gary S. Hoffman2
  • 1Department of Medicine, Division of Rheumatology, University of Pittsburgh, Pittsburgh, Pennsylvania
  • 2Department of Rheumatology and Immunology, Center for Vasculitis Care and Research, The Cleveland Clinic Foundation, Cleveland, Ohio
Further Information

Publication History

Publication Date:
09 November 2004 (online)

    Permissions and Reprints

The vasculitides affecting large and medium-sized vessels are heterogeneous. This group includes such disorders as giant cell arteritis, Takayasu's disease, and sarcoidosis. There are several challenges that may arise in the care of patients with these disorders.

Diagnosis may be elusive when initially evaluating patients with large-vessel vasculitis. Most serologic markers are not specific, and tissue biopsy is often impractical. We will present data emphasizing the most common disease manifestations, to aid in recognition of a clinical picture suggestive of large-vessel vasculitis. We will also focus on the more common pulmonary manifestations and minimize case reports, which may not reflect the usual course of disease.

Large cohort studies and randomized controlled trials to provide an evidence-based approach to therapy in these disorders are uncommon. We have included in this review discussion of those studies that we judged to be most rigorously conducted and clinically relevant. We provide guidelines for initial therapy, with caveats to assist in minimizing potential treatment-related complications and toxicities.

Although monitoring of disease activity is often difficult, this is a most crucial element in minimizing disease and treatment-associated morbidity and mortality.

KEYWORDS

Vasculitis - sarcoidosis - Takayasu's arteritis - giant cell arteritis

REFERENCES

  • 1 Nasu T. Takayasu's truncoarteritis in Japan: a statistical observation of 76 autopsy cases.  Pathol Microbiol (Basel). 1975;  43 140-146
    PubMedGoogle Scholar
  • 2 Hall S, Barr M D, Lie J T, Stanson A W, Kazmier F J, Hunder G G. Takayasu's arteritis: a study of 32 North American patients.  Medicine. 1985;  64 89-99
    PubMedGoogle Scholar
  • 3 Hata A, Noda M, Moriwaki R, Numano F. Angiographic findings of Takayasu arteritis: new classification.  Int J Cardiol. 1996;  54(suppl) S155-S163
    CrossrefPubMedGoogle Scholar
  • 4 Lupi-Herrera E, Sanchez-Torrez G, Marcushamer J et al.. Takayasu arteritis: clinical study of 107 cases.  Am Heart J. 1977;  93 94-103
    CrossrefPubMedGoogle Scholar
  • 5 Subramanyan R, Joy J, Balakrishnan K G. Natural history of aortoaortitis (Takayasu's disease).  Circulation. 1989;  80 429-437
    CrossrefPubMedGoogle Scholar
  • 6 Zhonggao W, Shen L, Yu J et al.. Management of cerebral ischemia due to Takayasu's arteritis.  Chin Med J (Engl). 2002;  115 342-346
    PubMedGoogle Scholar
  • 7 Moore P, Cupps T. Neurologic complications of vasculitis.  Ann Neurol. 1983;  14 155-167
    CrossrefPubMedGoogle Scholar
  • 8 Kiyosawa M, Baba T. Ophthalmological findings in patients with Takayasu disease.  Int J Cardiol. 1998;  66(suppl 1) S141-S147
    CrossrefPubMedGoogle Scholar
  • 9 Sheikhzadeh A, Tettenborn I, Noohi F, Eftekharzadeh M, Schnabel A. Occlusive thromboaortopathy (Takayasu disease): clinical and angiographic features and a brief review of literature.  Angiology. 2002;  53 29-40
    PubMedGoogle Scholar
  • 9a Sharma S, Kamalakar T, Rajani M, Talwar K K, Shrivastava S. The incidence and patterns of pulmonary artery involvement in Takayasu's arteritis.  Clin Radiol. 1990;  42 177-181
    CrossrefPubMedGoogle Scholar
  • 9b Yamada I, Nakagawa T, Himeno Y et al.. Takayasu arteritis: diagnosis with breath-hold contrast-enhanced three-dimensional MR angiography.  J Magn Reson Imaging. 2000;  11 481-487
    CrossrefPubMedGoogle Scholar
  • 9c Vanoli M, Castellani M, Bacchiani G et al.. Noninvasive assessment of pulmonary artery involvement in Takayasu's arteritis.  Clin Exp Rheumatol. 1999;  17 215-218
    PubMedGoogle Scholar
  • 9d Ogawa Y, Hayashi K, Sakamoto I, Matsunaga N. Pulmonary arterial lesions in Takayasu arteritis: relationship of inflammatory activity to scintigraphic findings and sequential changes.  Ann Nucl Med. 1996;  10 219-223
    PubMedGoogle Scholar
  • 10 Numano F, Kobayashi Y. Takayasu arteritis-beyond pulselessness.  Intern Med. 1999;  38 226-232
    PubMedGoogle Scholar
  • 11 Lie J T. Isolated pulmonary Takayasu arteritis: clinicopathological characteristics.  Mod Pathol. 9 469-474
    PubMedGoogle Scholar
  • 12 Haque U, Hellman D, Traill T, Venbrux A, Stone J. Takayasu's arteritis involving proximal pulmonary arteries and mimicking thromboembolic disease.  J Rheumatol. 1999;  26 450-453
    PubMedGoogle Scholar
  • 13 Kerr G, Hallahan C, Giordano J et al.. Takayasu arteritis.  Ann Intern Med. 1994;  120 919-929
    CrossrefPubMedGoogle Scholar
  • 14 Castellote E, Romero R, Bonet J et al.. Takayasu's arteritis as a cause of renovascular hypertension in a non-Asian population.  J Hum Hypertens. 1995;  9 841-845
    PubMedGoogle Scholar
  • 14a Rose A G, Sinclair C C. Takayasu's arteritis: a study of 16 autopsy cases.  Arch Pathol Lab Med. 1980;  104 231-237
    PubMedGoogle Scholar
  • 14b Lagneau P, Michel J. Renovascular hypertension and Takayasu's disease.  J Urol. 1985;  134 876-879
    PubMedGoogle Scholar
  • 14c Chugh K S et al.. Nonspecific aortoarteritis is a major cause of renovascular hypertension in Asian countries. Proc Asian Pacific Cong Nephrol Beijing; 1990: 450-456
    Google Scholar
  • 14d Sharma S, Rajani M, Kamalakar T, Talwar K K, Sunderam K R. Clinical and angiographic features of nonspecific aorto-arteritis in children and adults.  Acta Radiol. 1991;  32 485-487
    PubMedGoogle Scholar
  • 15 Tso E, Flamm S, White F et al.. Takayasu arteritis: utility and limitations of magnetic resonance imaging in diagnosis and treatment.  Arthritis Rheum. 2002;  46 1634-1642
    CrossrefPubMedGoogle Scholar
  • 16 Belhocine T, Blockmans D, Hustinx R, Vandevivere F, Mortelmans L. Imaging of large-vessel vasculitis with 18FDG PET: illusion or reality? A critical review of the literature data.  Eur J Nucl Med Mol Imaging. 2003;  30 1305-1313
    CrossrefPubMedGoogle Scholar
  • 17 Hoffman G S. Takayasu arteritis: lessons from the American National Institutes of Health experience.  Int J Cardiol. 1996;  54(suppl) S83-S86
    CrossrefPubMedGoogle Scholar
  • 18 Hoffman G S, Leavitt R Y, Kerr G S et al.. Treatment of glucocorticoid-resistant or relapsing Takayasu arteritis with methotrexate.  Arthritis Rheum. 1994;  37 578-582
    CrossrefPubMedGoogle Scholar
  • 19 Shelhamer J H, Volkman D J, Parrillo J E et al.. Takayasu's arteritis and its therapy.  Ann Intern Med. 1985;  103 121-126
    CrossrefPubMedGoogle Scholar
  • 20 Hoffman G S, Merkel P A, Brassington R D, Lenshcow D, Kissin E Y et al.. Utility of anti-TNF therapy in Takayasu's arteritis [abstract 1101].  Arthritis Rheum. 2003;  48(suppl)-S442
    PubMedGoogle Scholar
  • 21 Deyu Z, Lisheng L, Ruping D, Haiying W, Guozhang L. Percutaneous transluminal renal angioplasty in aortoarteritis.  Int J Cardiol. 1998;  66(suppl) S205-S211
    CrossrefPubMedGoogle Scholar
  • 22 Liang P, Tan-Ong M, Hoffman G S. Takayasu's arteritis: vascular interventions and outcomes.  J Rheumatol. 2004;  31 102-106
    PubMedGoogle Scholar
  • 23 Miyata T, Sato O, Koyama H, Shigematsu H, Tada Y. Long-term survival after surgical treatment of patients with Takayasu's arteritis.  Circulation. 2003;  108 1474-1480
    CrossrefPubMedGoogle Scholar
  • 24 Lagneau P, Michel J B, Vuong P N. Surgical treatment of Takayasu's disease.  Ann Surg. 1987;  205 157-166
    PubMedGoogle Scholar
  • 25 Hoffman G S. Treatment of giant-cell arteritis: where we have been and why we must move on.  Cleve Clin J Med. 2002;  69(suppl 2) SII117-SII120
    PubMedGoogle Scholar
  • 25a Gonzalez-Gay M A, Bianco R, Rodrigues-Valverde V et al.. Permanent visual loss and cerebrovascular accidents in giant cell arteritis: predictors and response to treatment.  Arthritis Rheum. 1998;  41 1497-1504
    CrossrefPubMedGoogle Scholar
  • 25b Chevalet P, Barrier J H, Pottier P et al.. A randomized, multicenter, controlled trial using intraversus pulses of methylprednisolone predissolving in the initial treatment of simple forms of giant cell arteritis: a one year follow-up study of 164 patients.  J Rheumatol. 2000;  27 1484-1491
    PubMedGoogle Scholar
  • 26 Brack A, Geisler A, Martinez-Taboada V M et al.. Giant cell vasculitis is a T cell-dependent disease.  Mol Med. 1997;  3 530-543
    PubMedGoogle Scholar
  • 27 Hunder G, Valente R. Giant cell arteritis: clinical aspects. In: Hoffman GS, Weyand CM Inflammatory Diseases of Blood Vessels New York, NY; Marcel Dekker 2002: 425-442
    Google Scholar
  • 28 Mohan N, Kerr G. Spectrum of giant cell vasculitis.  Curr Rheumatol Rep. 2000;  2 390-395
    PubMedGoogle Scholar
  • 29 Aiello P D, Trautmann J C, McPhee T J et al.. Visual prognosis in giant cell arteritis.  Ophthalmology. 1993;  100 550-555
    CrossrefPubMedGoogle Scholar
  • 30 Liozon E, Herrmann F, Ly K et al.. Risk factors for visual loss in giant cell (temporal) arteritis: a prospective study of 174 patients.  Am J Med. 2001;  111 211-217
    CrossrefPubMedGoogle Scholar
  • 31 Hunder G G. Giant cell arteritis and polymyalgia rheumatica. In: Ruddy S, Harris ED Jr, Sledge CB Kelley's textbook of Rheumatology Philadelphia, PA; Saunders 2001: 1158
    Google Scholar
  • 32 Hunder G G, Allen G L. Giant cell arteritis: a review.  Bull Rheum. 1978-9;  29 980-986
    PubMedGoogle Scholar
  • 33 Ostberg G. Morphological changes in the large arteries in polymyalgia rheumatica.  Acta Med Scand. 1972;  (suppl 33) 135-164
    PubMedGoogle Scholar
  • 34 Ladanyi M, Fraser R S. Pulmonary involvement in giant cell arteritis.  Arch Pathol Lab Med. 1987;  111 1178-1180
    PubMedGoogle Scholar
  • 35 Wagenaar S SC, Westermann C JJ, Corrin B. Giant cell arteritis limited to large elastic pulmonary arteries.  Thorax. 1981;  36 876-877
    PubMedGoogle Scholar
  • 36 Larson T S, Hall S, Hepper N GG, Hunder G G. Respiratory tract symptoms as a clue to giant cell arteritis.  Ann Intern Med. 1984;  101 594-597
    PubMedGoogle Scholar
  • 37 Lie J T. Illustrated histopathologic classification criteria for selected vasculitis syndromes. American College of Rheumatology Subcommittee on Classification of Vasculitis.  Arthritis Rheum. 1990;  33 1074-1087
    PubMedGoogle Scholar
  • 38 Salvarani C, Silingardi M, Ghirarduzzi A et al.. Is duplex ultrasonography useful for the diagnosis of giant cell arteritis?.  Ann Intern Med. 2002;  137 E232-E238
    PubMedGoogle Scholar
  • 38a Proven A, Gabriel S E, Orces C, O'Fallon W M, Hunder G G. Glucocorticoid therapy in giant cell arteritis: duration and adverse outcomes.  Arthritis Rheum. 2003;  49 703-708
    CrossrefPubMedGoogle Scholar
  • 38b Hachulla E, Boivin V, Pastuerel-Michon U et al.. Prognostic factors and long-term evolution in a cohort of 133 patients with giant cell arteritis.  Clin Exp Rheumatol. 2001;  19 171-176
    PubMedGoogle Scholar
  • 38c Liozon E, Roblot P, Paire D et al.. Anticardiolipin antibody levels predict flares and relapses in patients with giant cell (temporal) arteritis: a longitudinal study of 58 biopsy-proven cases.  Rheumatology (Oxford). 2000;  39 1089-1094
    CrossrefPubMedGoogle Scholar
  • 38d Andersson R, Malmvall B E, Bengtsson B A. Long-term corticosteroid treatment in giant cell arteritis.  Acta Med Scand. 1986;  220 465-469
    PubMedGoogle Scholar
  • 38e Jover J A, Hernanadez-Garcia C, Morado I C et al.. Combined treatment of giant-cell arteritis with methotrexate and prednisone.  Ann Intern Med. 2001;  134 106-114
    CrossrefPubMedGoogle Scholar
  • 38f Hoffman G S, Cid M C, Hellmann D B et al.. A multicenter, randomized, double-blind, placebo-controlled trial of adjuvant methotrexate treatment for giant cell arteritis.  Arthritis Rheum. 2002;  46 1309-1318
    CrossrefPubMedGoogle Scholar
  • 39 Schumacher H R. Sarcoidosis. In: Koopman Arthritis and Allied Conditions Baltimore, MD; Williams & Wilkins 1997: 1689-1695
    Google Scholar
  • 40 Neville E, Walker A N, James D G. Prognostic factors predicting the outcome of sarcoidosis: an analysis of 818 patients.  Q J Med. 1983;  52 525-533
    PubMedGoogle Scholar
  • 41 Skold C M, Larsen F F, Rasmussen E, Pehrsson S K, Eklund A G. Determination of cardiac involvement in sarcoidosis by magnetic resonance imaging and Doppler echocardiography.  J Intern Med. 2002;  252 465-471
    CrossrefPubMedGoogle Scholar
  • 42 Silverman K J, Hutchins G M, Bulkley B H. Cardiac sarcoidosis: a clinicopathological study of 84 unselected patients with systemic sarcoidosis.  Circulation. 1978;  58 1204-1215
    CrossrefPubMedGoogle Scholar
  • 43 Rendt K E, Hoffman G S. Systemic vasculitis in sarcoidosis. In Hoffman GS, Weyard CM Inflammatory Diseases of Blood Vessels New York, NY; Marcel Dekker 2001: 695-705
    Google Scholar
  • 44 Takemura T, Shishiba T, Akiyama O et al.. Vascular involvement in cutaneous sarcoidosis.  Pathol Int. 1997;  47 84-89
    PubMedGoogle Scholar
  • 45 Crystal R G. Sarcoidosis. In: Fauci, Braunwald, Issel'bacher, et al Harrison's Principles of Internal Medicine New York, NY; McGraw-Hill 1998: 1925
    Google Scholar
  • 46 Travis W D. Pathology of pulmonary granulomatous vasculitis.  Sarcoidosis Vasc Diffuse Lung Dis. 1996;  13 14-27
    PubMedGoogle Scholar
  • 47 Studdy P, Bird R, James D G. Serum angiotensin-converting enzyme (SACE) in sarcoidosis and other granulomatous disorders.  Lancet. 1978;  2 1331-1334
    PubMedGoogle Scholar
  • 48 Yamagishi H, Shirai N, Takagi M et al.. Identification of cardiac sarcoidosis with 13N-NH3/18F-FDG PET.  J Nucl Med. 2003;  44 1030-1036
    PubMedGoogle Scholar
  • 49 Utz J P, Limper A H, Kalra S et al.. Etanercept for the treatment of stage II and III progressive pulmonary sarcoidosis.  Chest. 2003;  124 177-185
    CrossrefPubMedGoogle Scholar
  • 50 Fernandes S, Singsen B, Hoffman G S. Sarcoidosis and systemic vasculitis.  Semin Arthritis Rheum. 2000;  30 33-46
    CrossrefPubMedGoogle Scholar

Kathleen Maksimowicz-McKinnonD.O. 

University of Pittsburgh, Division of Rheumatology

3500 Terrace St., BST S718

Pittsburgh, PA 15261

Email: kmaxdo@hotmail.com

      >