Large-Vessel Vasculitis

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
• 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
• 3 Hata A, Noda M, Moriwaki R, Numano F. Angiographic findings of Takayasu arteritis: new classification.  Int J Cardiol. 1996;  54(suppl) S155-S163
• 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
• 5 Subramanyan R, Joy J, Balakrishnan K G. Natural history of aortoaortitis (Takayasu's disease).  Circulation. 1989;  80 429-437
• 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
• 7 Moore P, Cupps T. Neurologic complications of vasculitis.  Ann Neurol. 1983;  14 155-167
• 8 Kiyosawa M, Baba T. Ophthalmological findings in patients with Takayasu disease.  Int J Cardiol. 1998;  66(suppl 1) S141-S147
• 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
• 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
• 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
• 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
• 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
• 10 Numano F, Kobayashi Y. Takayasu arteritis-beyond pulselessness.  Intern Med. 1999;  38 226-232
• 11 Lie J T. Isolated pulmonary Takayasu arteritis: clinicopathological characteristics.  Mod Pathol. 9 469-474
• 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
• 13 Kerr G, Hallahan C, Giordano J et al.. Takayasu arteritis.  Ann Intern Med. 1994;  120 919-929
• 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
• 14a Rose A G, Sinclair C C. Takayasu's arteritis: a study of 16 autopsy cases.  Arch Pathol Lab Med. 1980;  104 231-237
• 14b Lagneau P, Michel J. Renovascular hypertension and Takayasu's disease.  J Urol. 1985;  134 876-879
• 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
• 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
• 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
• 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
• 17 Hoffman G S. Takayasu arteritis: lessons from the American National Institutes of Health experience.  Int J Cardiol. 1996;  54(suppl) S83-S86
• 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
• 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
• 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
• 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
• 22 Liang P, Tan-Ong M, Hoffman G S. Takayasu's arteritis: vascular interventions and outcomes.  J Rheumatol. 2004;  31 102-106
• 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
• 24 Lagneau P, Michel J B, Vuong P N. Surgical treatment of Takayasu's disease.  Ann Surg. 1987;  205 157-166
• 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
• 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
• 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
• 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
• 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
• 28 Mohan N, Kerr G. Spectrum of giant cell vasculitis.  Curr Rheumatol Rep. 2000;  2 390-395
• 29 Aiello P D, Trautmann J C, McPhee T J et al.. Visual prognosis in giant cell arteritis.  Ophthalmology. 1993;  100 550-555
• 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
• 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
• 32 Hunder G G, Allen G L. Giant cell arteritis: a review.  Bull Rheum. 1978-9;  29 980-986
• 33 Ostberg G. Morphological changes in the large arteries in polymyalgia rheumatica.  Acta Med Scand. 1972;  (suppl 33) 135-164
• 34 Ladanyi M, Fraser R S. Pulmonary involvement in giant cell arteritis.  Arch Pathol Lab Med. 1987;  111 1178-1180
• 35 Wagenaar S SC, Westermann C JJ, Corrin B. Giant cell arteritis limited to large elastic pulmonary arteries.  Thorax. 1981;  36 876-877
• 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
• 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
• 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
• 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
• 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
• 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
• 38d Andersson R, Malmvall B E, Bengtsson B A. Long-term corticosteroid treatment in giant cell arteritis.  Acta Med Scand. 1986;  220 465-469
• 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
• 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
• 39 Schumacher H R. Sarcoidosis. In: Koopman Arthritis and Allied Conditions Baltimore, MD; Williams & Wilkins 1997: 1689-1695
• 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
• 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
• 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
• 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
• 44 Takemura T, Shishiba T, Akiyama O et al.. Vascular involvement in cutaneous sarcoidosis.  Pathol Int. 1997;  47 84-89
• 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
• 46 Travis W D. Pathology of pulmonary granulomatous vasculitis.  Sarcoidosis Vasc Diffuse Lung Dis. 1996;  13 14-27
• 47 Studdy P, Bird R, James D G. Serum angiotensin-converting enzyme (SACE) in sarcoidosis and other granulomatous disorders.  Lancet. 1978;  2 1331-1334
• 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
• 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
• 50 Fernandes S, Singsen B, Hoffman G S. Sarcoidosis and systemic vasculitis.  Semin Arthritis Rheum. 2000;  30 33-46