Abstract
Necrotizing systemic vasculitis was described nearly 135 years ago as a fatal condition [1]. Early reports of these forms of vasculitis indeed documented a rapidly progressive course with death occurring within months of diagnosis [2, 3]. Treatment with corticosteroids reduced the 1-year mortality, but after 3 years no difference in mortality was found between treated and untreated patients [4]. Since the introduction of a combination of cyclophosphamide and steroids, however, the outcome of vasculitis has dramatically changed, and 1-year survival has increased to 70-99% [5-9]. Vasculitis has now become a chronic disorder with accumulating morbidity resulting in impairment of employability, functional status, and social activities [10, 11]. The costs for vasculitis-related hospitalizations in the USA were roughly calculated to be over $150 million per year [12]. There is a constant need to improve therapeutic protocols for vasculitis since current protocols are toxic, contribute to morbidity and mortality, and are not always effective. Novel approaches that recently became available include therapy with mycophenolate and/or 15-deoxyspergualin (see the chapter by Stegeman and Birck in this volume), intravenous immunoglobulin (see the chapter by Jayne), tumor necrosis factor α (TNFα) directed therapy (see the chapter by Stone), anti-thymocyte globulin and anti-CD52/anti-CD4 therapy (see the chapter by Schmitt et al.), and immunoablation with or without stem cell rescue (see the chapter by Carruthers and Bacon). These novel approaches offer the possibility to treat patients with a less toxic and/or more effective treatment modality than with the therapeutic regimens that are currently used as “standard regimens”. These latter will be reviewed in this chapter.
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Kussmaul A, Maier K (1866) Ueber eine bisher nicht beschriebene eigentümliche Arte-rienkrankung (Periarteriitis Nodosa), die mit morbus Brightii und rapid fortschreitender allgemeiner Muskellähmung einhergeht. Deutsch Arch f Klin Med 1: 484–517
Walton EW (1958) Giant cell granuloma of the respiratory tract (Wegener’s granulo-matosis). BMJ 2: 265–270
Frohnert PP, Sheps SG (1967) Long-term follow-up study of periarteritis nodosa. Am J Med 43: 8–14
Treatment of polyarteritis nodosa with cortisone: results after three years. BMJ 4: 1399–1400
Gaskin G, Pusey CD (1999) Crescentic glomerulonephritis and systemic vasculitis. In: CD Pusey (ed): The treatment of glomerulonephritis. Kluwer Academic Publishers, Dordrecht, 113–142
Jayne D (2000) Evidence-based treatment of systemic vasculitis. Rheumatology 39: 585–595
McLaughlin K, Jerimiah P, Fox JG, Mactier RA, Simpson K, Boulton-Jones JM (1998) Has the prognosis for patients with pauci-immune necrotizing glomerulonephritis improved? Nephrol Dial Transplant 13: 1696–1701
Aasarod K, Iversen BM, Hammerstrom J, Bostad L, Vatten L, Jorstad S (2000) Wegener’s granulomatosis: clinical course in 108 patients with renal involvement. Nephrol Dial Transplant 15: 611–618
Reinhold-Keller E, Beuge N, Latza U, de Groot K, Rudert H, Nolle B, Heller M, Gross WL (2000) An interdisciplinary approach to the care of patients with Wegener’s granulomatosis: long-term outcome in 155 patients. Arthritis Rheum 43: 1021–1032
Hoffman GS, Drucker Y, Cotch MF, Locker GA, Easley K, Kwoh K (1998) Wegener’s granulomatosis: patient-reported effects of disease on health, function, and income. Arthritis Rheum 41: 2257–2262
Boomsma MM, Stegeman CA, Cohen Tervaert JW (1999) Comparison of Dutch and US patients’ perceptions of the effects of Wegener’s granulomatosis on health, function, income, and interpersonal relationships: comment on the article by Hoffman et al. [letter]. Arthritis Rheum 42: 2495–2497
Cotch MF (2000) The socioeconomic impact of vasculitis. Curr Opin Rheumatol 12: 20–23
Wilke WS (1997) Large vessel vasculitis (giant cell arteritis, Takayasu arteritis). Baillieres Clin Rheumatol 11: 285–313
Nordborg E, Nordborg C, Malmvall BE, Andersson R, Bengtsson BA (1995) Giant cell arteritis. Rheum Dis Clin North Am 21: 1013–1026
Weyand CM, Fulbright JW, Hunder GG, Evans JM, Goronzy JJ (2000) Treatment of giant cell arteritis: interleukin-6 as a biologic marker of disease activity. Arthritis Rheum 43: 1041–1048
De Silva M, Hazleman BL (1986) Azathioprine in giant cell arteritis/polymyalgia rheumatica: a double-blind study. Ann Rheum Dis 45: 136–138
Van der Veen MJ, Dinant HJ, Van Booma-Frankfort C, Van Albada-Kuipers GA, Bijlsma JW (1996) Can methotrexate be used as a steroid sparing agent in the treatment of polymyalgia rheumatica and giant cell arteritis? Ann Rheum Dis 55: 218–223
Jover J, Hernández-García C, Morado I, Vargas E, Bañares A, Fernández-Gutiérrez B (2001) Combined treatment of giant cell arteritis with methotrexate and prednisone: a randomized, double blind, and placebo-controlled study. Ann Intern Med 134: 106–114
Hoffman GS, Leavitt RY, Kerr GS, Rottem M, Sneller MC, Fauci AS (1994) Treatment of glucocorticoid-resistant or relapsing Takayasu arteritis with methotrexate. Arthritis Rheum 37: 578–582
Tyagi S, Singh B, Kaul UA, Sethi KK, Arora R, Khalilullah M (1993) Balloon angioplasty for renovascular hypertension in Takayasu’s arteritis. Am Heart J 125: 1386–1393
Dillon MJ, Ansell BM (1995) Vasculitis in children and adolescents. Rheum Dis Clin North Am 21: 1115–1136
Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, Hagen EC, Hoffman GS, Hunder GG, Kallenberg CG et al (1994) Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 37: 187–192
Guillevin L (1999) Treatment of classic polyarteritis nodosa in 1999 [editorial]. Nephrol Dial Transplant 14: 2077–2079
Lhote F, Guillevin L (1995) Polyarteritis nodosa, microscopic polyangiitis, and ChurgStrauss syndrome. Clinical aspects and treatment. Rheum Dis Clin North Am 21: 911–947
Guillevin L, Lhote F (1998) Treatment of polyarteritis nodosa and microscopic polyangiitis. Arthritis Rheum 41: 2100–2105
Franssen CF, Stegeman CA, Kallenberg CG, Gans RO, De Jong PE, Hoorntje SJ, Tervaert JW (2000) Antiproteinase 3- and antimyeloperoxidase-associated vasculitis. Kidney Int 57: 2195–2206
Fienberg R (1981) The protacted superficial phenomenon in pathergic (Wegener’s) granulomatosis. Hum Path 12: 458–467
Cohen Tervaert JW, van der Woude FJ, Kallenberg CG (1987) Analysis of symptoms preceding the diagnosis of Wegener’s disease. Ned Tijdschr Geneeskd 131: 1391–1394
Rasmussen NJ, Abramowicz D, Andrassy K et al (1995) European therapeutic trials in ANCA-associated systemic vasculitis: disease scoring, consensus regimens and proposed clinical trials. Clin Exp Immunol 101 (Suppl 1): 29–34
Jayne D, Rasmussen N (2000) European collaborative trials in vasculitis: EUVAS update and latest results. Clin Exp Immunol 120 (Suppl 1): 13–15
Klemmer PJ, Chalermskulrat W, Reif MS, Hogan SL, Henke DC, Falk RJ (2000) Treatment with plasmapheresis in diffuse alveolar hemorrhage in ANCA-SVV [abstract]. Clin Exp Immunol 120 (Suppl 1): 73
Pusey CD, Rees AJ, Evans DJ, Peters DK, Lockwood CM (1991) Plasma exchange in focal necrotizing glomerulonephritis without anti-GBM antibodies. Kidney Int 40: 757–763
Levy JB, Pusey CD (1997) Still a role for plasma exchange in rapidly progressive glomerulonephritis? J Nephrol 10: 7–13
Guillevin L, Cordier JF, Lhote F, Cohen P, Jarrousse B, Royer I, Lesavre P, Jacquot C, Bindi P, Bielefeld P, et al. (1997) A prospective, multicenter, randomized trial comparing steroids and pulse cyclophosphamide versus steroids and oral cyclophosphamide in the treatment of generalized Wegener’s granulomatosis. Arthritis Rheum 40: 2187–2198
Haubitz M, Schellong S, Gobel U, Schurek HJ, Schaumann D, Koch KM, Brunkhorst R (1998) Intravenous pulse administration of cyclophosphamide versus daily oral treatment in patients with antineutrophil cytoplasmic antibody-associated vasculitis and renal involvement: a prospective, randomized study. Arthritis Rheum 41: 1835–1844
Adu D, Pall A, Luqmani RA, Richards NT, Howie AJ, Emery P, Michael J, Savage CO, Bacon PA (1997) Controlled trial of pulse versus continuous prednisolone and cyclophosphamide in the treatment of systemic vasculitis. QJM 90: 401–409
de Groot K, Adu D, Savage CO (2000) To pulse or not to pulse in ANCA-associated vasculitis — a critical analysis [abstract]. Clin Exp Immunol 120 (Suppl 1): 68
Langford CA, Talar-Williams C, Sneller MC (2000) Use of methotrexate and glucocorticoids in the treatment of Wegener’s granulomatosis. Long-term renal outcome in patients with glomerulonephritis. Arthritis Rheum 43: 1836–1840
Haidinger M, Neumann L, Jaeger H, Gruetzmacher H, Bayer P, Meisl FT (2000) Mycophenolate Mofetil (MMF) treatment of ANCA-associated small-vessel vasculitis [abstract]. Clin Exp Immunol 120 (Suppl 1): 72
Nachman PH, Joy MS, Hogan SL, Jennette JC, Falk RJ (2000) Mycophenolate mofetil: preliminary results of a feasibility trial in relapsing ANCA small vessel vasculitis [abstract]. Clin Exp Immunol 120 (Suppl 1): 72
Pasavento RE, Falkenhain ME, Rovin BH, Hebert LA (1999) Mycophenolate in anti-neutrophil cytoplasmic antibody vasculitis [abstract]. J Am Soc Nephrol 10: 114A
Stegeman CA, Cohen Tervaert JW (2000) Mycophenolate mofetil for remission induction in patients with active Wegener’s granulomatosis intolerant to cyclophosphamide [abstract]. J Am Soc Nephrol 11: 98A
Waiser J, Budde K, Braasch E, Neumayer HH (1999) Treatment of acute c-ANCA-positive vasculitis with mycophenolate mofetil. Am J Kidney Dis 34: e9
Talar-Williams C, Hijazi YM, Walther MM, Linehan WM, Hallahan CW, Lubensky I, Kerr GS, Hoffman GS, Fauci AS, Sneller MC (1996) Cyclophosphamide-induced cystitis and bladder cancer in patients with Wegener granulomatosis. Ann Intern Med 124: 477–484
Westman KW, Bygren PG, Olsson H, Ranstam J, Wieslander J (1998) Relapse rate, renal survival, and cancer morbidity in patients with Wegener’s granulomatosis or microscopic polyangiitis with renal involvement. J Am Soc Nephrol 9: 842–852
Sneller MC, Hoffman GS, Talar-Williams C, Kerr GS, Hallahan CW, Fauci AS (1995) An analysis of forty-two Wegener’s granulomatosis patients treated with methotrexate and prednisone. Arthritis Rheum 38: 608–613
Stone JH, Tun W, Hellman DB (1999) Treatment of non-life threatening Wegener’s granulomatosis with methotrexate and daily prednisone as the initial therapy of choice. J Rheumatol 26: 1134–1139
de Groot K, Muhler M, Reinhold-Keller E, Paulsen J, Gross WL (1998) Induction of remission in Wegener’s granulomatosis with low dose methotrexate. J Rheumatol 25: 492–495
Langford CA, Talar-Williams C, Barron KS, Sneller MC (1999) A staged approach to the treatment of Wegener’s granulomatosis: induction of remission with glucocorticoids and daily cyclophosphamide switching to methotrexate for remission maintenance. Arthritis Rheum 42: 2666–2673
Reinhold-Keller E, De Groot K, Rudert H, Nolle B, Heller M, Gross WL (1996) Response to trimethoprim/sulfamethoxazole in Wegener’s granulomatosis depends on the phase of disease. QJM 89: 15–23
Stegeman CA, Cohen Tervaert JW, Kallenberg CG (1997) Co-trimoxazole and Wegen-er’s granulomatosis: more than a coincidence? [editorial]. Nephrol Dial Transplant 12: 652–655
Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, Rottem M, Fauci AS (1992) Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 116: 488–498
Franssen CF, Stegeman CA, Oost-Kort WW, Kallenberg CG, Limburg PC, Tiebosch A, De Jong PE, Cohen Tervaert JW (1998) Determinants of renal outcome in antimyeloperoxidase-associated necrotizing crescentic glomerulonephritis. J Am Soc Nephrol 9: 1915–1923
Jayne D, Gaskin G (1999) Randomised trial of cyclophosphamide versus azthioprine during remission in ANCA-associated vasculitis (CYCAZAREM) [abstract]. J Am Soc Nephrol 10: 105A
de Groot K, Reinhold-Keller E, Tatsis E, Paulsen J, Heller M, Nolle B, Gross WL (1996) Therapy for the maintenance of remission in sixty-five patients with generalized Wegen-er’s granulomatosis. Methotrexate versus trimethoprim/sulfamethoxazole. Arthritis Rheum 39: 2052–2061
Haubitz M, Koch KM, Brunkhorst R (1998) Cyclosporin for the prevention of disease reactivation in relapsing ANCA-associated vasculitis. Nephrol Dial Transplant 13: 2074–2076
Nowack R, Gobel U, Klooker P, Hergesell O, Andrassy K, van der Woude FJ (1999) Mycophenolate mofetil for maintenance therapy of Wegener’s granulomatosis and microscopic polyangiitis: a pilot study in 11 patients with renal involvement. J Am Soc Nephrol 10: 1965–1971
Cohen Tervaert JW, van der Woude FJ, Fauci AS, Ambrus JL, Velosa J, Keane WF, Meijer S, van der Giessen M, van der Hem GK, The TH et al (1989) Association between active Wegener’s granulomatosis and anticytoplasmic antibodies. Arch Intern Med 149: 2461–2465
Cohen Tervaert JW, Huitema MG, Hene RJ, Sluiter WJ, The TH, van der Hem GK, Kallenberg CG (1990) Prevention of relapses in Wegener’s granulomatosis by treatment based on antineutrophil cytoplasmic antibody titre. Lancet 336: 709–711
Stegeman CA, Cohen Tervaert JW, Sluiter WJ, Manson WL, de Jong PE, Kallenberg CG (1994) Association of chronic nasal carriage of Staphylococcus aureus and higher relapse rates in Wegener granulomatosis. Ann Intern Med 120: 12–17
Stegeman CA, Cohen Tervaert JW, de Jong PE, Kallenberg CG (1996) Trimethoprimsulfamethoxazole (co-trimoxazole) for the prevention of relapses of Wegener’s granulomatosis. Dutch Co-Trimoxazole Wegener Study Group. N Engl J Med 335: 16–20
Boomsma MM, Stegeman CA, van der Leij MJ, Oost W, Hermans J, Kallenberg CG, Limburg PC, Cohen Tervaert JW (2000) Prediction of relapses in Wegener’s granulomatosis by measurement of antineutrophil cytoplasmic antibody levels: a prospective study. Arthritis Rheum 43: 2025–2033
Jayne DR, Davies MJ, Fox CJ, Black CM, Lockwood CM (1991) Treatment of systemic vasculitis with pooled intravenous immunoglobulin. Lancet 337: 1137–1139
Jayne DR, Chapel H, Adu D, Misbah S, O’Donoghue D, Scott D, Lockwood CM (2000) Intravenous immunoglobulin for ANCA-associated systemic vasculitis with persistent disease activity. QJM 93: 433–439
Kjellstrand CM, Simmons RL, Uranga VM, Buselmeier TJ, Najarian JS (1974) Acute fulminant Wegener granulomatosis. Therapy with immunosuppression, hemodialysis, and renal transplantation. Arch Intern Med 134: 40–43
Hagen EC, de Keizer RJ, Andrassy K, van Boven WP, Bruijn JA, van Es LA, van der Woude FJ (1995) Compassionate treatment of Wegener’s granulomatosis with rabbit anti-thymocyte globulin. Clin Nephrol 43: 351–359
Lockwood CM, Thiru S, Isaacs JD, Hale G, Waldmann H (1993) Long-term remission of intractable systemic vasculitis with monoclonal antibody therapy. Lancet 341: 1620–1622
Van der Woude FJ, Schmitt WH, Birck R, Nowack R, Gobel U, Drexler JM, Hotta O (2000) New immodulating concepts in ANCA-associated disease. Clin Exp Immunol 120 (Suppl 1): 16
Lockwood CM (1998) Refractory Wegener’s granulomatosis: a model for shorter immunotherapy of autoimmune diseases. JR Coll Physicians Lond 32: 473–478
Cohen Tervaert JW, Kallenberg CG (2000) Leukocyte cell adhesion molecules in vasculitis. In: R Asherson, R Cervers, D Tripplet, S Abramson (eds): Vascular manifestations of systemic autoimmune diseases. CRC Press, London, 161–178
Lockwood CM, Elliott JD, Brettman L, Hale G, Rebello P, Frewin M, Ringler D, Merrill C, Waldmann H (1999) Anti-adhesion molecule therapy as an interventional strategy for autoimmune inflammation. Clin Immunol 93: 93–106
Tyndall A, Fassas A, Passweg J, Ruiz de Elvira C, Attal M, Brooks P, Black C, Durez P, Finke J, Forman S et al (1999) Autologous haematopoietic stem cell transplants for autoimmune disease — feasibility and transplant-related mortality. Autoimmune Disease and Lymphoma Working Parties of the European Group for Blood and Marrow Transplantation, the European League Against Rheumatism and the International Stem Cell Project for Autoimmune Disease. Bone Marrow Transplant 24: 729–734
Rai A, Nast C, Adler S (1999) Henoch-Schonlein purpura nephritis. J Am Soc Nephrol 10: 2637–2644
Hoffbrand BI (1991) Dapsone in Henoch-Schonlein purpura — worth a trial [editorial]. Postgrad Med J 67: 961–962
Albrecht J, Mempel M, Hein R, Abeck D, Ring J (1999) Henoch-Schonlein purpura: successful treatment with Dapsone. Hautarzt 50: 809–811
Niaudet P, Habib R (1998) Methylprednisolone pulse therapy in the treatment of severe forms of Schonlein-Henoch purpura nephritis. Pediatr Nephrol 12: 238–243
Hattori M, Ito K, Konomoto T, Kawaguchi H, Yoshioka T, Khono M (1999) Plasmapheresis as the sole therapy for rapidly progressive Henoch- Schonlein purpura nephritis in children. Am J Kidney Dis 33: 427–433
Agnello V (2000) Therapy for cryoglobulinemia secondary to hepatitis C virus: the need for tailored protocols and multiclinic studies [editorial]. J Rheumatol 27: 2065–2067
Casato M, Agnello V, Pucillo LP, Knight GB, Leoni M, Del Vecchio S, Mazzilli C, Antonelli G, Bonomo L (1997) Predictors of long-term response to high-dose interferon therapy in type II cryoglobulinemia associated with hepatitis C virus infection. Blood 90: 3865–3873
Zuckerman E, Keren D, Slobodin G, Rosner I, Rozenbaum M, Toubi E, Sabo E, Tsykounov I, Naschitz JE, Yeshurun D (2000) Treatment of refractory, symptomatic, hepatitis C virus related mixed cryoglobulinemia with ribavirin and interferon-alpha. J Rheumatol 27: 2172–2178
Calleja JL, Albillos A, Moreno-Otero R, Rossi I, Cacho G, Domper F, Yebra M, Escartin P (1999) Sustained response to interferon-alpha or to interferon-alpha plus ribavirin in hepatitis C virus-associated symptomatic mixed cryoglobulinaemia. Aliment Pharmacol Ther 13: 1179–1186
Kiyomoto H, Hitomi H, Hosotani Y, Hashimoto M, Uchida K, Kurokouchi K, Nagai M, Takahashi N, Fukunaga M, Mizushige K et al (1999) The effect of combination therapy with interferon and cryofiltration on mesangial proliferative glomerulonephritis originating from mixed cryoglobulinemia in chronic hepatitis C virus infection. Ther Apher 3: 329–333
Houwert DA, Hene RJ, Struyvenberg A, Kater L (1980) Effect of plasmapheresis (PP), corticosteroids and cyclophosphamide in essential mixed polyclonal cryoglobulinaemia associated with glomerulonephritis. Proc Eur Dial Transplant Assoc 17: 650–654
Mat C, Yurdakul S, Tuzuner N, Tuzun Y (1997) Small vessel vasculitis and vasculitis confined to skin. Baillieres Clin Rheumatol 11: 237–257
Petri M, Lakatta C, Magder L, Goldman D (1994) Effect of prednisone and hydroxychloroquine on coronary artery disease risk factors in systemic lupus erythematosus: a longitudinal data analysis. Am J Med 96: 254–259
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Tervaert, J.W.C., Stegeman, C.A., Kallenberg, C.G.M. (2001). Standard therapeutic regimens for vasculitis. In: Kallenberg, C.G.M., Tervaert, J.W.C. (eds) Disease-modifying Therapy in Vasculitides. Progress in Inflammation Research. Birkhäuser, Basel. https://doi.org/10.1007/978-3-0348-8235-4_2
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