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Fortschr Neurol Psychiatr 2002; 70(12): 663-677
DOI: 10.1055/s-2002-35855
Originalarbeit
© Georg Thieme Verlag Stuttgart · New York

Zerebrale Vaskulitis

Cerebral VasculitisP.  Berlit1 , A.  Steinbrecher2
  • 1Neurologische Klinik Alfried Krupp Krankenhaus Essen
  • 2Neurologische Universitätsklinik Regensburg
Auszüge der vorliegenden Arbeit wurden verwendet für die Erstellung der Leitlinie „Vaskulitis” der Deutschen Gesellschaft für Neurologie
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Publication History

Publication Date:
02 December 2002 (online)

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Zusammenfassung

In dieser Übersicht werden die isolierte ZNS-Vaskulitis, die zerebrale Beteiligung bei systemischen Vaskulitiden und Kollagenosen auf neuestem Stand dargestellt. ZNS-Vaskulitiden zeigen sich mit der Kombination von Kopfschmerzen, multifokalen Ausfällen und Enzephalopathie. In der Diagnostik sind MRT, digitale Subtraktionsangiographie, Liquor- und Serumbefunde hilfreich. Laborchemische und neuroradiologische Befunde sind aber letztlich unspezifisch - sie können die Verdachtsdiagnose einer Vaskulitis nie beweisen; mit Ausnahme des Behçet-Syndroms gilt für alle Vaskulitiden und Kollagenosen, dass die Diagnose bioptisch abgesichert werden muss, bevor eine immunsuppressive Therapie eingeleitet wird. Therapie der Wahl ist zumeist die kombinierte Gabe von Kortikosteroiden und Cyclophosphamid.

Abstract

This review focuses on isolated CNS-angiitis and cerebral involvement in systemic vasculitides and collagen vascular diseases. CNS-vasculitis presents with headache, multifocal symptoms, and encephalopathy, respectively. Clinical diagnosis is based on MRI, angiography and both CSF and serum examinations. But neither laboratory nor neuroradiological findings are specific - in all vasculitides with the exception of the Behçet-syndrome a biopsy is necessary before immunosuppressive treatment is started. Therapy of choice is the combination of corticosteroids and cyclophosphamide.

Literatur

  • 1 Padovan C S, Bise K, Hahn J. et al . Angiitis of the central nervous system after allogeneic bone marrow transplantation?.  Stroke. 1999;  30 1651-1656
    CrossrefPubMedSearch in Google Scholar
  • 2 Schmidley J W. Central nervous system angiitis. Boston: Butterworth-Heinemann 2000
    Search in Google Scholar
  • 3 Biller J, Loftus C M, Moore S A, Schelper R L, Danks K R, Cornell S H. Isolated central nervous system angiitis first presenting as spontaneous intracranial hemorrhage.  Neurosurgery. 1987;  20 310-315
    PubMedSearch in Google Scholar
  • 4 Calabrese L H, Mallek J A. Primary angiitis of the central nervous system. Report of 8 new cases, review of the literature, and proposal for diagnostic criteria.  Medicine (Baltimore). 1988;  67 20-39
    CrossrefPubMedSearch in Google Scholar
  • 5 Hankey G J. Isolated angiitis/angiopathy of the central nervous system.  Cerebrovasc Dis. 1991;  1 2-15
    PubMedSearch in Google Scholar
  • 6 Younger D S, Hays A P, Brust J C, Rowland L P. Granulomatous angiitis of the brain. An inflammatory reaction of diverse etiology.  Arch Neurol. 1988;  45 514-518
    PubMedSearch in Google Scholar
  • 7 Calabrese L H, Furlan A J, Gragg L A, Ropos T J. Primary angiitis of the central nervous system: diagnostic criteria and clinical approach.  Cleve Clin J Med. 1992;  59 293-306
    CrossrefPubMedSearch in Google Scholar
  • 8 Vollmer T L, Guarnaccia J, Harrington W, Pacia S V, Petroff O A. Idiopathic granulomatous angiitis of the central nervous system. Diagnostic challenges.  Arch Neurol. 1993;  50 925-930
    CrossrefPubMedSearch in Google Scholar
  • 9 Stone J H, Pomper M G, Roubenoff R, Miller T J, Hellmann D B. Sensitivities of noninvasive tests for central nervous system vasculitis: a comparison of lumbar puncture, computed tomography, and magnetic resonance imaging.  J Rheumatol. 1994;  21 1277-1282
    PubMedSearch in Google Scholar
  • 10 Pomper M G, Miller T J, Stone J H, Tidmore W C, Hellmann D B. CNS vasculitis in autoimmune disease: MR imaging findings and correlation with angiography.  Am J Neuroradiol. 1999;  20 75-85
    PubMedSearch in Google Scholar
  • 11 Alrawi A, Trobe J D, Blaivas M, Musch D C. Brain biopsy in primary angiitis of the central nervous system.  Neurology. 1999;  53 858-860
    CrossrefPubMedSearch in Google Scholar
  • 12 Chu C T, Gray L, Goldstein L B, Hulette C M. Diagnosis of intracranial vasculitis: a multi-disciplinary approach.  J Neuropathol Exp Neurol. 1998;  57 30-38
    CrossrefPubMedSearch in Google Scholar
  • 13 Duna G F, Calabrese L H. Limitations of invasive modalities in the diagnosis of primary angiitis of the central nervous system.  J Rheumatol. 1995;  22 662-667
    PubMedSearch in Google Scholar
  • 14 Parisi J E, Moore P M. The role of biopsy in vasculitis of the central nervous system.  Semin Neurol. 1994;  14 341-348
    Thieme ConnectPubMedSearch in Google Scholar
  • 15 Moore P M. Diagnosis and management of isolated angiitis of the central nervous system.  Neurology. 1989;  39 167-173
    CrossrefPubMedSearch in Google Scholar
  • 16 Berlit P. The spectrum of vasculopathies in the differential diagnosis of vasculitis.  Sem Neurol. 1994;  14 370-379
    PubMedSearch in Google Scholar
  • 17 Calabrese L H, Gragg L A, Furlan A J. Benign angiopathy. A distinct subset of angiographically defined primary angiitis of the CNS.  J Rheumatol. 1993;  20 2046-2050
    PubMedSearch in Google Scholar
  • 18 Woolfenden A R, Tong D C, Marks M P. et al . Angiographically defined primary angiitis of the CNS: Is it really benign?.  Neurology. 1998;  51 183-188
    PubMedSearch in Google Scholar
  • 19 Berlit P. Vaskulitis. In: Hamann-Siebler-von Scheidt (eds) Schlaganfall. Landsberg: Ecomed 2002
    Search in Google Scholar
  • 20 Caselli R J, Hunder G G. Giant cell (temporal) arteritis.  Neurol Clin. 1997;  15 893-902
    CrossrefPubMedSearch in Google Scholar
  • 21 Levine S M, Hellmann D B. Giant cell arteritis.  Curr Opin Rheumatol. 2002;  14 3-10
    CrossrefPubMedSearch in Google Scholar
  • 22 Berlit P. Giant Cell Arteritis. In: Lechtenberg R, Schutta HS (eds). Practice Guidelines for Neurologic Therapy. New York: Marcel Dekker 1997
    Search in Google Scholar
  • 23 Schmidt W A, Kraft H E, Vorpahl K, Völker L, Gromnica-Ihle E J. Color duplex ultrasonography in the diagnosis of temporal arteritis.  N Engl J Med. 1997;  337 1336-1342
    CrossrefPubMedSearch in Google Scholar
  • 24 De Silva M, Hazleman B L. Azathioprine in giant cell arteritis/polymyalgia rheumatica: a double-blind study.  Ann Rheum Dis. 1986;  45 136-138
    CrossrefPubMedSearch in Google Scholar
  • 25 Spiera R F, Mitnick H J, Kupersmith M, Richmond M, Spiera H, Peterson M G. et al . A prospective, double-blind, randomized, placebo controlled trial of methotrexate in the treatment of giant cell arteritis (GCA).  Clin Exp Rheumatol. 2001;  19 495-501
    PubMedSearch in Google Scholar
  • 26 Liozon F, Vidal E, Gaches F, Venot J, Liozon E, Cransac M, Loustaud V, Berdah J F. Death in Horton disease. Prognostic factors.  Rev Med Interne. 1992;  13 187-191
    PubMedSearch in Google Scholar
  • 27 Caselli R J, Hunder G G. Neurologic aspects of giant cell (temporal) arteritis.  Rheum Dis Clin North-Am. 1993;  19 941-953
    PubMedSearch in Google Scholar
  • 28 Hoffman G S, Leavitt R Y, Kerr G S, Rottem M, Sneller M C, Fauci A S. Treatment of glucocorticoid-resistant or relapsing Takayasu arteritis with methotrexate.  Arthritis Rheum. 1994;  37 578-582
    CrossrefPubMedSearch in Google Scholar
  • 29 Shelhamer J H, Volkman D J, Parrillo J E, Lawley T J, Johnston M R, Fauci A S. Takayasu's arteritis and its therapy.  Ann Intern Med. 1985;  103 121-126
    CrossrefPubMedSearch in Google Scholar
  • 30 Jennette J C, Falk R J, Andrassy K, Bacon P A, Churg J, Gross W L, Hagen E C, Hoffmann G S, Hunder G G, Kallenberg C G. et al . Nomenclature of systemic vasculitides. Proposal of an international consensus conference.  Arthritis-Rheum. 1994;  37 187-192
    CrossrefPubMedSearch in Google Scholar
  • 31 Watts R A, Jolliffe V A, Carruthers D M, Lockwood M, Scott D G. Effect of classification on the incidence of polyarteritis nodosa and microscopic polyangiitis.  Arthritis Rheum. 1996;  39 1208-1212
    PubMedSearch in Google Scholar
  • 32 Vassilopoulos D, Calabrese L H. Hepatitis C virus infection and vasculitis: implications of antiviral and immunosuppressive therapies.  Arthritis Rheum. 2002;  46 585-597
    CrossrefPubMedSearch in Google Scholar
  • 33 Langford C A. Treatment of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: where do we stand?.  Arthritis Rheum. 2001;  44 508-512
    CrossrefPubMedSearch in Google Scholar
  • 34 Gayraud M, Guillevin L, Toumelin P, Cohen P, Lhote F, Casassus P. et al . Long-term followup of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: analysis of four prospective trials including 278 patients.  Arthritis Rheum. 2001;  44 666-675
    CrossrefPubMedSearch in Google Scholar
  • 35 Gayraud M, Guillevin L, Cohen P, Lhote F, Cacoub P, Deblois P. et al . Treatment of good-prognosis polyarteritis nodosa and Churg-Strauss syndrome: comparison of steroids and oral or pulse cyclophosphamide in 25 patients. French Cooperative Study Group for Vasculitides.  Br J Rheumatol. 1997;  36 1290-1297
    CrossrefPubMedSearch in Google Scholar
  • 36 Haubitz M, Schellong S, Gobel U, Schurek H J, Schaumann D, Koch K M. et al . 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. 1998;  41 1835-1844
    CrossrefPubMedSearch in Google Scholar
  • 37 Hoffman G S, Kerr G S, Leavitt R Y, Hallahan C W, Lebovics R S, Travis W D. et al . Wegener granulomatosis: an analysis of 158 patients.  Ann Intern Med. 1992;  116 488-498
    CrossrefPubMedSearch in Google Scholar
  • 38 Reinhold-Keller E, Kekow J, Schnabel A, Schmitt W H, Heller M, Beigel A. et al . Influence of disease manifestation and antineutrophil cytoplasmic antibody titer on the response to pulse cyclophosphamide therapy in patients with Wegener’s granulomatosis.  Arthritis Rheum. 1994;  37 919-924
    CrossrefPubMedSearch in Google Scholar
  • 39 Guillevin L, Lhote F, Leon A, Fauvelle F, Vivitski L, Trepo C. Treatment of polyarteritis nodosa related to hepatitis B virus with short term steroid therapy associated with antiviral agents and plasma exchanges. A prospective trial in 33 patients.  J Rheumatol. 1993;  20 289-298
    PubMedSearch in Google Scholar
  • 40 Guillevin L, Lhote F, Cohen P, Sauvaget F, Jarrousse B, Lortholary O. et al . Polyarteritis nodosa related to hepatitis B virus. A prospective study with long-term observation of 41 patients.  Medicine. 1995;  74 238-253
    CrossrefPubMedSearch in Google Scholar
  • 41 Guillevin L, Lhote F, Gayraud M, Cohen P, Jarrousse B, Lortholary O, Thibult N, Casassus P. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients.  Medicine. 1996;  75 17-28
    CrossrefPubMedSearch in Google Scholar
  • 42 Lamprecht P, Gause A. Poly(Pan-)arteriitis nodosa, M. Kawasaki. In: Gross WL, ed. Therapie der Immunvaskulitiden. Bremen: UNI-MED 2000: 70-80
    Search in Google Scholar
  • 43 Groot K, Schmidt D K, Arlt A C, Gross W L, Reinhold-Keller E. Standardized neurologic evaluations of 128 patients with Wegener granulomatosis.  Arch Neurol. 2001;  58 1215-1221
    CrossrefPubMedSearch in Google Scholar
  • 44 Nishino H, Rubino F A, DeRemee R A, Swanson J W, Parisi J E. Neurological involvement in Wegener's granulomatosis. An analysis of 324 consecutive patients at the Mayo Clinic.  Ann Neurol. 1993;  33 4-9
    CrossrefPubMedSearch in Google Scholar
  • 45 Metzler C, Reinhold-Keller E. Wegenersche Granulomatose. In: Gross WL, editor. Therapie der Immunvaskulitiden. Bremen: UNI-MED 2000: 82-93
    Search in Google Scholar
  • 46 Bajema I M, Hagen E C. Evolving concepts about the role of antineutrophil cytoplasm autoantibodies in systemic vasculitides.  Curr Opin Rheumatol. 1999;  11 34-40
    CrossrefPubMedSearch in Google Scholar
  • 47 Hagen E C, Daha M R, Hermans J, Andrassy K, Csernok E, Gaskin G. et al . Diagnostic value of standardized assays for anti-neutrophil cytoplasmic antibodies in idiopathic systemic vasculitis. EC/BCR Project for ANCA Assay Standardization.  Kidney Int. 1998;  53 743-753
    CrossrefPubMedSearch in Google Scholar
  • 48 Jennette J C, Wilkman A S, Falk R J. Diagnostic predictive value of ANCA serology.  Kidney Int. 1998;  53 796-798
    CrossrefPubMedSearch in Google Scholar
  • 49 McRae D, Buchanan G. Long-term sulfamethoxazole-trimethoprim in Wegener's granulomatosis.  Arch Otolaryngol Head Neck Surg. 1993;  119 103-105
    PubMedSearch in Google Scholar
  • 50 Valeriano-Marcet J, Spiera H. Treatment of Wegener's granulomatosis with sulfamethoxazole-trimethoprim.  Arch Intern Med. 1991;  151 1649-1652
    CrossrefPubMedSearch in Google Scholar
  • 51 Guillevin L, Cordier J F, Lhote F, Cohen P, Jarrousse B, Royer I. et al . 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. 1997;  40 2187-2198
    CrossrefPubMedSearch in Google Scholar
  • 52 Steppat D, Gross W L. Stage-adapted treatment of Wegener's granulomatosis. First results of a prospective study.  Klin Wochenschr. 1989;  67 666-671
    CrossrefPubMedSearch in Google Scholar
  • 53 Groot K, Muhler M, Reinhold-Keller E, Paulsen J, Gross W L. Induction of remission in Wegener's granulomatosis with low dose methotrexate.  J Rheumatol. 1998;  25 492-495
    PubMedSearch in Google Scholar
  • 54 Langford C A, Talar-Williams C, Barron K S, Sneller M C. 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. 1999;  42 2666-2673
    CrossrefPubMedSearch in Google Scholar
  • 55 Sneller M C, Hoffman G S, Talar-Williams C, Kerr G S, Hallahan C W, Fauci A S. An analysis of forty-two Wegener's granulomatosis patients treated with methotrexate and prednisone.  Arthritis Rheum. 1995;  38 608-613
    CrossrefPubMedSearch in Google Scholar
  • 56 Stone J H, Tun W, Hellman D B. Treatment of non-life threatening Wegener's granulomatosis with methotrexate and daily prednisone as the initial therapy of choice.  J Rheumatol. 1999;  26 1134-1139
    PubMedSearch in Google Scholar
  • 57 De Groot K, Reinhold-Keller E, Tatsis E, Paulsen J, Heller M, Nolle B. et al . Therapy for the maintenance of remission in sixty-five patients with generalized Wegener's granulomatosis. Methotrexate versus trimethoprim/sulfamethoxazole.  Arthritis Rheum. 1996;  39 2052-2061
    PubMedSearch in Google Scholar
  • 58 Reinhold-Keller E, De Groot K, Rudert H, Nolle B, Heller M, Gross W L. Response to trimethoprim/sulfamethoxazole in Wegener's granulomatosis depends on the phase of disease.  QJM. 1996;  89 15-23
    CrossrefPubMedSearch in Google Scholar
  • 59 Stegeman C A, Cohen Tervaert J W, Jong P E, Kallenberg C G. Trimethoprim-sulfamethoxazole (co-trimoxazole) for the prevention of relapses of Wegener's granulomatosis. Dutch Co-Trimoxazole Wegener Study Group.  N Engl J Med. 1996;  335 16-20
    CrossrefPubMedSearch in Google Scholar
  • 60 Jayne D R, Chapel H, Adu D, Misbah S, O'Donoghue D, Scott D. et al . Intravenous immunoglobulin for ANCA-associated systemic vasculitis with persistent disease activity.  QJM. 2000;  93 433-439
    CrossrefPubMedSearch in Google Scholar
  • 61 Talar-Williams C, Hijazi Y M, Walther M M, Linehan W M, Hallahan C W, Lubensky I. et al . Cyclophosphamide-induced cystitis and bladder cancer in patients with Wegener granulomatosis.  Ann Intern Med. 1996;  124 477-484
    CrossrefPubMedSearch in Google Scholar
  • 62 Fauci A S, Haynes B F, Katz P, Wolff S M. Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years.  Ann Intern Med. 1983;  98 76-85
    CrossrefPubMedSearch in Google Scholar
  • 63 Girard T, Mahr A, Noel L H, Cordier J F, Lesavre P, Andre M H. et al . Are antineutrophil cytoplasmic antibodies a marker predictive of relapse in Wegener's granulomatosis? A prospective study.  Rheumatology (Oxford). 2001;  40 147-151
    CrossrefPubMedSearch in Google Scholar
  • 64 Chang Y, Kargas S A, Goates J J, Horoupian D S. Intraventricular and subarachnoid hemorrhage resulting from necrotizing vasculitis of the choroid plexus in a patient with Churg-Strauss syndrome.  Clin Neuropathol. 1993;  12 84-87
    PubMedSearch in Google Scholar
  • 65 Berlit P. Neurologische Manifestationen von Kollagenosen und Vaskulitiden.  Akt Neurol. 2000;  27 405-411
    PubMedSearch in Google Scholar
  • 66 Lanham J G, Elkon K B, Pusey C D, Hughes G R. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome.  Medicine (Baltimore). 1984;  63 65-81
    CrossrefPubMedSearch in Google Scholar
  • 67 Metzler C, Tatsis E. Churg-Strauss-Syndrom. In: Gross WL, ed. Therapie der Immunvaskulitiden. Bremen: UNI-MED 2000: 104-113
    Search in Google Scholar
  • 68 Tatsis E, Schnabel A, Gross W L. Interferon-alpha treatment of four patients with the Churg-Strauss syndrome.  Ann Intern Med. 1998;  129 370-374
    CrossrefPubMedSearch in Google Scholar
  • 69 De Groot K, Reinhold-Keller E. Mikroskopische Polyangiitis. In: Gross WL, ed. Therapie der Immunvaskulitiden. Bremen: UNI-MED 2000: 136
    Search in Google Scholar
  • 70 Askari A, Saadeh A, Buheis N I. Microscopic polyangiitis presenting as schizophrenia.  Rheumatol Int. 1999;  18 215-217
    CrossrefPubMedSearch in Google Scholar
  • 71 Kono H, Inokuma S, Nakayama H, Yamazaki J. Pachymeningitis in microscopic polyangiitis (MPA): a case report and a review of central nervous system involvement in MPA.  Clin Exp Rheumatol. 2000;  18 397-400
    PubMedSearch in Google Scholar
  • 72 Deshpande P V, Gilbert R, Alton H, Milford D V. Microscopic polyarteritis with renal and cerebral involvement.  Pediatr Nephrol. 2000;  15 134-135
    CrossrefPubMedSearch in Google Scholar
  • 73 Ehrlich G E. Vasculitis in Behçet's disease.  Int Rev Immunol. 1997;  14 81-88
    PubMedSearch in Google Scholar
  • 74 Yazici H, Yurdakul S, Hamuryudan V. Behçet's disease.  Curr Opin Rheumatol. 2001;  13 18-22
    CrossrefPubMedSearch in Google Scholar
  • 75 Kidd D, Steuer A, Denman A M, Rudge P. Neurological complications in Behçet's syndrome.  Brain. 1999;  122 2183-2194
    CrossrefPubMedSearch in Google Scholar
  • 76 Akman-Demir G, Serdaroglu P, Tasci B. Clinical patterns of neurological involvement in Behçet's disease: evaluation of 200 patients. The Neuro-Behçet Study Group.  Brain. 1999;  122 2171-2182
    CrossrefPubMedSearch in Google Scholar
  • 77 Coban O, Bahar S, Akman-Demir G, Tasci B, Yurdakul S, Yazici H. et al . Masked assessment of MRI findings: is it possible to differentiate neuro-Behçet's disease from other central nervous system diseases?.  Neuroradiology. 1999;  41 255-260
    CrossrefPubMedSearch in Google Scholar
  • 78 Serdaroglu P. Behçet's disease and the nervous system.  J Neurol. 1998;  245 197-205
    PubMedSearch in Google Scholar
  • 79 Sharief M K, Hentges R, Thomas E. Significance of CSF immunoglobulins in monitoring neurologic disease activity in Behçet's disease.  Neurology. 1991;  41 1398-1401
    PubMedSearch in Google Scholar
  • 80 Siva A, Fresko I I. Behçet’s Disease.  Curr Treat Options Neurol. 2000;  2 435-448
    PubMedSearch in Google Scholar
  • 81 Kaklamani V G, Kaklamanis P G. Treatment of Behçet's disease - an update.  Semin Arthritis Rheum. 2001;  30 299-312
    CrossrefPubMedSearch in Google Scholar
  • 82 Demiroglu H, Ozcebe O I, Barista I, Dundar S, Eldem B. Interferon alfa-2b, colchicine, and benzathine penicillin versus colchicine and benzathine penicillin in Behçet's disease: a randomised trial.  Lancet. 2000;  355 605-609
    CrossrefPubMedSearch in Google Scholar
  • 83 Sakane T, Takeno M, Suzuki N, Inaba G. Behçet's disease.  N Engl J Med. 1999;  341 1284-1291
    CrossrefPubMedSearch in Google Scholar
  • 84 Ruiz-Irastorza G, Khamashta M A, Castellino G, Hughes G R. Systemic lupus erythematosus.  Lancet. 2001;  357 1027-1032
    CrossrefPubMedSearch in Google Scholar
  • 85 Shlomchik M J, Craft J E, Mamula M J. From T to B and back again: positive feedback in systemic autoimmune disease.  Nature Rev Immunol. 2001;  1 147-153
    PubMedSearch in Google Scholar
  • 86 Sibbitt Jr W L, Sibbitt R R, Brooks W M. Neuroimaging in neuropsychiatric systemic lupus erythematosus.  Arthritis Rheum. 1999;  42 2026-2038
    CrossrefPubMedSearch in Google Scholar
  • 87 ACR Ad Hoc Committee on Neuropsychiatric Lupus Nomenclature . The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus.  Arthritis Rheum. 1999;  42 599-608
    CrossrefPubMedSearch in Google Scholar
  • 88 Morton S J, Powell R J. Management of systemic lupus erythematosus (SLE).  Clin Exp Allergy. 2001;  31 686-693
    CrossrefPubMedSearch in Google Scholar
  • 89 Mosca M, Ruiz-Irastorza G, Khamashta M A, Hughes G R. Treatment of systemic lupus erythematosus.  Int Immunopharmacol. 2001;  1 1065-1075
    PubMedSearch in Google Scholar
  • 90 Quisimorio F P. Systemic corticosteroid therapy in systemic lupus erythematosus. In: Wallace DJ and Hahn BH, eds. Dubois' lupus erythematosus. Baltimore: Williams & Wilkins 1997: 1141-1162
    Search in Google Scholar
  • 91 Ortmann R A, Klippel J H. Update on cyclophosphamide for systemic lupus erythematosus.  Rheum Dis Clin North Am. 2000;  26 363-375, vii
    PubMedSearch in Google Scholar
  • 92 Gourley M F, Austin H A, Scott D, Yarboro C H, Vaughan E M, Muir J. et al . Methylprednisolone and cyclophosphamide, alone or in combination, in patients with lupus nephritis. A randomized, controlled trial.  Ann Intern Med. 1996;  125 549-557
    PubMedSearch in Google Scholar
  • 93 Illei G G, Austin H A, Crane M, Collins L, Gourley M F, Yarboro C H. et al . Combination therapy with pulse cyclophosphamide plus pulse methylprednisolone improves long-term renal outcome without adding toxicity in patients with lupus nephritis.  Ann Intern Med. 2001;  135 248-257
    PubMedSearch in Google Scholar
  • 94 Takada K, Illei G G, Boumpas D T. Cyclophosphamide for the treatment of systemic lupus erythematosus.  Lupus. 2001;  10 154-161
    CrossrefPubMedSearch in Google Scholar
  • 95 D'Cruz D, Cuadrado M J, Mujic F, Tungekar M F, Taub N, Lloyd M. et al . Immunosuppressive therapy in lupus nephritis.  Clin Exp Rheumatol. 1997;  15 275-282
    PubMedSearch in Google Scholar
  • 96 Chan T M, Li F K, Hao W K, Chan K W, Lui S L, Tang S. et al . Treatment of membranous lupus nephritis with nephrotic syndrome by sequential immunosuppression.  Lupus. 1999;  8 545-551
    CrossrefPubMedSearch in Google Scholar
  • 97 Chan T M, Li F K, Tang C S, Wong R W, Fang G X, Ji Y L. et al . Efficacy of mycophenolate mofetil in patients with diffuse proliferative lupus nephritis. Hong Kong-Guangzhou Nephrology Study Group.  N Engl J Med. 2000;  343 1156-1162
    CrossrefPubMedSearch in Google Scholar
  • 98 Mok C C, Ho C T, Chan K W, Lau C S, Wong R W. Outcome and prognostic indicators of diffuse proliferative lupus glomerulonephritis treated with sequential oral cyclophosphamide and azathioprine.  Arthritis Rheum. 2002;  46 1003-1013
    CrossrefPubMedSearch in Google Scholar
  • 99 Ruiz-Irastorza G, Khamashta M A, Hughes G R. Antiaggregant and anticoagulant therapy in systemic lupus erythematosus and Hughes' syndrome.  Lupus. 2001;  10 241-245
    CrossrefPubMedSearch in Google Scholar
  • 100 Moore P, Richardson B. Neurology of the vasculitides and connective tissue diseases.  J Neurol Neurosurg Psychiat. 1998;  65 10-22
    CrossrefPubMedSearch in Google Scholar
  • 101 Alexander E L, Ranzenbach M R, Kumar A J, Kozachuk W E, Rosenbaum A E, Patronas N, Harley J B, Reichlin M. Anti-Ro (SS-A) autoantibodies in central nervous system disease associated with Sjögren's syndrome (CNS-SS): Clinical, neuroimaging, and angiographic correlates.  Neurology. 1994;  44 89-908
    PubMedSearch in Google Scholar

Prof. Dr med. P. Berlit

Alfried-Krupp-Krankenhaus

Alfried-Krupp-Straße 21

45117 Essen

Email: peter.berlit@krupp-krankenhaus.de