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
-
2 Schmidley J W. Central nervous system angiitis. Boston: Butterworth-Heinemann 2000
-
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
-
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
-
5
Hankey G J.
Isolated angiitis/angiopathy of the central nervous system.
Cerebrovasc Dis.
1991;
1
2-15
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
14
Parisi J E, Moore P M.
The role of biopsy in vasculitis of the central nervous system.
Semin Neurol.
1994;
14
341-348
-
15
Moore P M.
Diagnosis and management of isolated angiitis of the central nervous system.
Neurology.
1989;
39
167-173
-
16
Berlit P.
The spectrum of vasculopathies in the differential diagnosis of vasculitis.
Sem Neurol.
1994;
14
370-379
-
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
-
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
-
19 Berlit P. Vaskulitis. In: Hamann-Siebler-von Scheidt (eds) Schlaganfall. Landsberg: Ecomed 2002
-
20
Caselli R J, Hunder G G.
Giant cell (temporal) arteritis.
Neurol Clin.
1997;
15
893-902
-
21
Levine S M, Hellmann D B.
Giant cell arteritis.
Curr Opin Rheumatol.
2002;
14
3-10
-
22 Berlit P. Giant Cell Arteritis. In: Lechtenberg R, Schutta HS (eds). Practice Guidelines for Neurologic Therapy. New York: Marcel Dekker 1997
-
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
-
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
-
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
-
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
-
27
Caselli R J, Hunder G G.
Neurologic aspects of giant cell (temporal) arteritis.
Rheum Dis Clin North-Am.
1993;
19
941-953
-
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
-
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
-
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
-
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
-
32
Vassilopoulos D, Calabrese L H.
Hepatitis C virus infection and vasculitis: implications of antiviral and immunosuppressive therapies.
Arthritis Rheum.
2002;
46
585-597
-
33
Langford C A.
Treatment of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: where do we stand?.
Arthritis Rheum.
2001;
44
508-512
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
42 Lamprecht P, Gause A. Poly(Pan-)arteriitis nodosa, M. Kawasaki. In: Gross WL, ed. Therapie der Immunvaskulitiden. Bremen: UNI-MED 2000: 70-80
-
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
-
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
-
45 Metzler C, Reinhold-Keller E. Wegenersche Granulomatose. In: Gross WL, editor. Therapie der Immunvaskulitiden. Bremen: UNI-MED 2000: 82-93
-
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
-
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
-
48
Jennette J C, Wilkman A S, Falk R J.
Diagnostic predictive value of ANCA serology.
Kidney Int.
1998;
53
796-798
-
49
McRae D, Buchanan G.
Long-term sulfamethoxazole-trimethoprim in Wegener's granulomatosis.
Arch Otolaryngol Head Neck Surg.
1993;
119
103-105
-
50
Valeriano-Marcet J, Spiera H.
Treatment of Wegener's granulomatosis with sulfamethoxazole-trimethoprim.
Arch Intern Med.
1991;
151
1649-1652
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
65
Berlit P.
Neurologische Manifestationen von Kollagenosen und Vaskulitiden.
Akt Neurol.
2000;
27
405-411
-
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
-
67 Metzler C, Tatsis E. Churg-Strauss-Syndrom. In: Gross WL, ed. Therapie der Immunvaskulitiden. Bremen: UNI-MED 2000: 104-113
-
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
-
69 De Groot K, Reinhold-Keller E. Mikroskopische Polyangiitis. In: Gross WL, ed. Therapie der Immunvaskulitiden. Bremen: UNI-MED 2000: 136
-
70
Askari A, Saadeh A, Buheis N I.
Microscopic polyangiitis presenting as schizophrenia.
Rheumatol Int.
1999;
18
215-217
-
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
-
72
Deshpande P V, Gilbert R, Alton H, Milford D V.
Microscopic polyarteritis with renal and cerebral involvement.
Pediatr Nephrol.
2000;
15
134-135
-
73
Ehrlich G E.
Vasculitis in Behçet's disease.
Int Rev Immunol.
1997;
14
81-88
-
74
Yazici H, Yurdakul S, Hamuryudan V.
Behçet's disease.
Curr Opin Rheumatol.
2001;
13
18-22
-
75
Kidd D, Steuer A, Denman A M, Rudge P.
Neurological complications in Behçet's syndrome.
Brain.
1999;
122
2183-2194
-
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
-
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
-
78
Serdaroglu P.
Behçet's disease and the nervous system.
J Neurol.
1998;
245
197-205
-
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
-
80
Siva A, Fresko I I.
Behçet’s Disease.
Curr Treat Options Neurol.
2000;
2
435-448
-
81
Kaklamani V G, Kaklamanis P G.
Treatment of Behçet's disease - an update.
Semin Arthritis Rheum.
2001;
30
299-312
-
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
-
83
Sakane T, Takeno M, Suzuki N, Inaba G.
Behçet's disease.
N Engl J Med.
1999;
341
1284-1291
-
84
Ruiz-Irastorza G, Khamashta M A, Castellino G, Hughes G R.
Systemic lupus erythematosus.
Lancet.
2001;
357
1027-1032
-
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
-
86
Sibbitt Jr W L, Sibbitt R R, Brooks W M.
Neuroimaging in neuropsychiatric systemic lupus erythematosus.
Arthritis Rheum.
1999;
42
2026-2038
-
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
-
88
Morton S J, Powell R J.
Management of systemic lupus erythematosus (SLE).
Clin Exp Allergy.
2001;
31
686-693
-
89
Mosca M, Ruiz-Irastorza G, Khamashta M A, Hughes G R.
Treatment of systemic lupus erythematosus.
Int Immunopharmacol.
2001;
1
1065-1075
-
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
-
91
Ortmann R A, Klippel J H.
Update on cyclophosphamide for systemic lupus erythematosus.
Rheum Dis Clin North Am.
2000;
26
363-375, vii
-
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
-
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
-
94
Takada K, Illei G G, Boumpas D T.
Cyclophosphamide for the treatment of systemic lupus erythematosus.
Lupus.
2001;
10
154-161
-
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
-
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
-
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
-
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
-
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
-
100
Moore P, Richardson B.
Neurology of the vasculitides and connective tissue diseases.
J Neurol Neurosurg Psychiat.
1998;
65
10-22
-
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
Prof. Dr med. P. Berlit
Alfried-Krupp-Krankenhaus
Alfried-Krupp-Straße 21
45117 Essen
Email: peter.berlit@krupp-krankenhaus.de
