Nonsystemic vasculitic neuropathy: Update on diagnosis, classification, pathogenesis, and treatment

Frontiers of Neurology and Neuroscience

Volumn 26, Issue , 2009, Pages 26-66

  Collins, Michael P ^a   Periquet Collins, Isabel ^a  

^a MEDICAL COLLEGE OF WISCONSIN   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

15 DEOXYSPERGUALIN; ALEMTUZUMAB; AZATHIOPRINE; C REACTIVE PROTEIN; CORTICOSTEROID; CYCLOPHOSPHAMIDE; HEMOSIDERIN; IMMUNOGLOBULIN; INFLIXIMAB; LEFLUNOMIDE; METHOTREXATE; METHYLPREDNISOLONE; MYCOPHENOLIC ACID; MYCOPHENOLIC ACID 2 MORPHOLINOETHYL ESTER; NEUTROPHIL CYTOPLASMIC ANTIBODY; PREDNISOLONE; PREDNISONE; RHEUMATOID FACTOR; RITUXIMAB; THYMOCYTE ANTIBODY;

ALLERGIC VASCULITIS; ANAPHYLACTOID PURPURA; AORTA ARCH SYNDROME; ARM; ARTERIOLE; BEHCET DISEASE; BIOPSY; BLADDER CANCER; BLOOD VESSEL; CELL INFILTRATION; CEREBROSPINAL FLUID; CHRONIC PAIN; CHURG STRAUSS SYNDROME; CLINICAL TRIAL; CONTINUOUS INFUSION; CRANIAL NERVE; CYTOMEGALOVIRUS INFECTION; CYTOTOXICITY; DERMATOMYOSITIS; DIABETIC NEUROPATHY; DISEASE CLASSIFICATION; DISEASE SEVERITY; DRUG DOSE REDUCTION; DRUG MEGADOSE; DRUG SUBSTITUTION; DRUG WITHDRAWAL; ELECTRODIAGNOSIS; ENTERITIS; ERYTHROCYTE SEDIMENTATION RATE; FEVER; GIANT CELL ARTERITIS; GONADAL DISEASE; HEPATITIS B; HEPATITIS C; HUMAN; HUMAN T CELL LEUKEMIA VIRUS 1; IMMUNE COMPLEX DEPOSITION; IMMUNE RESPONSE; INFECTION; INFLAMMATION; LEG; LEPROSY; LEUKOCYTOSIS; LEUKOPENIA; LIMB WEAKNESS; LIVER DYSFUNCTION; LOW DRUG DOSE; LYME DISEASE; LYMPHOMA; MACROPHAGE; MICROSCOPIC POLYANGIITIS; MIXED CONNECTIVE TISSUE DISEASE; MONOTHERAPY; MORTALITY; MUSCLE NECROSIS; MUSCLE REGENERATION; NEOPLASM; NEOVASCULARIZATION (PATHOLOGY); NERVE BLOCK; NERVE DEGENERATION; NERVE FIBER DEGENERATION; NEUROPATHY; NONSYSTEMIC VASCULITIC NEUROPATHY; OVARY INSUFFICIENCY; PAIN; PARESTHESIA; PATHOGENESIS; PERIPHERAL NERVOUS SYSTEM; PERONEUS NERVE; PLASMAPHERESIS; POLYARTERITIS NODOSA; PRIORITY JOURNAL; PURPURA; RELAPSE; REMISSION; REVIEW; RHEUMATOID ARTHRITIS; SARCOIDOSIS; SCLERODERMA; SEDIMENTATION RATE; SENSORY DYSFUNCTION; SJOEGREN SYNDROME; SURVIVAL; SYSTEMIC LUPUS ERYTHEMATOSUS; SYSTEMIC VASCULITIC NEUROPATHY; T LYMPHOCYTE; TREATMENT OUTCOME; ULNAR NERVE; UNSPECIFIED SIDE EFFECT; VASCULARIZATION; VASCULITIS; WEGENER GRANULOMATOSIS; WEIGHT REDUCTION; CLASSIFICATION; PATHOPHYSIOLOGY; PERIPHERAL NEUROPATHY;

HUMANS; PERIPHERAL NERVOUS SYSTEM DISEASES; VASCULITIS;

EID: 67649726479     PISSN: 16604431     EISSN: 16622804     Source Type: Book Series    
DOI: 10.1159/000212368     Document Type: Review

References (267)

1. Davies DJ: Small vessel vasculitis. Cardiovasc Pathol 2005;14:335-346.
2. Jennette JC, Falk RJ: Nosology of primary vasculitis. Curr Opin Rheumatol 2007;19:10-16.
3. Kussmaul A, Maier R: Ueber eine bisher nicht be schriebene eigenthümliche Arterienerkrankung (Periarteritis nodosa), die mit Morbus Brightii und rapid fortschreitender allgemeiner Muskellähmung einhergeht. Dtsch Arch Klin Med 1866;1:484-515.
4. Ferrari E: Ueber polyarteritis acuta nodosa (soge-nannte Periarteritis nodosa) und ihre Beziehungen zur Polymositis und Polyneuritis acuta. Beitr Pathol Anat 1903;34:350.
5. Hutchinson J: Diseases of the arteries. On a peculiar form of thrombotic arteritis of the aged which is sometimes productive of gangrene. Arch Surg (Lond) 1890;1:323-329.
6. Horton BT, Magath TB, Brown GE: An undescribed form of arteritis of the temporal vessels. Proc Staff Meet Mayo Clin 1932;7:700-701.
7. Klinger H: Grenzformen der Periarteritis nodosa. Frankf Z Pathol 1931;42:455.
8. Wegener F: Ueber generalisierte, septische Gefässerkrankungen. Verh Dtsch Ges Pathol 1936; 29:202-210.
9. Godman GC, Churg J: Wegener's granulomatosis. Pathology and review of the literature. Arch Pathol 1954;58:533-553.
10. Wohlwill F: Ueber die nur mikroskopisch erkennbare Form der Periarteritis nodosa. Arch Pathol Anat 1923;246:377.
11. Davson J, Ball J, Platt R: The kidney in periarteritis nodosa. Q J Med 1943;17:175-202.
12. Zeek PM, Smith CC, Weeter JC: Studies on periar-teritis nodosa. 3. The differentiation between the vascular lesions of periarteritis nodosa and of hypersensitivity. Am J Pathol 1948;24:889-917.
13. Gairdner D: The Schönlein-Henoch syndrome (anaphylactoid purpura). Q J Med 1948;17:95-122.
14. Churg J, Strauss L: Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 1951; 27:277-301.
15. Bannatyne GA: Rheumatoid Arthritis: Its Pathology, Morbid Anatomy and Treatment. Bristol, Wright, 1898.
16. Ball J: Rheumatoid arthritis and polyarteritis nodosa. Ann Rheum Dis 1954;13:277-290.
17. Cruickshank B: The arteritis of rheumatoid arthritis. Ann Rheum Dis 1954;13:136-146.
18. Klemperer P, Pollack AD, Baehr G: Pathology of disseminated lupus erythematosus. Arch Pathol 1941;32:569-631.
19. Hunder GG, Arend W P, Bloch DA, Calabrese LH, Fauci AS, Fries J F, Leavitt RY, Lie J T, Lightfoot RW Jr, Masi AT: The American College of Rheumatology 1990 criteria for the classification of vasculitis. Introduction. Arthritis Rheum 1990;33:1065-1067.
20. Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, Hagen EC, Hoffman GS, Hunder GG, Kallenberg CG: Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 1994;37:187-192.
21. Henegar C, Pagnoux C, Puechal X, Zucker JD, Bar-Hen A, Le Guern V, Saba M, Bagneres D, Meyer O, Guillevin L: A paradigm of diagnostic criteria for polyarteritis nodosa: analysis of a series of 949 patients with vasculitides. Arthritis Rheum 2008;58: 1528-1538.
22. Bosch X, Guilabert A, Font J: Antineutrophil cytoplasmic antibodies. Lancet 2006;368:404-418.
23. Puechal X: Antineutrophil cytoplasmic antibody-associated vasculitides. Joint Bone Spine 2007;74: 427-435.
24. Beauvillain C, Delneste Y, Renier G, Jeannin P, Subra JF, Chevailler A: Antineutrophil cytoplasmic autoantibodies: how should the biologist manage them? Clin Rev Allergy Immunol 2008;35:47-58.
25. Pagnoux C, Guilpain P, Guillevin L: Churg-Strauss syndrome. Curr Opin Rheumatol 2007;19:25-32.
26. Jayne D: Challenges in the management of microscopic polyangiitis: past, present and future. Curr Opin Rheumatol 2008;20:3-9.
27. Sarraf P, Sneller MC: Pathogenesis of Wegener's granulomatosis: current concepts. Expert Rev Mol Med 2005;7:1-19.
28. Jennette JC, Falk RJ: New insight into the pathogenesis of vasculitis associated with antineutrophil cytoplasmic autoantibodies. Curr Opin Rheumatol 2008;20:55-60.
29. Kallenberg CG: Pathogenesis of PR3-ANCA associated vasculitis. J Autoimmun 2008;30:29-36.
30. Smyth L, Gaskin G, Pusey CD: Microscopic polyangiitis. Semin Respir Crit Care Med 2004;25:523-533.
31. Collins M P, Kissel JT: Neuropathies with systemic vasculitis; in Dyck PJ, Thomas PK (eds): Peripheral Neuropathy. Philadelphia, Elsevier Saunders, 2005, pp 2335-2404.
32. Keogh KA, Specks U: Churg-Strauss syndrome. Semin Respir Crit Care Med 2006;27:148-157.
33. Lanham JG, Elkon KB, Pusey CD, Hughes GR: Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Medicine (Baltimore) 1984;63:65-81.
34. Bruce IN, Bell AL: A comparison of two nomenclature systems for primary systemic vasculitis. Br J Rheumatol 1997;36:453-458.
35. Watts R, Lane S, Hanslik T, Hauser T, Hellmich B, Koldingsnes W, Mahr A, Segelmark M, Cohen-Tervaert JW, Scott D: Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis 2007;66:222-227.
36. Burke A P, Virmani R: Localized vasculitis. Semin Diagn Pathol 2001;18:59-66.
37. Hernandez-Rodriguez J, Molloy ES, Hoffman GS: Single-organ vasculitis. Curr Opin Rheumatol 2008;20:40-46.
38. Salvarani C, Brown RD Jr, Calamia KT, Christianson TJ, Weigand SD, Miller D V, Giannini C, Meschia JF, Huston J 3rd, Hunder GG: Primary central nervous system vasculitis: analysis of 101 patients. Ann Neurol 2007;62:442-451.
39. Diaz-Perez JL, De Lagran ZM, Diaz-Ramon JL, Winkelmann RK: Cutaneous polyarteritis nodosa. Semin Cutan Med Surg 2007;26:77-86.
40. Gorriz JL, Sancho A, Ferrer R, Alcoy E, Crespo JF, Palmero J, Pallardo LM: Renal-limited polyarteritis nodosa presenting with loin pain and haematuria. Nephrol Dial Transplant 1997;12:2737-2739.
41. George RK, Walton RC, Whitcup SM, Nussenblatt RB: Primary retinal vasculitis. Systemic associations and diagnostic evaluation. Ophthalmology 1996; 103:384-389.
42. Khellaf M, Hamidou M, Pagnoux C, Michel M, Brisseau JM, Chevallier X, Cohen P, Guillevin L, Godeau B: Vasculitis restricted to the lower limbs: a clinical and histopathological study. Ann Rheum Dis 2007;66:554-556.
43. Lepper PM, Koenig W, Moller P, Perner S: A case of sudden cardiac death due to isolated eosinophilic coronary arteritis. Chest 2005;128:1047-1050.
44. Jennings CA, King TE Jr, Tuder R, Cherniack RM, Schwarz MI: Diffuse alveolar hemorrhage with underlying isolated, pauciimmune pulmonary capillaritis. Am J Respir Crit Care Med 1997;155:1101-1109.
45. Gonzalez-Gay MA, Vazquez-Rodriguez TR, Miranda-Filloy JA, Pazos-Ferro A, Garcia-Rodeja E: Localized vasculitis of the gastrointestinal tract: a case report and literature review. Clin Exp Rheumatol 2008;26:S101-S104.
46. Hoppe E, de Ybarlucea LR, Collet J, Dupont J, Fabiani B, Puechal X: Isolated vasculitis of the female genital tract: a case series and review of literature. Virchows Arch 2007;451:1083-1089.
47. Hernandez-Rodriguez J, Tan CD, Molloy ES, Khasnis A, Rodriguez ER, Hoffman GS: Vasculitis involving the breast: a clinical and histopathologic analysis of 34 patients. Medicine (Baltimore) 2008; 87:61-69.
48. Miller DV, Isotalo PA, Weyand CM, Edwards WD, Aubry MC, Tazelaar HD: Surgical pathology of noninfectious ascending aortitis: a study of 45 cases with emphasis on an isolated variant. Am J Surg Pathol 2006;30:1150-1158.
49. Collins M P, Periquet MI: Isolated vasculitis of the peripheral nervous system. Clin Exp Rheumatol 2008;26:S118-S130.
50. Cattaneo L, Chierici E, Pavone L, Grasselli C, Manganelli P, Buzio C, Pavesi G: Peripheral neuropathy in Wegener's granulomatosis, Churg-Strauss syndrome and microscopic polyangiitis. J Neurol Neurosurg Psychiatry 2007;78:1119-1123.
51. Guillevin L, Mahr A, Callard P, Godmer P, Pagnoux C, Leray E, Cohen P: Hepatitis B virus-associated polyarteritis nodosa: clinical characteristics, outcome, and impact of treatment in 115 patients. Medicine (Baltimore) 2005;84:313-322.
52. Ferri C, Sebastiani M, Giuggioli D, Cazzato M, Longombardo G, Antonelli A, Puccini R, Michelassi C, Zignego AL: Mixed cryoglobulinemia: demographic, clinical, and serologic features and survival in 231 patients. Semin Arthritis Rheum 2004;33: 355-374.
53. Collins M P, Periquet-Collins I, Kissel JT: Vasculitis of the peripheral nervous system; in Noseworthy JH (ed): Neurological Therapeutics Principles and Practice. London, Informa, 2006, vol 2, pp 2348-2382.
54. Kissel JT, Collins M P, Katirji B, Kaminski HJ, Preston DC, Ruff RL, Shapiro BE: Vasculitic neuropathies and neuropathies of connective tissue disorders; in Katirji B, Kaminski HJ, Preston DC (eds): Neuromuscular Disorders in Clinical Practice. Boston, Butterworth Heinemann, 2002, vol 1, pp 669-702.
55. Genta MS, Genta RM, Gabay C: Systemic rheumatoid vasculitis: a review. Semin Arthritis Rheum 2006;36:88-98.
56. Kernohan J W, Woltman HW: Periarteritis nodosa: a clinicopathological study with special reference to the nervous system. Arch Neurol Psychiatry 1938;39:655-686.
57. Minkowski O: Beiträge zur Pathologie der multiplen Neuritis; in Naunyn B (ed): Mittheilungen aus der medizinischen Klinik zu Königsberg i.Pr. Leipzig, Vogel, 1888, vol 8, pp 59ff.
58. Lorenz H: Beitrag zur Kenntniss der multiplen degenerativen Neuritis. Z Klin Med 1891;18:493-516.
59. Joffroy A, Achard C: Névrite périphérique d'origine vasculaire. Arch Med Exp 1889;1:229-240.
60. Kissel JT, Slivka A P, Warmolts JR, Mendell JR: The clinical spectrum of necrotizing angiopathy of the peripheral nervous system. Ann Neurol 1985;18: 251-257.
61. Dyck PJ, Benstead TJ, Conn DL, Stevens JC, Windebank AJ, Low PA: Nonsystemic vasculitic neuropathy. Brain 1987;110:843-853.
62. Collins M P, Periquet MI: Non-systemic vasculitic neuropathy. Curr Opin Neurol 2004;17:587-598.
63. Brune MB, Walton S P, Varga D: Extensive mononeuritis multiplex as a solitary presentation of polyarteritis nodosa. J Ky Med Assoc 1995;93:15-18.
64. Saif M, Ciccone E, Grunnet ML: A case of vasculitic neuropathy. Consultant 1999;39:3135-3141.
65. Said G, Lacroix-Ciaudo C, Fujimura H, Blas C, Faux N: The peripheral neuropathy of necrotizing arteritis: a clinicopathological study. Ann Neurol 1988; 23:461-465.
66. Davies L, Spies JM, Pollard JD, McLeod JG: Vasculitis confined to peripheral nerves. Brain 1996; 119:1441-1448.
67. Collins M P, Periquet MI, Mendell JR, Sahenk Z, Nagaraja HN, Kissel JT: Nonsystemic vasculitic neuropathy: insights from a clinical cohort. Neurology 2003;61:623-630.
68. Kararizou E, Davaki P, Karandreas N, Davou R, Vassilopoulos D: Nonsystemic vasculitic neuropathy: a clinicopathological study of 22 cases. J Rheumatol 2005;32:853-858.
69. Sugiura M, Koike H, Iijima M, Mori K, Hattori N, Katsuno M, Tanaka F, Sobue G: Clinicopathologic features of nonsystemic vasculitic neuropathy and microscopic polyangiitis-associated neuropathy: a comparative study. J Neurol Sci 2006;241:31-37.
70. Bennett DL, Groves M, Blake J, Holton JL, King RH, Orrell RW, Ginsberg L, Reilly MM: The use of nerve and muscle biopsy in the diagnosis of vasculitis: a 5 year retrospective study. J Neurol Neurosurg Psychiatry 2008;79:1376-1381.
71. Said G, Lacroix C: Primary and secondary vasculitic neuropathy. J Neurol 2005;252:633-641.
72. Yamanaka Y, Hiraga A, Arai K, Mori M, Kanai K, Hattori T, Kuwabara S, Oide T, Tanizawa T: Leucocytoclastic vasculitic neuropathy diagnosed by biopsy of normal appearing skin. J Neurol Neurosurg Psychiatry 2006;77:706-707.
73. Vital C, Vital A, Canron M-H, Jaffre A, Viallard J-F, Ragnaud J-M, Brechenmacher C, Lagueny A: Combined nerve and muscle biopsy in the diagnosis of vasculitic neuropathy. A 16-year retrospective study of 202 cases. J Peripher Nerv Syst 2006;11:20-29.
74. Burns TM, Schaublin GA, Dyck PJB: Vasculitic neuropathies. Neurol Clin 2007;25:89-113.
75. Rosenbaum JT, Bell LA, Rosenbaum RB: Nonsystemic necrotizing vasculitis of the peripheral nervous system. Arthritis Rheum 1998;41:1324-1325.
76. Midroni G, Bilbao JM: The peripheral nerve vasculature; in Midroni G, Bilbao JM (eds): Biopsy Diagnosis of Peripheral Neuropathy. Boston, Butterworth-Heinemann, 1995, pp 105-117.
77. Watts RA, Lane SE, Bentham G, Scott DG: Epidemiology of systemic vasculitis: a ten-year study in the United Kingdom. Arthritis Rheum 2000;43:414-419.
78. Gonzalez-Gay MA, Garcia-Porrua C: Epidemiology of the vasculitides. Rheum Dis Clin North Am 2001;27:729-749.
79. Reinhold-Keller E, Herlyn K, Wagner-Bastmeyer R, Gutfleisch J, Peter HH, Raspe HH, Gross WL: No difference in the incidences of vasculitides between north and south Germany: first results of the German vasculitis register. Rheumatology (Oxford) 2002;41:540-549.
80. Gonzalez-Gay MA, Garcia-Porrua C: Systemic vasculitis in adults in northwestern Spain, 1988-1997. Clinical and epidemiologic aspects. Medicine (Baltimore) 1999;78:292-308.
81. Dadoniene J, Kirdaite G, Mackiewicz Z, Rimkevicius A, Haugeberg G: Incidence of primary systemic vasculitides in Vilnius: a university hospital population based study. Ann Rheum Dis 2005;64:335-336.
82. Mahr A, Guillevin L, Poissonnet M, Ayme S: Prevalences of polyarteritis nodosa, microscopic polyangiitis, Wegener's granulomatosis, and Churg-Strauss syndrome in a French urban multiethnic population in 2000: a capture-recapture estimate. Arthritis Rheum 2004;51:92-99.
83. Mohammad AJ, Jacobsson LT, Mahr AD, Sturfelt G, Segelmark M: Prevalence of Wegener's granulomatosis, microscopic polyangiitis, polyarteritis nodosa and Churg-Strauss syndrome within a defined population in southern Sweden. Rheumatology (Oxford) 2007;46:1329-1337.
84. Ormerod AS, Cook MC: Epidemiology of primary systemic vasculitis in the Australian Capital Territory and south-eastern New South Wales. Intern Med J 2008;38:816-823.
85. Chio A, Cocito D, Bottacchi E, Buffa C, Leone M, Plano F, Mutani R, Calvo A: Idiopathic chronic inflammatory demyelinating polyneuropathy: an epidemiological study in Italy. J Neurol Neurosurg Psychiatry 2007;78:1349-1353.
86. Collins M P, Mendell JR, Periquet MI, Sahenk Z, Amato AA, Gronseth GS, Barohn RJ, Jackson CE, Kissel JT: Superficial peroneal nerve/peroneus brevis muscle biopsy in vasculitic neuropathy. Neurology 2000;55:636-643.
87. Garces Sanchez M, Villanueva-Haba VE, Sevilla Mantecon T, Mayordomo Fernandez F, Vilchez Padilla JJ: Nonsystemic vasculitic neuropathy. Neurologia 2002;17:616-620.
88. Schweikert K, Fuhr P, Probst A, Tolnay M, Renaud S, Steck AJ: Contribution of nerve biopsy to unclassified neuropathy. Eur Neurol 2007;57:86-90.
89. Harris AW, Lynch G W, O'Hare JP: Periarteritis nodosa. Arch Intern Med 1939;63:1163-1182.
90. Lamb AR: Periarteritis nodosa - A clinical and pathological review of the disease with a report of two cases. Arch Intern Med 1914;14:481-516.
91. Curtis AC, Coffey RM: Periarteritis nodosa. A brief review of the literature and a report of one case. Ann Intern Med 1934;7:1345-1358.
92. Gruber GB: Kasuistik und Kritik der Periarteriitis nodosa. Zentralbl Herz Gefasskr 1926;18:145-158; 165-178; 185-198; 205-213; 245-255; 269-277.
93. Rose GA, Spencer H: Polyarteritis nodosa. Q J Med 1957;26:43-81.
94. Walton EW: Giant cell granuloma of the respiratory tract (Wegener's granulomatosis). Br Med J 1958;2: 265-270.
95. Macfarlane DG, Bourne JT, Dieppe PA, Easty DL: Indolent Wegener's granulomatosis. Ann Rheum Dis 1983;42:398-407.
96. Anderson G, Coles ET, Crane M, Douglas AC, Gibbs AR, Geddes DM, Peel ET, Wood JB: Wegener's gran-uloma. A series of 265 British cases seen between 1975 and 1985. A report by a sub-committee of the British Thoracic Society Research Committee. Q J Med 1992;83:427-438.
97. Schleiffer T, Burkhard B, Klooker P, Brass H: Clinical course and symptomatic prediagnostic period of patients with Wegener's granulomatosis and microscopic polyangiitis. Ren Fail 1998;20:519-532.
98. Seo JH, Ryan HF, Claussen GC, Thomas TD, Oh SJ: Sensory neuropathy in vasculitis: a clinical, pathologic, and electrophysiologic study. Neurology 2004; 63:874-878.
99. Behari M, Garg R, Dinda AK, Ahuja GK: Peripheral neuropathy due to small vessel vasculitis. J Assoc Physicians India 1995;43:495-497.
100. Larrode P, Ramon YCS, Iniguez C, Dominguez M, Sanz JM, Morales F: Isolated vasculitis of the peripheral nervous system (in Spanish). Neurologia 1997; 12:197-199.
101. De la Sayette V, Macro M, Diraison P, Bertran F, Lechevalier B, Chapon F: Acute necrotizing vasculi-tis (polyarteritis nodosa?) confined to the nerve with spontaneous recovery. Br J Rheumatol 1995;34:694-695.
102. Mathew L, Talbot K, Love S, Puvanarajah S, Donaghy M: Treatment of vasculitic peripheral neuropathy: a retrospective analysis of outcome. Q J Med 2007; 100:41-51.
103. Borrie P: Cutaneous polyarteritis nodosa. Br J Dermatol 1972;87:87-95.
104. Daoud MS, Hutton KP, Gibson LE: Cutaneous periarteritis nodosa: a clinicopathological study of 79 cases. Br J Dermatol 1997;136:706-713.
105. Ishibashi M, Chen KR: A morphological study of evolution of cutaneous polyarteritis nodosa. Am J Dermatopathol 2008;30:319-326.
106. Nakamura T, Kanazawa N, Ikeda T, Yamamoto Y, Nakabayashi K, Ozaki S, Furukawa F: Cutaneous polyarteritis nodosa: revisiting its definition and diagnostic criteria. Arch Dermatol Res 2009;301: 117-121.
107. Barohn RJ, Sahenk Z, Warmolts JR, Mendell JR: The Bruns-Garland syndrome (diabetic amyotrophy). Revisited 100 years later. Arch Neurol 1991;48:1130-1135.
108. Dyck PJ, Norell JE, Dyck PJ: Microvasculitis and ischemia in diabetic lumbosacral radiculoplexus neuropathy. Neurology 1999;53:2113-2121.
109. Kelkar P, Masood M, Parry GJ: Distinctive pathologic findings in proximal diabetic neuropathy (diabetic amyotrophy). Neurology 2000;55:83-88.
110. Dyck PJ, Windebank AJ: Diabetic and nondiabetic lumbosacral radiculoplexus neuropathies: new insights into pathophysiology and treatment. Muscle Nerve 2002;25:477-491.
111. Katz JS, Saperstein DS, Wolfe G, Nations SP, Alkhersam H, Amato AA, Barohn RJ: Cervicobrachial involvement in diabetic radiculoplexopathy. Muscle Nerve 2001;24:794-798.
112. van Alfen N, van Engelen BG: The clinical spectrum of neuralgic amyotrophy in 246 cases. Brain 2006; 129:438-450.
113. Dyck PJ, Engelstad J, Norell J, Dyck PJ: Microvasculitis in non-diabetic lumbosacral radiculoplexus neuropathy (LSRPN): similarity to the diabetic variety (DLSRPN). J Neuropathol Exp Neurol 2000; 59:525-538.
114. Dyck PJ, Norell JE, Dyck PJ: Non-diabetic lumbosacral radiculoplexus neuropathy: natural history, outcome and comparison with the diabetic variety. Brain 2001;124:1197-1207.
115. Bradley WG, Chad D, Verghese J P, Liu HC, Good P, Gabbai AA, Adelman LS: Painful lumbosacral plexopathy with elevated erythrocyte sedimentation rate: a treatable inflammatory syndrome. Ann Neurol 1984;15:457-464.
116. Collins M P, Kissel JT, Noseworthy JH: Vasculitis of the peripheral nervous system; in Noseworthy JH: Neurological Therapeutics: Principles and Practice. London, Dunitz, 2003, pp 2078-2110.
117. Olney RK: AAEM minimonograph #38: neuropathies in connective tissue disease. Muscle Nerve 1992;15:531-542.
118. Parry G: AAEE case report #11: mononeuropathy multiplex. Muscle Nerve 1985;8:493-498.
119. Claussen GC, Thomas TD, Goyne C, Vazquez LG, Oh SJ: Diagnostic value of nerve and muscle biopsy in suspected vasculitis cases. J Clin Neuromusc Dis 2000;1:117-123.
120. Zivkovic SA, Ascherman D, Lacomis D: Vasculitic neuropathy - Electrodiagnostic findings and association with malignancies. Acta Neurol Scand 2007; 115:432-436.
121. Jamieson P W, Giuliani MJ, Martinez AJ: Necrotizing angiopathy presenting with multifocal conduction blocks. Neurology 1991;41:442-444.
122. McCluskey L, Feinberg D, Cantor C, Bird S: 'Pseudoconduction block' in vasculitic neuropathy. Muscle Nerve 1999;22:1361-1366.
123. Briemberg HR, Levin K, Amato AA: Multifocal conduction block in peripheral nerve vasculitis. J Clin Neuromusc Dis 2002;3:153-158.
124. Moss JP, Meckler RJ, Moss WE: Consistent, effective technique for muscle and nerve biopsy. Am J Surg 1979;138:736-737.
125. Amrami KK, Felmlee JP, Spinner RJ: MRI of peripheral nerves. Neurosurg Clin N Am 2008;19:559-572, vi.
126. Neundorfer B, Grahmann F, Engelhardt A, Harte U: Postoperative effects and value of sural nerve biopsies: a retrospective study. Eur Neurol 1990;30:350-352.
127. Flachenecker P, Janka M, Goldbrunner R, Toyka KV: Clinical outcome of sural nerve biopsy: a retrospective study. J Neurol 1999;246:93-96.
128. Kumar S, Jacob J: Variability in the extent of sensory deficit after sural nerve biopsy. Neurol India 2004; 52:436-438.
129. Hilton DA, Jacob J, Househam L, Tengah C: Complications following sural and peroneal nerve biopsies. J Neurol Neurosurg Psychiatry 2007;78: 1271-1272.
130. Perry JR, Bril V: Complications of sural nerve biopsy in diabetic versus non-diabetic patients. Can J Neurol Sci 1994;21:34-37.
131. Rappaport WD, Valente J, Hunter GC, Rance NE, Lick S, Lewis T, Neal D: Clinical utilization and complications of sural nerve biopsy. Am J Surg 1993;166:252-256.
132. Gabriel CM, Howard R, Kinsella N, Lucas S, McColl I, Saldanha G, Hall SM, Hughes RA: Prospective study of the usefulness of sural nerve biopsy. J Neurol Neurosurg Psychiatry 2000;69:442-446.
133. Ruth A, Schulmeyer FJ, Roesch M, Woertgen C, Brawanski A: Diagnostic and therapeutic value due to suspected diagnosis, long-term complications, and indication for sural nerve biopsy. Clin Neurol Neurosurg 2005;107:214-217.
134. Theriault M, Dort J, Sutherland G, Zochodne DW: A prospective quantitative study of sensory deficits after whole sural nerve biopsies in diabetic and nondiabetic patients. Surgical approach and the role of collateral sprouting. Neurology 1998;50:480-484.
135. Deprez M, de Groote CC, Gollogly L, Reznik M, Martin JJ: Clinical and neuropathological parameters affecting the diagnostic yield of nerve biopsy. Neuromuscul Disord 2000;10:92-98.
136. Lindenlaub T, Sommer C: Cytokines in sural nerve biopsies from inflammatory and non-inflammatory neuropathies. Acta Neuropathol 2003;105:593-602.
137. Terrier B, Lacroix C, Guillevin L, Hatron PY, Dhote R, Maillot F, Diot E, Sarrot-Reynauld F, Sordet C, Dubourg O, Meyer L, Mariette X, Gottenberg JE: Diagnostic and prognostic relevance of neuromuscular biopsy in primary Sjogren's syndrome-related neuropathy. Arthritis Care Res 2007;57:1520-1529.
138. Vrancken AFJE, Notermans NC, Jansen GH, Wokke JHJ, Said G: Progressive idiopathic axonal neuropathy - A comparative clinical and histopathological study with vasculitic neuropathy. J Neurol 2004;251: 269-278.
139. Chalk CH, Homburger HA, Dyck PJ: Anti-neutrophil cytoplasmic antibodies in vasculitis peripheral neuropathy. Neurology 1993;43:1826-1827.
140. Midroni G, Bilbao JM: Vasculitic neuropathy; in Midroni G, Bilbao JM (eds): Biopsy Diagnosis of Peripheral Neuropathy. Boston, Butterworth-Heine- mann, 1995, pp 241-262.
141. Lie JT: Biopsy diagnosis of systemic vasculitis. Baillieres Clin Rheumatol 1997;11:219-236.
142. Bosboom WM, Van den Berg LH, Mollee I, Sasker LD, Jansen J, Wokke JH, Logtenberg T: Sural nerve T-cell receptor Vbeta gene utilization in chronic inflammatory demyelinating polyneuropathy and vasculitic neuropathy. Neurology 2001;56:74-81.
143. Broglio L, Erne B, Tolnay M, Schaeren-Wiemers N, Fuhr P, Steck AJ, Renaud S: Allograft inflammatory factor-1: a pathogenetic factor for vasculitic neuropathy. Muscle Nerve 2008;38:1272-1279.
144. Kissel JT, Riethman JL, Omerza J, Rammohan K W, Mendell JR: Peripheral nerve vasculitis: immune characterization of the vascular lesions. Ann Neurol 1989;25:291-297.
145. Satoi H, Oka N, Kawasaki T, Miyamoto K, Akiguchi I, Kimura J: Mechanisms of tissue injury in vasculitic neuropathies. Neurology 1998;50:492-496.
146. Hawke SHB, Davies L, Pamphlett R, Guo Y P, Pollard JD, McLeod JG: Vasculitic neuropathy: a clinical and pathological study. Brain 1991;114:2175-2190.
147. Dyck PJB, Engelstad J, Dyck PJ: Microvasculitis; in Dyck PJ, Thomas PK (eds): Peripheral Neuropathy. Philadelphia, Elsevier Saunders, 2005, pp 2405-2414.
148. Vincent D, Dubas F, Hauw JJ, Escourolle R, Godeau P, Buge A, Lhermitte F, Castaigne P: Nerve and muscle microvasculitis: 50 cases. Rev Neurol (Paris) 1985;141:440-446.
149. Authier FJ, Bassez G, Payan C, Guillevin L, Pawlotsky JM, Degos JD, Gherardi RK, Belec L: Detection of genomic viral RNA in nerve and muscle of patients with HCV neuropathy. Neurology 2003;60:808-812.
150. Fujimura H, Lacroix C, Said G: Vulnerability of nerve fibres to ischaemia. A quantitative light and electron microscope study. Brain 1991;114:1929-1942.
151. Yamamoto M, Ito Y, Mitsuma N, Hattori N, Sobue G: Pain-related differential expression of NGF, GDNF, IL-6, and their receptors in human vasculitic neuropathies. Intern Med 2003;42:1100-1103.
152. Vital A, Vital C: Polyarteritis nodosa and peripheral neuropathy. Ultrastructural study of 13 cases. Acta Neuropathol (Berl) 1985;67:136-141.
153. Dyck PJ, Conn DL, Okazaki H: Necrotizing angiopathic neuropathy. Three-dimensional morphology of fiber degeneration related to sites of occluded vessels. Mayo Clin Proc 1972;47:461-475.
154. Said G, Lacroix C, Lozeron P, Ropert A, Plante V, Adams D: Inflammatory vasculopathy in multifocal diabetic neuropathy. Brain 2003;126:376-385.
155. Adams CWM, Buk SJA, Hughes RAC, Leibowitz S, Sinclair E: Perls' ferrocyanide test for iron in the diagnosis of vasculitic neuropathy. Neuropathol Appl Neurobiol 1989;15:433-439.
156. Schenone A, De Martini I, Tabaton M, Romagnoli P, Akkad Wattar AS, Bianchini D, Mancardi G: Direct immunofluorescence in sural nerve biopsies. Eur Neurol 1988;28:262-269.
157. Collins MP, Mendell JR, Periquet MI, Amato AA, Kissel JT: Immunostaining in vasculitic neuropathy: sensitivity and specificity of findings. Ann Neurol 1997;42:415.
158. Panegyres PK, Blumbergs PC, Leong AS, Bourne AJ: Vasculitis of peripheral nerve and skeletal muscle: clinicopathological correlation and immunopathic mechanisms. J Neurol Sci 1990;100:193-202.
159. Abgrall S, Mouthon L, Cohen P, Authier FJ, Nizou R, Ropert A, Gherardi R, Guillevin L: Localized neurological necrotizing vasculitides. Three cases with isolated mononeuritis multiplex. J Rheumatol 2001;28:631-633.
160. Gurer G, Erdem S, Kocaefe C, Ozguc M, Tan E: Expression of matrix metalloproteinases in vasculitic neuropathy. Rheumatol Int 2004;24:255-259.
161. Lee JE, Shun CT, Hsieh SC, Hsieh ST: Skin denervation in vasculitic neuropathy. Arch Neurol 2005; 62:1570-1573.
162. Russell JH, Ley TJ: Lymphocyte-mediated cytotoxicity. Annu Rev Immunol 2002;20:323-370.
163. Andersen MH, Schrama D, Thor Straten P, Becker JC: Cytotoxic T cells. J Invest Dermatol 2006;126:32-41.
164. Williams MA, Bevan MJ: Effector and memory CTL differentiation. Annu Rev Immunol 2007;25:171-192.
165. van de Berg PJ, van Leeuwen EM, ten Berge IJ, van Lier R: Cytotoxic human CD4(+) T cells. Curr Opin Immunol 2008;20:339-343.
166. Stinchcombe JC, Griffiths GM: Secretory mechanisms in cell-mediated cytotoxicity. Annu Rev Cell Dev Biol 2007;23:495-517.
167. Pipkin ME, Lieberman J: Delivering the kiss of death: progress on understanding how perforin works. Curr Opin Immunol 2007;19:301-308.
168. Clayberger C, Krensky AM: Granulysin. Curr Opin Immunol 2003;15:560-565.
169. Hahn S, Gehri R, Erb P: Mechanism and biological significance of CD4-mediated cytotoxicity. Immunol Rev 1995;146:57-79.
170. Engelhardt A, Lorler H, Neundorfer B: Immunohistochemical findings in vasculitic neuropathies. Acta Neurol Scand 1993;87:318-321.
171. Heuss D, Probst-Cousin S, Kayser C, Neundorfer B: Cell death in vasculitic neuropathy. Muscle Nerve 2000;23:999-1004.
172. Khalili-Shirazi A, Gregson NA, Londei M, Summers L, Hughes RA: The distribution of CD1 molecules in inflammatory neuropathy. J Neurol Sci 1998;158:154-163.
173. Scarpini E, Lisak R P, Beretta S, Velicogna M, Doronzo R, Moggio M, Jann S, Scarlato G: Quantitative assessment of class II molecules in normal and pathological nerves. Immunocytochemical studies in vivo and in tissue culture. Brain 1990;113:659-675.
174. Panegyres PK, Faull RJ, Russ GR, Appleton SL, Wangel AG, Blumbergs PC: Endothelial cell activation in vasculitis of peripheral nerve and skeletal muscle. J Neurol Neurosurg Psychiatry 1992;55:4-7.
175. Van Rhijn I, Van den Berg LH, Bosboom WM, Otten HG, Logtenberg T: Expression of accessory molecules for T-cell activation in peripheral nerve of patients with CIDP and vasculitic neuropathy. Brain 2000;123:2020-2029.
176. Hu W, Janke A, Ortler S, Hartung HP, Leder C, Kieseier BC, Wiendl H: Expression of CD28-related costimulatory molecule and its ligand in inflammatory neuropathies. Neurology 2007;68:277-282.
177. Renaud S, Hays AP, Brannagan TH 3rd, Sander H W, Edgar M, Weimer LH, Olarte MR, Dalakas MC, Xiang Z, Danon MJ, Latov N: Gene expression profiling in chronic inflammatory demyelinating polyneuropathy. J Neuroimmunol 2005;159:203-214.
178. Tsukada N, Koh CS, Owa M, Yanagisawa N: Chronic neuropathy associated with immune complexes of hepatitis B virus. J Neurol Sci 1983;61:193-210.
179. Graham AR, Johnson PC: Direct immunofluorescence findings in peripheral nerve from patients with diabetic neuropathy. Ann Neurol 1985;17:450-454.
180. Bonetti B, Invernizzi F, Rizzuto N, Bonazzi ML, Zanusso GL, Chinaglia G, Monaco S: T-cell-mediated epineurial vasculitis and humoral-mediated microangiopathy in cryoglobulinemic neuropathy. J Neuroim-munol 1997;73:145-154.
181. Nydegger UE: Immune complex pathophysiology. Ann NY Acad Sci 2007;1109:66-83.
182. Deprez M, Lubke U, Verlaet M, Debrus S, Delvenne P, Martin JJ: Detection of cytokines in human sural nerve biopsies: an immunohistochemical and molecular study. Acta Neuropathol (Berl) 2001;101: 393-404.
183. Kawamura N, Dyck PJ, Schmeichel AM, Engelstad JK, Low PA, Dyck PJ: Inflammatory mediators in diabetic and non-diabetic lumbosacral radiculoplexus neuropathy. Acta Neuropathol 2008;115:231-239.
184. Haslbeck KM, Bierhaus A, Erwin S, Kirchner A, Nawroth P, Schlotzer U, Neundorfer B, Heuss D: Receptor for advanced glycation endproduct (RAGE)-mediated nuclear factor-kappaB activation in vasculitic neuropathy. Muscle Nerve 2004;29:853-860.
185. Saadoun D, Bieche I, Maisonobe T, Asselah T, Laurendeau I, Piette JC, Vidaud M, Cacoub P: Involvement of chemokines and type 1 cytokines in the pathogenesis of hepatitis C virus-associated mixed cryoglobulinemia vasculitis neuropathy. Arthritis Rheum 2005;52:2917-2925.
186. Hu W, Mathey E, Hartung H P, Kieseier BC: Cyclooxygenases and prostaglandins in acute inflammatory demyelination of the peripheral nerve. Neurology 2003;61:1774-1779.
187. Saadoun D, Bieche I, Authier FJ, Laurendeau I, Jambou F, Piette JC, Vidaud M, Maisonobe T, Cacoub P: Role of matrix metalloproteinases, proin-flammatory cytokines, and oxidative stress-derived molecules in hepatitis C virus-associated mixed cryoglobulinemia vasculitis neuropathy. Arthritis Rheum 2007;56:1315-1324.
188. Leppert D, Hughes P, Huber S, Erne B, Grygar C, Said G, Miller KM, Steck AJ, Probst A, Fuhr P: Matrix metalloproteinase upregulation in chronic inflammatory demyelinating polyneuropathy and nonsystemic vasculitic neuropathy. Neurology 1999; 53:62-70.
189. Renaud S, Erne B, Fuhr P, Said G, Lacroix C, Steck AJ, Leppert D: Matrix metalloproteinases-9 and -2 in secondary vasculitic neuropathies. Acta Neuropathol (Berl) 2003;105:37-42.
190. Mazzeo A, Aguennouz M, Messina C, Vita G: Immunolocalization and activation of transcription factor nuclear factor kappa B in dysimmune neuropathies and familial amyloidotic polyneuropathy. Arch Neurol 2004;61:1097-1102.
191. Coll-Vinent B, Cebrian M, Cid MC, Font C, Esparza J, Juan M, Yague J, Urbano-Marquez A, Grau JM: Dynamic pattern of endothelial cell adhesion molecule expression in muscle and perineural vessels from patients with classic polyarteritis nodosa. Arthritis Rheum 1998;41:435-444.
192. Oka N, Kawasaki T, Mizutani K, Sugiyama H, Akiguchi I: Hypoxia-inducible factor 1alpha may be a marker for vasculitic neuropathy. Neuropathology 2007;27:509-515.
193. Baggenstoss AH, Shick RM, Polley HF: The effect of cortisone on the lesions of periarteritis nodosa. Am J Pathol 1951;27:537-559.
194. Hollander D, Manning RT: The use of alkylating agents in the treatment of Wegener's granulomatosis. Ann Intern Med 1967;67:393-398.
195. Chumbley LC, Harrison EG Jr, DeRemee RA: Allergic granulomatosis and angiitis (Churg-Strauss syndrome). Report and analysis of 30 cases. Mayo Clin Proc 1977;52:477-484.
196. Cohen RD, Conn DL, Ilstrup DM: Clinical features, prognosis, and response to treatment in polyarteritis. Mayo Clin Proc 1980;55:146-155.
197. Treatment of polyarteritis nodosa with cortisone: results after one year; report to the Medical Research Council by the collagen diseases and hypersensitivity panel. Br Med J 1957;1:608-611.
198. Treatment of polyarteritis nodosa with cortisone: results after three years. Br Med J 1960;1:1399-1400.
199. Frohnert P P, Sheps SG: Long-term follow-up study of periarteritis nodosa. Am J Med 1967;43:8-14.
200. Leib ES, Restivo C, Paulus HE: Immunosuppressive and corticosteroid therapy of polyarteritis nodosa. Am J Med 1979;67:941-947.
201. Fahey JL, Leonard E, Churg J, Godman G: Wegener's granulomatosis. Am J Med 1954;17:168-179.
202. Froud PJ, Henderson AH: The treatment of Wegener's granulomatosis with immunosuppressive-cytotoxic drugs. J Laryngol Otol 1971;85:703-710.
203. Fauci AS, Haynes BF, Katz P, Wolff SM: Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med 1983;98:76-85.
204. Reza MJ, Dornfeld L, Goldberg LS, Bluestone R, Pearson CM: Wegener's granulomatosis. Long-term follow-up of patients treated with cyclophosphamide. Arthritis Rheum 1975;18:501-506.
205. McDonald TJ, DeRemee RA: Wegener's granulomatosis. Laryngoscope 1983;93:220-231.
206. Bligny D, Mahr A, Toumelin PL, Mouthon L, Guillevin L: Predicting mortality in systemic Wegener's granulomatosis: a survival analysis based on 93 patients. Arthritis Rheum 2004;51:83-91.
207. Guillevin L, Jarrousse B, Lok C, Lhote F, Jais J P, Le Thi Huong Du D, Bussel A: Long-term follow-up after treatment of polyarteritis nodosa and Churg-Strauss angiitis with comparison of steroids, plasma exchange and cyclophosphamide to steroids and plasma exchange. A prospective randomized trial of 71 patients. The Cooperative Study Group for Polyarteritis Nodosa. J Rheumatol 1991;18:567-574.
208. Guillevin L, Le Thi Huong Du, Godeau P, Jais P, Wechsler B: Clinical findings and prognosis of polyarteritis nodosa and Churg-Strauss angiitis: a study in 165 patients. Br J Rheumatol 1988;27:258-264.
209. Gayraud M, Guillevin L, le Toumelin P, Cohen P, Lhote F, Casassus P, Jarrousse B, French Vasculitis Study Group: Long-term follow-up of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: analysis of four prospective trials including 278 patients. Arthritis Rheum 2001;44: 666-675.
210. Guillevin L, Durand-Gasselin B, Cevallos R, Gayraud M, Lhote F, Callard P, Amouroux J, Casassus P, Jarrousse B: Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients. Arthritis Rheum 1999;42:421-430.
211. Hogan SL, Nachman PH, Wilkman AS, Jennette JC, Falk RJ: Prognostic markers in patients with antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis. J Am Soc Nephrol 1996;7:23-32.
212. Nachman PH, Hogan SL, Jennette JC, Falk RJ: Treatment response and relapse in antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis. J Am Soc Nephrol 1996;7:33-39.
213. Hogan SL, Falk RJ, Chin H, Cai J, Jennette CE, Jennette JC, Nachman PH: Predictors of relapse and treatment resistance in antineutrophil cytoplasmic antibody-associated small-vessel vasculitis. Ann Intern Med 2005;143:621-631.
214. Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, Rottem M, Fauci AS: Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 1992;116:488-498.
215. Pedersen-Bjergaard J, Ersboll J, Hansen VL, Sorensen BL, Christoffersen K, Hou-Jensen K, Nissen NI, Knudsen JB, Hansen MM: Carcinoma of the urinary bladder after treatment with cyclophosphamide for non-Hodgkin's lymphoma. N Engl J Med 1988;318:1028-1032.
216. Radis CD, Kahl LE, Baker GL, Wasko MC, Cash JM, Gallatin A, Stolzer BL, Agarwal AK, Medsger TA Jr, Kwoh CK: Effects of cyclophosphamide on the development of malignancy and on long-term survival of patients with rheumatoid arthritis. A 20-year followup study. Arthritis Rheum 1995;38:1120-1127.
217. Travis LB, Curtis RE, Glimelius B, Holowaty EJ, Van Leeuwen FE, Lynch CF, Hagenbeek A, Stovall M, Banks PM, Adami J, et al: Bladder and kidney cancer following cyclophosphamide therapy for non-Hodgkin's lymphoma. J Natl Cancer Inst 1995;87: 524-530.
218. Guillevin L, Cordier JF, Lhote F, Cohen P, Jarrousse B, Royer I, Lesavre P, Jacquot C, Bindi P, Bielefeld P, Desson JF, Detree F, Dubois A, Hachulla E, Hoen B, Jacomy D, Seigneuric C, Lauque D, Stern M, LongyBoursier M: 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.
219. Westman K W, Bygren PG, Olsson H, Ranstam J, Wieslander J: Relapse rate, renal survival, and cancer morbidity in patients with Wegener's granulomatosis or microscopic polyangiitis with renal involvement. J Am Soc Nephrol 1998;9:842-852.
220. Knight A, Askling J, Granath F, Sparen P, Ekbom A: Urinary bladder cancer in Wegener's granulomatosis: risks and relation to cyclophosphamide. Ann Rheum Dis 2004;63:1307-1311.
221. Faurschou M, Sorensen IJ, Mellemkjaer L, Loft AG, Thomsen BS, Tvede N, Baslund B: Malignancies in Wegener's granulomatosis: incidence and relation to cyclophosphamide therapy in a cohort of 293 patients. J Rheumatol 2008;35:100-105.
222. Williams DG: Renal vasculitis in microscopic polyarteritis nodosa. J R Soc Med 1979;72:729-733.
223. Sneller MC, Hoffman GS, Talar-Williams C, Kerr GS, Hallahan CW, Fauci AS: An analysis of forty-two Wegener's granulomatosis patients treated with methotrexate and prednisone. Arthritis Rheum 1995;38:608-613.
224. de Groot K, Muhler M, Reinhold-Keller E, Paulsen J, Gross WL: Induction of remission in Wegener's granulomatosis with low dose methotrexate. J Rheumatol 1998;25:492-495.
225. Stone JH, Tun W, Hellman DB: 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.
226. Villa-Forte A, Clark TM, Gomes M, Carey J, Mascha E, Karafa MT, Roberson G, Langford CA, Hoffman GS: Substitution of methotrexate for cyclophosphamide in Wegener granulomatosis: a 12-year single-practice experience. Medicine (Baltimore) 2007;86: 269-277.
227. de Groot K, Rasmussen N, Bacon PA, Tervaert JWC, Feighery C, Gregorini G, Gross WL, Luqmani R, Jayne DRW: Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum 2005;52:2461-2469.
228. Hu W, Liu C, Xie H, Chen H, Liu Z, Li L: Mycophenolate mofetil versus cyclophosphamide for inducing remission of ANCA vasculitis with moderate renal involvement. Nephrol Dial Transplant 2008; 23:1307-1312.
229. Adu D, Pall A, Luqmani RA, Richards NT, Howie AJ, Emery P, Michael J, Savage CO, Bacon PA: Controlled trial of pulse versus continuous prednisolone and cyclophosphamide in the treatment of systemic vasculitis. Q J Med 1997;90:401-409.
230. Gayraud M, Guillevin L, Cohen P, Lhote F, Cacoub P, Deblois P, Godeau B, Ruel M, Vidal E, Piontud M, Ducroix J P, Lassoued S, Christoforov B, Babinet P: 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.
231. Haubitz M, Schellong S, Gobel U, Schurek HJ, Schaumann D, Koch KM, Brunkhorst R: 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.
232. de Groot K, Jayne DR, Tesar V, Savage CO: Randomised controlled trial of daily oral versus pulse cyclophosphamide for induction of remission in ANCA-associated systemic vasculitis. Kidney Blood Press Res 2005;2005:195.
233. Pusey CD, Gaskin G, Rees AJ: Treatment of primary systemic vasculitis. APMIS Suppl 1990;19:48-50.
234. Jayne D, Rasmussen N, Andrassy K, Bacon P, Tervaert JWC, Dadoniene J, Ekstrand A, Gaskin G, Gregorini G, de Groot K, Gross W, Hagen EC, Mirapeix E, Pettersson E, Siegert C, Sinico A, Tesar V, Westman K, Pusey C, European Vasculitis Study Group: A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med 2003;349:36-44.
235. Slot MC, Tervaert JWC, Boomsma MM, Stegeman CA: Positive classic antineutrophil cytoplasmic antibody (C-ANCA) titer at switch to azathioprine therapy associated with relapse in proteinase 3-related vasculitis. Arthritis Rheum 2004;51:269-273.
236. Pagnoux C, Mahr A, Hamidou MA, Boffa J-J, Ruivard M, Ducroix J-P, Kyndt X, Lifermann F, Papo T, Lambert M, Le Loach J, Khellaf M, Merrien D, Puechal X, Vinzio S, Cohen P, Mouthon L, Cordier J-F, Guillevin L: Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. N Engl J Med 2008;359:2790-2803.
237. Metzler C, Miehle N, Manger K, Iking-Konert C, de Groot K, Hellmich B, Gross WL, Reinhold-Keller E, German Network of Rheumatic Diseases: Elevated relapse rate under oral methotrexate versus leflunomide for maintenance of remission in Wegener's granulomatosis. Rheumatology 2007;46:1087-1091.
238. Guillevin L, Fain O, Lhote F, Jarrousse B, Le Thi Huong D, Bussel A, Leon A: Lack of superiority of steroids plus plasma exchange to steroids alone in the treatment of polyarteritis nodosa and Churg-Strauss syndrome. A prospective, randomized trial in 78 patients. Arthritis Rheum 1992;35:208-215.
239. Guillevin L, Lhote F, Cohen P, Jarrousse B, Lortholary O, Genereau T, Leon A, Bussel A: Corticosteroids plus pulse cyclophosphamide and plasma exchanges versus corticosteroids plus pulse cyclophosphamide alone in the treatment of polyarteritis nodosa and Churg-Strauss syndrome patients with factors predicting poor prognosis. A prospective, randomized trial in sixty-two patients. Arthritis Rheum 1995;38:1638-1645.
240. Pusey CD, Rees AJ, Evans DJ, Peters DK, Lockwood CM: Plasma exchange in focal necrotizing glomerulonephritis without anti-GBM antibodies. Kidney Int 1991;40:757-763.
241. Jayne DRW, Gaskin G, Rasmussen N, Abramowicz D, Ferrario F, Guillevin L, Mirapeix E, Savage COS, Sinico RA, Stegeman CA, Westman K W, van der Woude FJ, de Lind van Wijngaarden RAF, Pusey CD, European Vasculitis Study Group: Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis. J Am Soc Nephrol 2007;18:2180-2188.
242. Jayne DR, Chapel H, Adu D, Misbah S, O'Donoghue D, Scott D, Lockwood CM: Intravenous immunoglobulin for ANCA-associated systemic vasculitis with persistent disease activity. Q J Med 2000;93:433-439.
243. Jayne DR, Lockwood CM: Pooled intravenous immunoglobulin in the management of systemic vasculitis. Adv Exp Med Biol 1993;336:469-472.
244. Martinez V, Cohen P, Pagnoux C, Vinzio S, Mahr A, Mouthon L, Sailler L, Delaunay C, Sadoun A, Guillevin L: Intravenous immunoglobulins for relapses of systemic vasculitides associated with antineutrophil cytoplasmic autoantibodies: results of a multicenter, prospective, open-label study of twenty-two patients. Arthritis Rheum 2008;58:308-317.
245. Joy MS, Hogan SL, Jennette JC, Falk RJ, Nachman PH: A pilot study using mycophenolate mofetil in relapsing or resistant ANCA small vessel vasculitis. Nephrol Dial Transplant 2005;20:2725-2732.
246. Koukoulaki M, Jayne DR: Mycophenolate mofetil in anti-neutrophil cytoplasm antibodies-associated systemic vasculitis. Nephron Clin Pract 2006;102: c100-c107.
247. Stassen PM, Cohen Tervaert J W, Stegeman CA: Induction of remission in active anti-neutrophil cytoplasmic antibody-associated vasculitis with mycophenolate mofetil in patients who cannot be treated with cyclophosphamide. Ann Rheum Dis 2007;66:798-802.
248. Lovric S, Erdbruegger U, Kumpers P, Woywodt A, Koenecke C, Wedemeyer H, Haller H, Haubitz M: Rituximab as rescue therapy in anti-neutrophil cytoplasmic antibody-associated vasculitis: a single-centre experience with 15 patients. Nephrol Dial Transplant 2009;24:179-185.
249. Keogh KA, Wylam ME, Stone JH, Specks U: Induction of remission by B lymphocyte depletion in eleven patients with refractory antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum 2005;52:262-268.
250. Keogh KA, Ytterberg SR, Fervenza FC, Carlson KA, Schroeder DR, Specks U: Rituximab for refractory Wegener's granulomatosis: report of a prospective, open-label pilot trial. Am J Respir Crit Care Med 2006;173:180-187.
251. Stasi R, Stipa E, Del Poeta G, Amadori S, Newland AC, Provan D: Long-term observation of patients with anti-neutrophil cytoplasmic antibody-associated vasculitis treated with rituximab. Rheumatology (Oxford) 2006;45:1432-1436.
252. Smith KG, Jones RB, Burns SM, Jayne DR: Long-term comparison of rituximab treatment for refractory systemic lupus erythematosus and vasculitis: remission, relapse, and re-treatment. Arthritis Rheum 2006;54:2970-2982.
253. Booth A, Harper L, Hammad T, Bacon P, Griffith M, Levy J, Savage C, Pusey C, Jayne D: Prospective study of TNFalpha blockade with infliximab in anti-neutrophil cytoplasmic antibody-associated systemic vasculitis. J Am Soc Nephrol 2004;15:717-721.
254. Josselin L, Mahr A, Cohen P, Pagnoux C, Guaydier-Souquieres G, Hayem G, Job-Deslandre C, Liferman F, Pourrat J, Guillevin L: Infliximab efficacy and safety against refractory systemic necrotising vasculitides: long-term follow-up of 15 patients. Ann Rheum Dis 2008;67:1343-1346.
255. Walsh M, Chaudhry A, Jayne D: Long-term follow-up of relapsing/refractory anti-neutrophil cytoplasm antibody associated vasculitis treated with the lymphocyte depleting antibody alemtuzumab (CAMPATH-1H). Ann Rheum Dis 2008;67:1322-1327.
256. Birck R, Warnatz K, Lorenz HM, Choi M, Haubitz M, Grunke M, Peter HH, Kalden JR, Gobel U, Drexler JM, Hotta O, Nowack R, Van Der Woude FJ: 15-Deoxyspergualin in patients with refractory ANCA-associated systemic vasculitis: a six-month open-label trial to evaluate safety and efficacy. J Am Soc Nephrol 2003;14:440-447.
257. Schmitt WH, Hagen EC, Neumann I, Nowack R, Flores-Suarez LF, van der Woude FJ: Treatment of refractory Wegener's granulomatosis with antithy-mocyte globulin (ATG): an open study in 15 patients. Kidney Int 2004;65:1440-1448.
258. Flossmann O, Baslund B, Bruchfeld A, Cohen Tervaert J W, Hall C, Heinzel P, Hellmich B, Luqmani RA, Nemoto K, Tesar V, Jayne DR: Deoxyspergualin in relapsing and refractory Wegener's granulomatosis. Ann Rheum Dis 2008, E-pub ahead of print.
259. Bosch X, Guilabert A, Espinosa G, Mirapeix E: Treatment of antineutrophil cytoplasmic antibody associated vasculitis: a systematic review. JAMA 2007;298:655-669.
260. Lapraik C, Watts R, Bacon P, Carruthers D, Chakravarty K, D'Cruz D, Guillevin L, Harper L, Jayne D, Luqmani R, Mooney J, Scott D: BSR and BHPR guidelines for the management of adults with ANCA associated vasculitis. Rheumatology (Oxford) 2007;46:1615-1616.
261. Mukhtyar C, Guillevin L, Cid MC, Dasgupta B, de Groot K, Gross W, Hauser T, Hellmich B, Jayne D, Kallenberg CG, Merkel PA, Raspe H, Salvarani C, Scott DG, Stegeman C, Watts R, Westman K, Witter J, Yazici H, Luqmani R: EULAR Recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis 2009;68:310-317.
262. Guillevin L: Advances in the treatments of systemic vasculitides. Clin Rev Allergy Immunol 2008;35:72-78.
263. Klemmer PJ, Chalermskulrat W, Reif MS, Hogan SL, Henke DC, Falk RJ: Plasmapheresis therapy for diffuse alveolar hemorrhage in patients with small-vessel vasculitis. Am J Kidney Dis 2003;42:1149-1153.
264. Falk RJ, Hoffman GS: Controversies in small vessel vasculitis - Comparing the rheumatology and nephrology views. Curr Opin Rheumatol 2007;19:1-9.
265. Danieli MG, Cappelli M, Malcangi G, Logullo F, Salvi A, Danieli G: Long-term effectiveness of intravenous immunoglobulin in Churg-Strauss syndrome. Ann Rheum Dis 2004;63:1649-1654.
266. Puechal X, Said G: Necrotizing vasculitis of the peripheral nervous system: nonsystemic or clinically undetectable? Arthritis Rheum 1999;42:824-825.
267. Luqmani RA, Flossmann O: Outcome in small-vessel systemic vasculitis. J Rheumatol 2006;33:1224-1227.