多发性单神经炎 (MNM) 是一种少见临床综合征。MNM 的总发病率和患病率不详。血管炎性神经病(MNM 的最常见病因)最常见发患者群为老年人,但可能在各年龄段出现。报道的血管炎性神经病平均发病年龄为 51~67 岁。[3]Chia L, Fernandez A, Lacroix C, et al. Contribution of nerve biopsy findings to the diagnosis of disabling neuropathy in the elderly: a retrospective review of 100 consecutive patients. Brain. 1996;119:1091-1098.http://brain.oxfordjournals.org/content/brain/119/4/1091.full.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/8813273?tool=bestpractice.com[4]Said G, Lacroix-Ciaudo C, Fujimura H, et al. The peripheral neuropathy of necrotizing arteritis: a clinicopathological study. Ann Neurol. 1988;23:461-465.http://www.ncbi.nlm.nih.gov/pubmed/2839104?tool=bestpractice.com[5]Hawke SH, Davies L, Pamphlett R, et al. Vasculitic neuropathy: a clinical and pathological study. Brain. 1991;114:2175-2190.http://www.ncbi.nlm.nih.gov/pubmed/1657271?tool=bestpractice.com
65% 的非系统性血管炎性神经病患者,非系统性血管炎性神经病是影响周围神经的最常见血管炎类型[6]Langford CA. Vasculitis. J Allergy Clin Immunol. 2003;111(2 Suppl):S602-S612.http://www.ncbi.nlm.nih.gov/pubmed/12592306?tool=bestpractice.com[7]Kamesh L, Harper L, Savage CO. ANCA-positive vasculitis. J Am Soc Nephrol. 2002;13:1953-1960.http://jasn.asnjournals.org/content/13/7/1953.fullhttp://www.ncbi.nlm.nih.gov/pubmed/12089393?tool=bestpractice.com[8]Dyck PJ, Benstead TJ, Conn, DL, et al. Nonsystemic vasculitic neuropathy. Brain. 1987;110:843-853.http://www.ncbi.nlm.nih.gov/pubmed/3651797?tool=bestpractice.com
60% 的典型结节性多动脉炎患者[9]Stone JH. Polyarteritis nodosa. JAMA. 2002;288:1632-1639.http://www.ncbi.nlm.nih.gov/pubmed/12350194?tool=bestpractice.com
8% 的应治疗丙型肝炎但未治疗的患者(但该比例很可能被高估)[9]Stone JH. Polyarteritis nodosa. JAMA. 2002;288:1632-1639.http://www.ncbi.nlm.nih.gov/pubmed/12350194?tool=bestpractice.com
25% 有 Churg-Strauss 综合征或显微镜下多动脉炎的患者[10]Cattaneo L, Chierici E, Pavone L, et al. Peripheral neuropathy in Wegener's granulomatosis, Churg-Strauss syndrome and microscopic polyangiitis. J Neurol Neurosurg Psychiatr. 2007;78:1119-1123.http://www.ncbi.nlm.nih.gov/pubmed/17299018?tool=bestpractice.com
13% 的肉芽肿性血管炎(韦格纳)患者[11]Nishino H, Rubino FA, DeRemee RA, et al. Neurological involvement in Wegener's granulomatosis: an analysis of 324 consecutive patients at the Mayo Clinic. Ann Neurol. 1993;33:4-9.http://www.ncbi.nlm.nih.gov/pubmed/8388187?tool=bestpractice.com
8% 的 Sjogren 综合征患者[12]Delalande S, de Seze J, Fauchais AL, et al. Neurologic manifestations in primary Sjögren syndrome: a study of 82 patients. Medicine (Baltimore). 2004;83:280-291.http://www.ncbi.nlm.nih.gov/pubmed/15342972?tool=bestpractice.com
1% 的 SLE 患者和 9% 有狼疮相关血管炎迹象的患者[13]Ramos-Casals M, Nardi N, Lagrutta M, et al. Vasculitis in systemic lupus erythematosus: prevalence and clinical characteristics in 670 patients. Medicine (Baltimore). 2006;85:95-104.http://www.ncbi.nlm.nih.gov/pubmed/16609348?tool=bestpractice.com
40% 至 50% 类风湿性血管炎患者,[14]Genta MS, Genta RM, Gabay C. Systemic rheumatoid vasculitis: a review. Semin Arthritis Rheum. 2006;36:88-98.http://www.ncbi.nlm.nih.gov/pubmed/17023257?tool=bestpractice.com[15]Vollertsen RS, Conn DL, Ballard DJ, et al. Rheumatoid vasculitis: survival and associated risk factors. Medicine (Baltimore). 1986;65:365-375.http://www.ncbi.nlm.nih.gov/pubmed/3784899?tool=bestpractice.com 5% 至 20% 的类风湿性关节炎患者[1]Collins MP, Kissel JT. Neuropathies with systemic vasculitis. In: Dyck PJ, Thomas PK, eds. Peripheral neuropathy. 4th ed. Vol. 2. Philadelphia, PA: Elsevier Saunders; 2005:2335-2404.[16]Burns TM. Vasculitic neuropathy. Continuum. 2003;9:146-159.
17% 的神经系统结节病患者,但不到 1% 的结节病患者[17]Chapelon C, Ziza JM, Piette JC, et al. Neurosarcoidosis: signs, course and treatment in 35 confirmed cases. Medicine (Baltimore). 1990;69:261-276.http://www.ncbi.nlm.nih.gov/pubmed/2205782?tool=bestpractice.com
非常小的比例的巨细胞动脉炎和过敏性紫癜患者。[1]Collins MP, Kissel JT. Neuropathies with systemic vasculitis. In: Dyck PJ, Thomas PK, eds. Peripheral neuropathy. 4th ed. Vol. 2. Philadelphia, PA: Elsevier Saunders; 2005:2335-2404.[18]Golbus J, McCune WJ. Giant cell arteritis and peripheral neuropathy: a report of 2 cases and review of the literature. J Rheumatol. 1987;14:129-134.http://www.ncbi.nlm.nih.gov/pubmed/3033236?tool=bestpractice.com[19]Chan WM, Liu DT, Chan AY, et al. ANCA-associated giant cell arteritis presenting with mononeuritis multiplex and central retinal artery occlusion: a case report. Rheumatology (Oxford). 2004;43:529-531.http://www.ncbi.nlm.nih.gov/pubmed/15024142?tool=bestpractice.com[20]Campbell SB, Hawley CM, Staples C. Mononeuritis multiplex complicating Henoch-Schonlein purpura. Aust N Z J Med. 1994;24:580.http://www.ncbi.nlm.nih.gov/pubmed/7848168?tool=bestpractice.com