MNM is a relatively uncommon clinical syndrome. Overall incidence and prevalence rates for MNM are not known. Vasculitic neuropathies (the most common etiology for MNM) occur most frequently in older people, although they may present at any age. The mean age of onset for vasculitic neuropathy ranges from 51-67 years.[3]Said G, Lacroix-Ciaudo C, Fujimura H, et al. The peripheral neuropathy of necrotizing arteritis: a clinicopathological study. Ann Neurol. 1988 May;23(5):461-5.
http://www.ncbi.nlm.nih.gov/pubmed/2839104?tool=bestpractice.com
[4]Hawke SH, Davies L, Pamphlett R, et al. Vasculitic neuropathy: a clinical and pathological study. Brain. 1991 Oct;114 ( Pt 5):2175-90.
http://www.ncbi.nlm.nih.gov/pubmed/1657271?tool=bestpractice.com
[5]Mathew L, Talbot K, Love S, et al. Treatment of vasculitic peripheral neuropathy: a retrospective analysis of outcome. QJM. 2007 Jan;100(1):41-51.
http://www.ncbi.nlm.nih.gov/pubmed/17189246?tool=bestpractice.com
[6]Graf J, Imboden J. Vasculitis and peripheral neuropathy. Curr Opin Rheumatol. 2019 Jan;31(1):40-5.
http://www.ncbi.nlm.nih.gov/pubmed/30461543?tool=bestpractice.com
[7]Beachy N, Satkowiak K, Gwathmey KG. Vasculitic neuropathies. Semin Neurol. 2019 Oct;39(5):608-19.
http://www.ncbi.nlm.nih.gov/pubmed/31639844?tool=bestpractice.com
MNM has been reported to be associated with several conditions.
MNM was reported in 65% of patients with nonsystemic vasculitic neuropathy, the most common type of vasculitis affecting the peripheral nerves.[8]Langford CA. Vasculitis. J Allergy Clin Immunol. 2003 Feb;111(2 Suppl):S602-12.
http://www.ncbi.nlm.nih.gov/pubmed/12592306?tool=bestpractice.com
[9]Kamesh L, Harper L, Savage CO. ANCA-positive vasculitis. J Am Soc Nephrol. 2002 Jul;13(7):1953-60.
http://jasn.asnjournals.org/content/13/7/1953.full
http://www.ncbi.nlm.nih.gov/pubmed/12089393?tool=bestpractice.com
[10]Dyck PJ, Benstead TJ, Conn, DL, et al. Nonsystemic vasculitic neuropathy. Brain. 1987 Aug;110 ( Pt 4):843-53.
http://www.ncbi.nlm.nih.gov/pubmed/3651797?tool=bestpractice.com
Polyarteritis nodosa targets peripheral nerves more often than other organ systems. Up to 85% of patients with polyarteritis nodosa demonstrate vasculitic neuropathy.[7]Beachy N, Satkowiak K, Gwathmey KG. Vasculitic neuropathies. Semin Neurol. 2019 Oct;39(5):608-19.
http://www.ncbi.nlm.nih.gov/pubmed/31639844?tool=bestpractice.com
[11]Stone JH. Polyarteritis nodosa. JAMA. 2002 Oct 2;288(13):1632-9.
http://www.ncbi.nlm.nih.gov/pubmed/12350194?tool=bestpractice.com
Peripheral neuropathy develops in around 50% to 70% patients with eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome) or microscopic polyarteritis.[7]Beachy N, Satkowiak K, Gwathmey KG. Vasculitic neuropathies. Semin Neurol. 2019 Oct;39(5):608-19.
http://www.ncbi.nlm.nih.gov/pubmed/31639844?tool=bestpractice.com
[12]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 Oct;78(10):1119-23.
http://www.ncbi.nlm.nih.gov/pubmed/17299018?tool=bestpractice.com
MNM was reported in about 15% to 20% of patients with granulomatosis with polyangiitis (formerly known as Wegener granulomatosis).[7]Beachy N, Satkowiak K, Gwathmey KG. Vasculitic neuropathies. Semin Neurol. 2019 Oct;39(5):608-19.
http://www.ncbi.nlm.nih.gov/pubmed/31639844?tool=bestpractice.com
[13]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 Jan;33(1):4-9.
http://www.ncbi.nlm.nih.gov/pubmed/8388187?tool=bestpractice.com
MNM was reported in approximately 8% of untreated patients referred for treatment of hepatitis C (but this is probably an overestimate).[11]Stone JH. Polyarteritis nodosa. JAMA. 2002 Oct 2;288(13):1632-9.
http://www.ncbi.nlm.nih.gov/pubmed/12350194?tool=bestpractice.com
Up to 65% of patients with hepatitis C-related cryoglobulinemic vasculitis develop clinically significant neuropathy.[7]Beachy N, Satkowiak K, Gwathmey KG. Vasculitic neuropathies. Semin Neurol. 2019 Oct;39(5):608-19.
http://www.ncbi.nlm.nih.gov/pubmed/31639844?tool=bestpractice.com
MNM is present in 30% to 50% of patients with rheumatoid vasculitis, which affects 5% to 20% of patients with rheumatoid arthritis.[7]Beachy N, Satkowiak K, Gwathmey KG. Vasculitic neuropathies. Semin Neurol. 2019 Oct;39(5):608-19.
http://www.ncbi.nlm.nih.gov/pubmed/31639844?tool=bestpractice.com
[14]Genta MS, Genta RM, Gabay C. Systemic rheumatoid vasculitis: a review. Semin Arthritis Rheum. 2006 Oct;36(2):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 Nov;65(6):365-75.
http://www.ncbi.nlm.nih.gov/pubmed/3784899?tool=bestpractice.com
[16]Burns TM. Vasculitic neuropathy. Continuum. 2003;9:146-59. Rheumatoid vasculitis usually occurs late in the course of seropositive rheumatoid arthritis, and its incidence has declined in recent past with effective disease-modifying therapies.
Approximately 8% to 13% of patients with Sjogren syndrome have MNM.[7]Beachy N, Satkowiak K, Gwathmey KG. Vasculitic neuropathies. Semin Neurol. 2019 Oct;39(5):608-19.
http://www.ncbi.nlm.nih.gov/pubmed/31639844?tool=bestpractice.com
[17]Delalande S, de Seze J, Fauchais AL, et al. Neurologic manifestations in primary Sjögren syndrome: a study of 82 patients. Medicine (Baltimore). 2004 Sep;83(5):280-91.
http://www.ncbi.nlm.nih.gov/pubmed/15342972?tool=bestpractice.com
Around 1% of patients with systemic lupus erythematosus have vasculitis, and 9% of those with vasculitis have evidence of vasculitic neuropathy.[7]Beachy N, Satkowiak K, Gwathmey KG. Vasculitic neuropathies. Semin Neurol. 2019 Oct;39(5):608-19.
http://www.ncbi.nlm.nih.gov/pubmed/31639844?tool=bestpractice.com
[18]Ramos-Casals M, Nardi N, Lagrutta M, et al. Vasculitis in systemic lupus erythematosus: prevalence and clinical characteristics in 670 patients. Medicine (Baltimore). 2006 Mar;85(2):95-104.
http://www.ncbi.nlm.nih.gov/pubmed/16609348?tool=bestpractice.com
Approximately 17% of patients with neurosarcoidosis have neuropathy, but <1% of all patients with sarcoidosis develop vasculitic neuropathy.[19]Chapelon C, Ziza JM, Piette JC, et al. Neurosarcoidosis: signs, course and treatment in 35 confirmed cases. Medicine (Baltimore). 1990 Sep;69(5):261-76.
http://www.ncbi.nlm.nih.gov/pubmed/2205782?tool=bestpractice.com
A very small proportion of patients with giant cell arteritis and Henoch-Schonlein purpura have MNM.[20]Golbus J, McCune WJ. Giant cell arteritis and peripheral neuropathy: a report of 2 cases and review of the literature. J Rheumatol. 1987 Feb;14(1):129-34.
http://www.ncbi.nlm.nih.gov/pubmed/3033236?tool=bestpractice.com
[21]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 Apr;43(4):529-31.
http://www.ncbi.nlm.nih.gov/pubmed/15024142?tool=bestpractice.com
[22]Campbell SB, Hawley CM, Staples C. Mononeuritis multiplex complicating Henoch-Schonlein purpura. Aust N Z J Med. 1994 Oct;24(5):580.
http://www.ncbi.nlm.nih.gov/pubmed/7848168?tool=bestpractice.com
Vasculitic neuropathy does not occur in large-vessel arteritis (Takayasu arteritis).