Advertisement

Vasculitic Neuropathies

  • Elie Naddaf
  • P. James Bonham Dyck
Neuromuscular Disorders (SA Rudnicki, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Neuromuscular Disorders

Opinion statement

From pathological standpoint, we divide vasculitic neuropathies in two categories: nerve large arteriole vasculitides and nerve microvasculitis. It is also important to determine whether a large arteriole vasculitis has an infectious etiology as it entails different treatment approach. Treatment of non-infectious large arteriole vasculitides consists initially of induction therapy with corticosteroids. Adding an immunosuppressant, mainly cyclophosphamide, is often needed. Treatment of infectious large arteriole vasculitides needs a multidisciplinary approach to target both the underlying infection and the vasculitis. Corticosteroids are the first-line therapy for classic non-systemic vasculitic neuropathy. Stable or improving patients without biopsy evidence of active vasculitis can be either observed or treated. Currently, adding an immunosuppressant is only indicated for patients who continue to progress on corticosteroids alone or patients with a rapidly progressive course. The treatment of the radiculoplexus neuropathies such as diabetic lumbosacral radiculoplexus neuropathy, lumbosacral radiculoplexus neuropathy (in non-diabetic patients), and diabetic cervical radiculoplexus neuropathy, as well as painless diabetic motor neuropathy, is not well established yet. We treat patients, if they present early on in the disease course or if they have severe disabling symptoms, with IV methylprednisolone 1 g once a week for 12 weeks.

Keywords

Vasculitic neuropathy Necrotizing vasculitis Nerve large arteriole Microvasculitis Microscopic polyangiitis Polyarteritis nodosa Churg-Strauss syndrome Wegener’s granulomatosis Rheumatoid vasculitis Sjögren’s syndrome Hepatitis C vasculitic neuropathy Cryoglobulinemia HIV-associated vasculitic neuropathy Non-systemic vasculitic neuropathy Diabetic lumbosacral radiculoplexus neuropathy Lumbosacral radiculoplexus neuropathy Diabetic cervical radiculoplexus neuropathy Painless diabetic motor neuropathy 

Notes

Acknowledgments

We thank Janean Engelstad for her assistance with the figures.

Compliance with Ethics Guidelines

Conflict of Interest

Elie Naddaf and P. James Bonham Dyck declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

References and Recommended Reading

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. 1.
    Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994;37(2):187–92.PubMedCrossRefGoogle Scholar
  2. 2.
    Saleh A, Stone JH. Classification and diagnostic criteria in systemic vasculitis. Best Pract Res Clin Rheumatol. 2005;19(2):209–21. doi: 10.1016/j.berh.2004.09.001.PubMedCrossRefGoogle Scholar
  3. 3.
    Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1–11. doi: 10.1002/art.37715.PubMedCrossRefGoogle Scholar
  4. 4.••
    Gwathmey KG, Burns TM, Collins MP, Dyck PJB. Vasculitic neuropathies. Lancet Neurol. 2014;13(1):67–82. doi: 10.1016/S1474-4422(13)70236-9. It is a review of vasculitic neuropathies that complements this treatment-focused article, as it details further the clinical and laboratory findings.PubMedCrossRefGoogle Scholar
  5. 5.
    Collins MP, Periquet MI, Mendell JR, Sahenk Z, Nagaraja HN, Kissel JT. Nonsystemic vasculitic neuropathy: insights from a clinical cohort. Neurology. 2003;61(5):623–30.PubMedCrossRefGoogle Scholar
  6. 6.
    Kurt S, Alsharabati M, Lu L, Claussen GC, Oh SJ. Asymptomatic vasculitic neuropathy. Muscle Nerve. 2014. doi: 10.1002/mus.24494.PubMedGoogle Scholar
  7. 7.
    Vrancken AFJE, Gathier CS, Cats EA, Notermans NC, Collins MP. The additional yield of combined nerve/muscle biopsy in vasculitic neuropathy. Eur J Neurol. 2011;18(1):49–58. doi: 10.1111/j.1468-1331.2010.03041.x.PubMedCrossRefGoogle Scholar
  8. 8.
    Collins MP, Periquet MI. Isolated vasculitis of the peripheral nervous system. Clin Exp Rheumatol. 26(3 Suppl 49):S118-S130.Google Scholar
  9. 9.
    Guillevin L, Lhote F, Gayraud M. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. a prospective study in 342 patients. Medicine (Baltimore). 1996;75(1):17–28.CrossRefGoogle Scholar
  10. 10.
    Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Le Toumelin P. The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine (Baltimore). 2011;90(1):19–27. doi: 10.1097/MD.0b013e318205a4c6.CrossRefGoogle Scholar
  11. 11.•
    Comarmond C, Pagnoux C, Khellaf M, et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort. Arthritis Rheum. 2013;65(1):270–81. doi: 10.1002/art.37721. It is a large cohort describing the main characteristics and the long-term outcome of patients with EGPA.PubMedCrossRefGoogle Scholar
  12. 12.
    Moosig F, Bremer JP, Hellmich B, et al. A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis and polyangiitis (Churg-Strauss, EGPA): monocentric experiences in 150 patients. Ann Rheum Dis. 2013;72(6):1011–7. doi: 10.1136/annrheumdis-2012-201531.PubMedCrossRefGoogle Scholar
  13. 13.
    Sinico RA, Di Toma L, Maggiore U, et al. Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome. Arthritis Rheum. 2005;52(9):2926–35. doi: 10.1002/art.21250.PubMedCrossRefGoogle Scholar
  14. 14.
    Healy B, Bibby S, Steele R, Weatherall M, Nelson H, Beasley R. Antineutrophil cytoplasmic autoantibodies and myeloperoxidase autoantibodies in clinical expression of Churg-Strauss syndrome. J Allergy Clin Immunol. 2013;131(2):571–6. doi: 10.1016/j.jaci.2012.05.058. e1-e6.PubMedCrossRefGoogle Scholar
  15. 15.
    Sablé-Fourtassou R, Cohen P, Mahr A, et al. Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Intern Med. 2005;143(9):632–8.PubMedCrossRefGoogle Scholar
  16. 16.
    Mahr A, Moosig F, Neumann T, et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): evolutions in classification, etiopathogenesis, assessment and management. Curr Opin Rheumatol. 2014;26(1):16–23. doi: 10.1097/BOR.0000000000000015.PubMedCrossRefGoogle Scholar
  17. 17.
    Samson M, Puéchal X, Devilliers H, et al. Long-term outcomes of 118 patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) enrolled in two prospective trials. J Autoimmun. 2013;43:60–9. doi: 10.1016/j.jaut.2013.03.003.PubMedCrossRefGoogle Scholar
  18. 18.
    Nishino H, Rubino FA, DeRemee RA, Swanson JW, Parisi JE. Neurological involvement in Wegener‘s granulomatosis: an analysis of 324 consecutive patients at the Mayo Clinic. Ann Neurol. 1993;33(1):4–9. doi: 10.1002/ana.410330103.PubMedCrossRefGoogle Scholar
  19. 19.
    Stone JH. Limited versus severe Wegener’s granulomatosis: baseline data on patients in the Wegener's granulomatosis etanercept trial. Arthritis Rheum. 2003;48(8):2299–309. doi: 10.1002/art.11075.PubMedCrossRefGoogle Scholar
  20. 20.
    Miloslavsky EM, Specks U, Merkel PA, et al. Clinical outcomes of remission induction therapy for severe antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2013;65(9):2441–9. doi: 10.1002/art.38044.PubMedCrossRefGoogle Scholar
  21. 21.
    Godman GC, Churg J. Wegener’s granulomatosis: pathology and review of the literature. AMA Arch Pathol. 1954;58(6):533–53.PubMedGoogle Scholar
  22. 22.
    Collins MP, Periquet MI. Prevalence of vasculitic neuropathy in Wegener granulomatosis. Arch Neurol. 2002;59(8):1333–4. author reply 1334.PubMedCrossRefGoogle Scholar
  23. 23.
    De Groot K, Schmidt DK, Arlt AC, Gross WL, Reinhold-Keller E. Standardized neurologic evaluations of 128 patients with Wegener granulomatosis. Arch Neurol. 2001;58(8):1215–21.PubMedCrossRefGoogle Scholar
  24. 24.
    Guillevin L, Le Thi Huong D u, 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(4):258–64.PubMedCrossRefGoogle Scholar
  25. 25.
    Travers RL, Allison DJ, Brettle RP, Hughes GR. Polyarteritis nodosa: a clinical and angiographic analysis of 17 cases. Semin Arthritis Rheum. 1979;8(3):184–99.PubMedCrossRefGoogle Scholar
  26. 26.
    Pagnoux C, Seror R, Henegar C, et al. Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database. Arthritis Rheum. 2010;62(2):616–26. doi: 10.1002/art.27240.PubMedCrossRefGoogle Scholar
  27. 27.
    Caselli RJ, Daube JR, Hunder GG, Whisnant JP. Peripheral neuropathic syndromes in giant cell (temporal) arteritis. Neurology. 1988;38(5):685–9.PubMedCrossRefGoogle Scholar
  28. 28.
    Turesson C, Matteson EL. Vasculitis in rheumatoid arthritis. Curr Opin Rheumatol. 2009;21(1):35–40. doi: 10.1097/BOR.0b013e32831c5303.PubMedCrossRefGoogle Scholar
  29. 29.
    Makol A, Crowson CS, Wetter DA, Sokumbi O, Matteson EL, Warrington KJ. Vasculitis associated with rheumatoid arthritis: a case–control study. Rheumatology. 2014;53(5):890–9. doi: 10.1093/rheumatology/ket475.PubMedCentralPubMedCrossRefGoogle Scholar
  30. 30.
    Erhardt CC, Mumford PA, Venables PJ, Maini RN. Factors predicting a poor life prognosis in rheumatoid arthritis: an eight year prospective study. Ann Rheum Dis. 1989;48(1):7–13.PubMedCentralPubMedCrossRefGoogle Scholar
  31. 31.
    Mellgren SI, Conn DL, Stevens JC, Dyck PJ. Peripheral neuropathy in primary Sjögren’s syndrome. Neurology. 1989;39(3):390–4.PubMedCrossRefGoogle Scholar
  32. 32.
    Pavlakis PP, Alexopoulos H, Kosmidis ML, et al. Peripheral neuropathies in Sjögren’s syndrome: a critical update on clinical features and pathogenetic mechanisms. J Autoimmun. 2012;39(1–2):27–33. doi: 10.1016/j.jaut.2012.01.003.PubMedCrossRefGoogle Scholar
  33. 33.
    Govoni M, Bajocchi G, Rizzo N, et al. Neurological involvement in primary Sjögren’s syndrome: clinical and instrumental evaluation in a cohort of Italian patients. Clin Rheumatol. 1999;18(4):299–303.PubMedCrossRefGoogle Scholar
  34. 34.
    Jamilloux Y, Magy L, Hurtevent J-F, et al. Immunological profiles determine neurological involvement in Sjögren’s syndrome. Eur J Intern Med. 2014;25(2):177–81. doi: 10.1016/j.ejim.2013.10.005.PubMedCrossRefGoogle Scholar
  35. 35.
    Wernicke D, Hess H, Gromnica-Ihle E, Krause A, Schmidt WA. Ultrasonography of salivary glands—a highly specific imaging procedure for diagnosis of Sjögren’s syndrome. J Rheumatol. 2008;35(2):285–93.PubMedGoogle Scholar
  36. 36.
    Theander E, Mandl T. Primary Sjögren’s syndrome: diagnostic and prognostic value of salivary gland ultrasonography using a simplified scoring system. Arthrit Care Res (Hoboken). 2014;66(7):1102–7. doi: 10.1002/acr.22264.CrossRefGoogle Scholar
  37. 37.
    Jayne D, Rasmussen N, Andrassy K, et al. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med. 2003;349(1):36–44. doi: 10.1056/NEJMoa020286.PubMedCrossRefGoogle Scholar
  38. 38.
    Westman KW, 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(5):842–52.PubMedGoogle Scholar
  39. 39.
    Hamour S, Salama AD, Pusey CD. Management of ANCA-associated vasculitis: current trends and future prospects. Ther Clin Risk Manag. 2010;6:253–64.PubMedCentralPubMedGoogle Scholar
  40. 40.
    Guillevin L, Lhote F, Jarrousse B, Fain O. Treatment of polyarteritis nodosa and Churg-Strauss syndrome. a meta-analysis of 3 prospective controlled trials including 182 patients over 12 years. Ann Med Interne (Paris). 1992;143(6):405–16.Google Scholar
  41. 41.
    Samson M, Puéchal X, Devilliers H, et al. Mononeuritis multiplex predicts the need for immunosuppressive or immunomodulatory drugs for EGPA, PAN and MPA patients without poor-prognosis factors. Autoimmun Rev. 2014;13(9):945–53. doi: 10.1016/j.autrev.2014.08.002.PubMedCrossRefGoogle Scholar
  42. 42.
    Guillevin L, Cordier JF, Lhote F, 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(12):2187–98. doi: 10.1002/1529-0131(199712)40:12<2187::AID-ART12>3.0.CO;2-H.PubMedCrossRefGoogle Scholar
  43. 43.
    Adu D, Pall A, Luqmani RA, et al. Controlled trial of pulse versus continuous prednisolone and cyclophosphamide in the treatment of systemic vasculitis. QJM. 1997;90(6):401–9.PubMedCrossRefGoogle Scholar
  44. 44.
    Gayraud M, Guillevin L, Cohen 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(12):1290–7.PubMedCrossRefGoogle Scholar
  45. 45.
    Haubitz M, Schellong S, Göbel U, 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(10):1835–44. doi: 10.1002/1529-0131(199810)41:10<1835::AID-ART16>3.0.CO;2-Q.PubMedCrossRefGoogle Scholar
  46. 46.
    De Groot K, Harper L, Jayne DRW, et al. Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial. Ann Intern Med. 2009;150(10):670–80.PubMedCrossRefGoogle Scholar
  47. 47.
    Harper L, Morgan MD, Walsh M, et al. Pulse versus daily oral cyclophosphamide for induction of remission in ANCA-associated vasculitis: long-term follow-up. Ann Rheum Dis. 2012;71(6):955–60. doi: 10.1136/annrheumdis-2011-200477.PubMedCrossRefGoogle Scholar
  48. 48.
    Faurschou M, Sorensen IJ, Mellemkjaer L, et al. Malignancies in Wegener’s granulomatosis: incidence and relation to cyclophosphamide therapy in a cohort of 293 patients. J Rheumatol. 2008;35(1):100–5.PubMedGoogle Scholar
  49. 49.
    Mahr A, Heijl C, Le Guenno G, Faurschou M. ANCA-associated vasculitis and malignancy: current evidence for cause and consequence relationships. Best Pract Res Clin Rheumatol. 2013;27(1):45–56. doi: 10.1016/j.berh.2012.12.003.PubMedCrossRefGoogle Scholar
  50. 50.
    Le Guenno G, Mahr A, Pagnoux C, Dhote R, Guillevin L. Incidence and predictors of urotoxic adverse events in cyclophosphamide-treated patients with systemic necrotizing vasculitides. Arthritis Rheum. 2011;63(5):1435–45. doi: 10.1002/art.30296.PubMedCrossRefGoogle Scholar
  51. 51.
    Gorson KC. Therapy for vasculitic neuropathies. Curr Treat Options Neurol. 2006;8(2):105–17.PubMedCrossRefGoogle Scholar
  52. 52.
    Wall N, Harper L. Complications of long-term therapy for ANCA-associated systemic vasculitis. Nat Rev Nephrol. 2012;8(9):523–32. doi: 10.1038/nrneph.2012.107.PubMedCrossRefGoogle Scholar
  53. 53.
    Mukhtyar C, Guillevin L, Cid MC, et al. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis. 2009;68(3):310–7. doi: 10.1136/ard.2008.088096.PubMedCrossRefGoogle Scholar
  54. 54.
    Specks U, Merkel PA, Seo P, et al. Efficacy of remission-induction regimens for ANCA-associated vasculitis. N Engl J Med. 2013;369(5):417–27. doi: 10.1056/NEJMoa1213277.PubMedCrossRefGoogle Scholar
  55. 55.
    Guillevin L, Pagnoux C, Karras A, et al. Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis. N Engl J Med. 2014;371(19):1771–80. doi: 10.1056/NEJMoa1404231.PubMedCrossRefGoogle Scholar
  56. 56.
    Miloslavsky EM, Specks U, Merkel PA. Rituximab for the treatment of relapses in antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheumatol (Hoboken, NJ). 2014;66(11):3151–9. doi: 10.1002/art.38788.CrossRefGoogle Scholar
  57. 57.
    Thiel J, Hässler F, Salzer U, Voll RE, Venhoff N. Rituximab in the treatment of refractory or relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Arthritis Res Ther. 2013;15(5):R133. doi: 10.1186/ar4313.PubMedCentralPubMedCrossRefGoogle Scholar
  58. 58.
    Jones RB, Ferraro AJ, Chaudhry AN, et al. A multicenter survey of rituximab therapy for refractory antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2009;60(7):2156–68. doi: 10.1002/art.24637.PubMedCrossRefGoogle Scholar
  59. 59.
    Cartin-Ceba R, Golbin JM, Keogh KA, et al. Rituximab for remission induction and maintenance in refractory granulomatosis with polyangiitis (Wegener’s): ten-year experience at a single center. Arthritis Rheum. 2012;64(11):3770–8. doi: 10.1002/art.34584.PubMedCrossRefGoogle Scholar
  60. 60.
    Puéchal X, Gottenberg JE, Berthelot JM, et al. Rituximab therapy for systemic vasculitis associated with rheumatoid arthritis: results from the AutoImmunity and Rituximab Registry. Arthrit Care Res (Hoboken). 2012;64(3):331–9. doi: 10.1002/acr.20689.CrossRefGoogle Scholar
  61. 61.
    Kissel JT, Levy RJ, Mendell JR, Griggs RC. Azathioprine toxicity in neuromuscular disease. Neurology. 1986;36(1):35–9.PubMedCrossRefGoogle Scholar
  62. 62.
    De Groot K, Rasmussen N, Bacon PA, et al. Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2005;52(8):2461–9. doi: 10.1002/art.21142.PubMedCrossRefGoogle Scholar
  63. 63.
    Jayne DR, Chapel H, Adu D, et al. Intravenous immunoglobulin for ANCA-associated systemic vasculitis with persistent disease activity. QJM. 2000;93(7):433–9.PubMedCrossRefGoogle Scholar
  64. 64.
    Levy Y, Uziel Y, Zandman G, et al. Response of vasculitic peripheral neuropathy to intravenous immunoglobulin. Ann N Y Acad Sci. 2005;1051:779–86. doi: 10.1196/annals.1361.121.PubMedCrossRefGoogle Scholar
  65. 65.
    Tsurikisawa N, Taniguchi M, Saito H, et al. Treatment of Churg-Strauss syndrome with high-dose intravenous immunoglobulin. Ann Allergy Asthma Immunol. 2004;92(1):80–7. doi: 10.1016/S1081-1206(10)61714-0.PubMedCrossRefGoogle Scholar
  66. 66.
    Martinez V, Cohen P, Pagnoux C, et al. 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(1):308–17. doi: 10.1002/art.23147.PubMedCrossRefGoogle Scholar
  67. 67.
    Matsuda T, Arimura Y, Yoshihara K, Komagata Y, Kaname S, Yamada A. Efficacy of high-dose intravenous immunoglobulin therapy for peripheral neuropathy in the remission stage of eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome). Nihon Rinsho Meneki Gakkai Kaishi. 2013;36(4):217–25.PubMedCrossRefGoogle Scholar
  68. 68.
    Koike H, Akiyama K, Saito T, Sobue G. Intravenous immunoglobulin for chronic residual peripheral neuropathy in eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome): a multicenter, double-blind trial. J Neurol. 2015;262(3):752–9. doi: 10.1007/s00415-014-7618-y.PubMedCentralPubMedCrossRefGoogle Scholar
  69. 69.
    Walsh M, Casian A, Flossmann O, et al. Long-term follow-up of patients with severe ANCA-associated vasculitis comparing plasma exchange to intravenous methylprednisolone treatment is unclear. Kidney Int. 2013;84(2):397–402. doi: 10.1038/ki.2013.131.PubMedCrossRefGoogle Scholar
  70. 70.
    Guillevin L, Fain O, Lhote F, et al. 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(2):208–15.PubMedCrossRefGoogle Scholar
  71. 71.
    Hiemstra TF, Walsh M, Mahr A, et al. Mycophenolate mofetil vs azathioprine for remission maintenance in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized controlled trial. JAMA. 2010;304(21):2381–8. doi: 10.1001/jama.2010.1658.PubMedCrossRefGoogle Scholar
  72. 72.
    Metzler C, Miehle N, Manger K, et al. Elevated relapse rate under oral methotrexate versus leflunomide for maintenance of remission in Wegener’s granulomatosis. Rheumatology (Oxford). 2007;46(7):1087–91. doi: 10.1093/rheumatology/kem029.CrossRefGoogle Scholar
  73. 73.
    Metzler C, Schnabel A, Gross WL, Hellmich B. A phase II study of interferon-alpha for the treatment of refractory Churg-Strauss syndrome. Clin Exp Rheumatol. 2008;26(3 Suppl 49):S35–40.PubMedGoogle Scholar
  74. 74.
    Moosig F, Gross WL, Herrmann K, Bremer JP, Hellmich B. Targeting interleukin-5 in refractory and relapsing Churg-Strauss syndrome. Ann Intern Med. 2011;155(5):341–3. doi: 10.7326/0003-4819-155-5-201109060-00026.PubMedCrossRefGoogle Scholar
  75. 75.
    Guillevin L, Pagnoux C. Therapeutic strategies for systemic necrotizing vasculitides. Allergol Int. 2007;56(2):105–11. doi: 10.2332/allergolint.R-07-144.PubMedCrossRefGoogle Scholar
  76. 76.
    Mazzaro C, Zorat F, Caizzi M, et al. Treatment with peg-interferon alfa-2b and ribavirin of hepatitis C virus-associated mixed cryoglobulinemia: a pilot study. J Hepatol. 2005;42(5):632–8. doi: 10.1016/j.jhep.2004.10.031.PubMedCrossRefGoogle Scholar
  77. 77.
    Saadoun D, Resche-Rigon M, Thibault V, Piette J-C, Cacoub P. Antiviral therapy for hepatitis C virus—associated mixed cryoglobulinemia vasculitis: a long-term followup study. Arthritis Rheum. 2006;54(11):3696–706. doi: 10.1002/art.22168.PubMedCrossRefGoogle Scholar
  78. 78.
    Saadoun D, Resche Rigon M, Thibault V, et al. Peg-IFNα/ribavirin/protease inhibitor combination in hepatitis C virus associated mixed cryoglobulinemia vasculitis: results at week 24. Ann Rheum Dis. 2014;73(5):831–7. doi: 10.1136/annrheumdis-2012-202770.PubMedCentralPubMedCrossRefGoogle Scholar
  79. 79.
    Saadoun D, Resche Rigon M, Pol S, et al. PegIFNα/ribavirin/protease inhibitor combination in severe hepatitis C virus-associated mixed cryoglobulinemia vasculitis. J Hepatol. 2015;62(1):24–30. doi: 10.1016/j.jhep.2014.08.015.PubMedCrossRefGoogle Scholar
  80. 80.•
    Cacoub P, Terrier B, Saadoun D. Hepatitis C virus-induced vasculitis: therapeutic options. Ann Rheum Dis. 2014;73(1):24–30. doi: 10.1136/annrheumdis-2013-203883. It is an overview of the treatment options for infectious large arteriole vasculitis caused by hepatitis C virus.PubMedCrossRefGoogle Scholar
  81. 81.
    Saadoun D, Resche Rigon M, Sene D, et al. Rituximab plus Peg-interferon-alpha/ribavirin compared with Peg-interferon-alpha/ribavirin in hepatitis C-related mixed cryoglobulinemia. Blood. 2010;116(3):326–34. doi: 10.1182/blood-2009-10-248518. quiz 504–505.PubMedCrossRefGoogle Scholar
  82. 82.
    Saadoun D, Resche-Rigon M, Sene D, Perard L, Karras A, Cacoub P. Rituximab combined with Peg-interferon-ribavirin in refractory hepatitis C virus-associated cryoglobulinaemia vasculitis. Ann Rheum Dis. 2008;67(10):1431–6. doi: 10.1136/ard.2007.081653.PubMedCrossRefGoogle Scholar
  83. 83.
    Ramos-Casals M, Stone JH, Cid MC, Bosch X. The cryoglobulinaemias. Lancet. 2012;379(9813):348–60. doi: 10.1016/S0140-6736(11)60242-0.PubMedCrossRefGoogle Scholar
  84. 84.
    Saadoun D, Rosenzwajg M, Joly F, et al. Regulatory T-cell responses to low-dose interleukin-2 in HCV-induced vasculitis. N Engl J Med. 2011;365(22):2067–77. doi: 10.1056/NEJMoa1105143.PubMedCrossRefGoogle Scholar
  85. 85.
    Guillevin L, Mahr A, Callard P, et al. Hepatitis B virus-associated polyarteritis nodosa: clinical characteristics, outcome, and impact of treatment in 115 patients. Medicine (Baltimore). 2005;84(5):313–22.CrossRefGoogle Scholar
  86. 86.
    Dyck PJ, Benstead TJ, Conn DL, Stevens JC, Windebank AJ, Low PA. Nonsystemic vasculitic neuropathy. Brain. 1987;110(Pt 4):843–53.PubMedCrossRefGoogle Scholar
  87. 87.
    Davies L, Spies JM, Pollard JD, McLeod JG. Vasculitis confined to peripheral nerves. Brain. 1996;119(Pt 5):1441–8.PubMedCrossRefGoogle Scholar
  88. 88.
    Sugiura M, Koike H, Iijima M, et al. Clinicopathologic features of nonsystemic vasculitic neuropathy and microscopic polyangiitis-associated neuropathy: a comparative study. J Neurol Sci. 2006;241(1–2):31–7. doi: 10.1016/j.jns.2005.10.018.PubMedCrossRefGoogle Scholar
  89. 89.
    Dyck PJ, Norell JE. Microvasculitis and ischemia in diabetic lumbosacral radiculoplexus neuropathy. Neurology. 1999;53(9):2113–21.PubMedCrossRefGoogle Scholar
  90. 90.
    Dyck PJB, Windebank AJ. Diabetic and nondiabetic lumbosacral radiculoplexus neuropathies: new insights into pathophysiology and treatment. Muscle Nerve. 2002;25(4):477–91.PubMedCrossRefGoogle Scholar
  91. 91.
    Massie R, Mauermann ML, Staff NP, et al. Diabetic cervical radiculoplexus neuropathy: a distinct syndrome expanding the spectrum of diabetic radiculoplexus neuropathies. Brain. 2012;135(Pt 10):3074–88. doi: 10.1093/brain/aws244.PubMedCrossRefGoogle Scholar
  92. 92.
    Dyck PJ, Engelstad J, Norell J. Microvasculitis in non-diabetic lumbosacral radiculoplexus neuropathy (LSRPN): similarity to the diabetic variety (DLSRPN). J Neuropathol Exp Neurol. 2000;59(6):525–38.PubMedGoogle Scholar
  93. 93.
    Evans BA, Stevens JC, Dyck PJ. Lumbosacral plexus neuropathy. Neurology. 1981;31(10):1327–30.PubMedCrossRefGoogle Scholar
  94. 94.
    Sander JE, Sharp FR. Lumbosacral plexus neuritis. Neurology. 1981;31(4):470–3.PubMedCrossRefGoogle Scholar
  95. 95.
    Dyck PJ, Norell JE. Non-diabetic lumbosacral radiculoplexus neuropathy: natural history, outcome and comparison with the diabetic variety. Brain. 2001;124(Pt 6):1197–207.PubMedCrossRefGoogle Scholar
  96. 96.
    Garces-Sanchez M, Laughlin RS, Dyck PJ, Engelstad JK, Norell JE, Dyck PJB. Painless diabetic motor neuropathy: a variant of diabetic lumbosacral radiculoplexus neuropathy? Ann Neurol. 2011;69(6):1043–54. doi: 10.1002/ana.22334.PubMedCentralPubMedCrossRefGoogle Scholar
  97. 97.
    Collins MP, Dyck PJB, Gronseth GS, et al. Peripheral Nerve Society Guideline on the classification, diagnosis, investigation, and immunosuppressive therapy of non-systemic vasculitic neuropathy: executive summary. J Peripher Nerv Syst. 2010;15(3):176–84. doi: 10.1111/j.1529-8027.2010.00281.x.PubMedCrossRefGoogle Scholar
  98. 98.
    Thaisetthawatkul P, Dyck PJB. Treatment of diabetic and nondiabetic lumbosacral radiculoplexus neuropathy. Curr Treat Options Neurol. 2010;12(2):95–9. doi: 10.1007/s11940-010-0059-8.PubMedCrossRefGoogle Scholar
  99. 99.
    Dyck PJB, O’brien PBP. The multi-center double-blind controlled trial of IV methylprednisolone in diabetic lumbosacral radiculoplexus neuropathy. Neurology. 2006;66 suppl 2:A191.Google Scholar
  100. 100.
    Said G, Goulon-Goeau C, Lacroix C, Moulonguet A. Nerve biopsy findings in different patterns of proximal diabetic neuropathy. Ann Neurol. 1994;35(5):559–69. doi: 10.1002/ana.410350509.PubMedCrossRefGoogle Scholar
  101. 101.
    Krendel DA, Costigan DA, Hopkins LC. Successful treatment of neuropathies in patients with diabetes mellitus. Arch Neurol. 1995;52(11):1053–61.PubMedCrossRefGoogle Scholar
  102. 102.
    Pascoe MK, Low PA, Windebank AJ, Litchy WJ. Subacute diabetic proximal neuropathy. Mayo Clin Proc. 1997;72(12):1123–32. doi: 10.1016/S0025-6196(11)63674-4.PubMedCrossRefGoogle Scholar
  103. 103.
    Kilfoyle D, Kelkar P, Parry GJ. Pulsed methylprednisolone is a safe and effective treatment for diabetic amyotrophy. J Clin Neuromuscul Dis. 2003;4(4):168–70.PubMedCrossRefGoogle Scholar
  104. 104.
    Dyck PJ, Norell JE. Methylprednisolone may improve lumbosacral radiculoplexus neuropathy. Can J Neurol Sci. 2001;28(3):224–7.PubMedCrossRefGoogle Scholar
  105. 105.
    Bradley WG, Chad D, Verghese JP, et al. Painful lumbosacral plexopathy with elevated erythrocyte sedimentation rate: a treatable inflammatory syndrome. Ann Neurol. 1984;15(5):457–64. doi: 10.1002/ana.410150510.PubMedCrossRefGoogle Scholar
  106. 106.
    Tamburin S, Zanette G. Intravenous immunoglobulin for the treatment of diabetic lumbosacral radiculoplexus neuropathy. Pain Med. 2009;10(8):1476–80. doi: 10.1111/j.1526-4637.2009.00704.x.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2015

Authors and Affiliations

  1. 1.Department of NeurologyMayo ClinicRochesterUSA

Personalised recommendations