DOI QR코드

DOI QR Code

길랭-바레증후군과 유사한 급성다발신경병증을 보인 호산구육아종증 1예

Eosinophilic granulomatosis with polyangiitis presenting with acutepolyneuropathy mimicking Guillain-Barré syndrome: A case report

  • 김지선 (영남대학교 의과대학 내과학교실) ;
  • 안준홍 (영남대학교 의과대학 내과학교실) ;
  • 진현정 (영남대학교 의과대학 내과학교실)
  • Kim, Jee Seon (Department of Internal Medicine, Yeungnam University College of Medicine) ;
  • Ahn, June Hong (Department of Internal Medicine, Yeungnam University College of Medicine) ;
  • Jin, Hyun Jung (Department of Internal Medicine, Yeungnam University College of Medicine)
  • 투고 : 2017.07.22
  • 심사 : 2017.08.30
  • 발행 : 2018.01.31

초록

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis that commonly affects the peripheral nervous system. EGPA rarely presents with acute polyneuropathy resembling $Guillain-Barr{\acute{e}}$ syndrome (GBS). A 51-year-old female patient with a history of asthma suddenly developed bilateral lower extremityparesthesia that progressed to asymmetric ascending paralysis within 10 days of onset. Nerve conduction study results were compatible with acute motor sensory axonal neuropathy, consistent with a GBS subtype. A clinical and neurophysiological diagnosis of GBS was made, and high-dose intravenous immunoglobulins were administered. However, the patient's painful motor weakness persisted. Furthermore, she had newly developed skin lesions on her back, face, and arms. Her blood test revealed marked eosinophilia (> 60%). In addition, antineutrophil cytoplasmic antibodies were reported positive. A Water's view radiographic image showed bilateral maxillary sinusitis. Considering the history of asthma, we suspected EGPA-associated polyneuropathy and started steroid treatment. The patient's strength and eosinophilia improved rapidly and dramatically. EGPA can mimic GBS and should be differentiated because of different treatment strategies. Early diagnosis and prompt treatment help achieve a good outcome.

키워드

참고문헌

  1. Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 1951;27:277-301.
  2. Noth O, Strek ME, Leff AR. Churg-Strauss syndrome. Lancet 2003;361; 587-94. https://doi.org/10.1016/S0140-6736(03)12518-4
  3. Kang DW, Jeong IK, Kim HJ, Lee KW. Neurologic manifestations of Churg-Strauss syndrome. J Korean Neurol Assoc 1999;17:836-40.
  4. Yi JS, Jung HY, Choi SW. A case of acute fulminant neuropathy in patient with allergic granulomatosis and angiitis. J Korean Rheum Assoc 1998;5: 298-302.
  5. Oh MJ, Lee JY, Kwon NH, Choi DC. Churg-Strauss syndrome: the clinical features and long-term follow-up of 17 patients. J Korean Med Sci 2006;21:265-71. https://doi.org/10.3346/jkms.2006.21.2.265
  6. Camara-Lemarroy CR, Infante-Valenzuela A, Villareal-Montemayor HJ, Soto-Rincon CA, Davila-Olalde JA, Villareal-Velazquez HJ. Eosinophilic granulomatosis with polyangiitis presenting as acute polyneuropathy mimicking Guillain-Barre syndrome. Case Rep Neurol Med 2015;2015: 981439.
  7. Cho HJ, Yune S, Seok JM, Cho EB, Min JH, Seo YL, et al. Clinical characteristics and treatment response of peripheral neuropathy in the presence of eosinophilic granulomatosis with polyangiitis (Churg-Strauss Syndrome): experience at a single tertiary center. J Clin Neurol 2017;13:77-83. https://doi.org/10.3988/jcn.2017.13.1.77
  8. Santos-Pinheiro F, Li Y. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) presenting with polyneuropathy: a case series. J Clin Neuromuscul Dis 2015;16:125-30. https://doi.org/10.1097/CND.0000000000000068
  9. Seok JI, Bae JS, Joo EY, Min TH, Choi DC, Kim BJ. Clinical and electrophysiologic features of peripheral neuropathy in Churg-Strauss syndrome. J Korean Neurol Assoc 2004;22:127-33.
  10. Jamieson PW, Giuliani MJ, Martinez AJ. Necrotizing angiopathy presenting with multifocal conduction blocks. Neurology 1991;41:442-4. https://doi.org/10.1212/WNL.41.3.442
  11. Winer JB. An update in guillain-barre syndrome. Autoimmune Dis 2014; 2014:793024.
  12. Cornblath DR. Electrophysiology in Guillain-Barre syndrome. Ann Neurol 1990;27 Suppl:S17-20. https://doi.org/10.1002/ana.410270706
  13. Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 1990;33:1094-100.
  14. Giusti Del Giardino L, Cavallaro T, Anzola GP, Lombardi C, Ferrari S. Neuropathy in eosinophilic granulomatosis with polyangiitis: a comparison study of 24 cases with or without prior leukotriene antagonist exposure. Eur Ann Allergy Clin Immunol 2014;46:201-9.
  15. Ribi C, Cohen P, Pagnoux C, Mahr A, Arene JP, Lauque D, et al. Treatment of Churg-Strauss syndrome without poor-prognosis factors: a multicenter, prospective, randomized, open-label study of seventy-two patients. Arthritis Rheum 2008;58:586-94. https://doi.org/10.1002/art.23198
  16. Guillevin L, Lhote F, Gayraud M, Cohen P, Jarrousse B, Lortholary O, et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients. Medicine (Baltimore) 1996; 75:17-28. https://doi.org/10.1097/00005792-199601000-00003
  17. Koike H, Akiyama K, Saito T, Sobue G; Research Group for IVIg for EGPA/CSS in Japan. Intravenous immunoglobulin for chronic residual peripheral neuropathy in eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome): a multicenter, double-blind trial. J Neurol 2015;262:752-9. https://doi.org/10.1007/s00415-014-7618-y
  18. Hattori N, Ichimura M, Nagamatsu M, Li M, Yamamoto K, Kumazawa K, et al. Clinicopathological features of Churg-Strauss syndrome-associated neuropathy. Brain 1999;122(Pt 3):427-39. https://doi.org/10.1093/brain/122.3.427