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嗜酸性肉芽肿性血管炎(Churg-Strauss综合征)的流行病学、发病机制和病理学

Author
Talmadge E King, Jr, MD
Section Editors
Kevin R Flaherty, MD, MS
Richard J Glassock, MD, MACP
Bruce S Bochner, MD
Deputy Editor
Helen Hollingsworth, MD
Translators
徐健, 副主任医师，教授

引言

嗜酸性肉芽肿性血管炎(eosinophilic granulomatosis with polyangiitis, EGPA)，以前被称作Churg-Strauss综合征(Churg-Strauss syndrome, CSS)或变应性肉芽肿血管炎，该病是一种多系统疾病，其特征为变态反应性鼻炎、哮喘和显著的外周血嗜酸性粒细胞增多[1-9]。EGPA被归类为中小动脉的血管炎，但血管炎在疾病初期常不明显。

最常受累的器官是肺，其次是皮肤。然而，EGPA可影响任何器官系统，包括心血管系统、胃肠道系统、肾脏系统和中枢神经系统。肺外器官的血管炎是导致EGPA相关并发症和死亡的主要原因。

EGPA的流行病学、发病机制和病理学将总结在此。该病的临床特征、诊断、治疗和预后，以及对血管炎和/或嗜酸性粒细胞增多的患者的概述将单独讨论。 (参见“嗜酸性肉芽肿性多血管炎(变态性肉芽肿血管炎)的临床特征和诊断”和“Treatment and prognosis of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)”和“成人血管炎的分类及诊断方法”和“对不明原因嗜酸性粒细胞增多患者的概述”)

流行病学

由于EGPA诊断相关的不确定性，所有目前仍不清楚其流行病学特征[10]。有一种主要形式的血管炎的患者中，认为约10%有EGPA。在3种抗中性粒细胞胞质抗体(anti-neutrophil cytoplasmic antibodies, ANCA)相关血管炎[即，EGPA、肉芽肿性血管炎(Wegener肉芽肿)和显微镜下多血管炎]中，EGPA最少见[5]。 (参见“成人血管炎的分类及诊断方法”)

EGPA诊断时的平均年龄为40岁[11]。EGPA是65岁以上人群发生血管炎的不常见原因；一项研究显示，在38例存在不同系统形式血管炎的老年患者中，经过组织学检查证实，EGPA占血管炎的5%[12,13]。EGPA在儿童和青少年中也罕见；当EGPA发生于这一年龄人群时，其病程似乎更具侵袭性，有明显的肺部和心血管表现[14,15]。

          

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Literature review current through: 2017-06 . | This topic last updated: 2016-07-11.
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References
Top
  1. CHURG J, STRAUSS L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 1951; 27:277.
  2. Churg A. Pulmonary angiitis and granulomatosis revisited. Hum Pathol 1983; 14:868.
  3. Sinico RA, Bottero P. Churg-Strauss angiitis. Best Pract Res Clin Rheumatol 2009; 23:355.
  4. Pagnoux C, Guilpain P, Guillevin L. Churg-Strauss syndrome. Curr Opin Rheumatol 2007; 19:25.
  5. Keogh KA, Specks U. Churg-Strauss syndrome. Semin Respir Crit Care Med 2006; 27:148.
  6. Guillevin L, Cohen P, Gayraud M, et al. Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine (Baltimore) 1999; 78:26.
  7. 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.
  8. Masi AT, Hunder GG, Lie JT, 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.
  9. Szczeklik W, Jakieła B, Adamek D, Musiał J. Cutting edge issues in the Churg-Strauss syndrome. Clin Rev Allergy Immunol 2013; 44:39.
  10. Schwartz RA, Churg J. Churg-Strauss syndrome. Br J Dermatol 1992; 127:199.
  11. Conron M, Beynon HL. Churg-Strauss syndrome. Thorax 2000; 55:870.
  12. Mouthon L, Le Toumelin P, Andre MH, et al. Polyarteritis nodosa and Churg-Strauss angiitis: characteristics and outcome in 38 patients over 65 years. Medicine (Baltimore) 2002; 81:27.
  13. Abril A, Calamia KT, Cohen MD. The Churg Strauss syndrome (allergic granulomatous angiitis): review and update. Semin Arthritis Rheum 2003; 33:106.
  14. Zwerina J, Eger G, Englbrecht M, et al. Churg-Strauss syndrome in childhood: a systematic literature review and clinical comparison with adult patients. Semin Arthritis Rheum 2009; 39:108.
  15. Gendelman S, Zeft A, Spalding SJ. Childhood-onset eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome): a contemporary single-center cohort. J Rheumatol 2013; 40:929.
  16. Harrold LR, Andrade SE, Go AS, et al. Incidence of Churg-Strauss syndrome in asthma drug users: a population-based perspective. J Rheumatol 2005; 32:1076.
  17. Sada KE, Amano K, Uehara R, et al. A nationwide survey on the epidemiology and clinical features of eosinophilic granulomatosis with polyangiitis (Churg-Strauss) in Japan. Mod Rheumatol 2014; 24:640.
  18. Hellmich B, Ehlers S, Csernok E, Gross WL. Update on the pathogenesis of Churg-Strauss syndrome. Clin Exp Rheumatol 2003; 21:S69.
  19. Zwerina J, Axmann R, Jatzwauk M, et al. Pathogenesis of Churg-Strauss syndrome: recent insights. Autoimmunity 2009; 42:376.
  20. Schmitt WH, Csernok E, Kobayashi S, et al. Churg-Strauss syndrome: serum markers of lymphocyte activation and endothelial damage. Arthritis Rheum 1998; 41:445.
  21. Hellmich B, Csernok E, Gross WL. Proinflammatory cytokines and autoimmunity in Churg-Strauss syndrome. Ann N Y Acad Sci 2005; 1051:121.
  22. Tsurikisawa N, Saito H, Tsuburai T, et al. Differences in regulatory T cells between Churg-Strauss syndrome and chronic eosinophilic pneumonia with asthma. J Allergy Clin Immunol 2008; 122:610.
  23. Vaglio A, Martorana D, Maggiore U, et al. HLA-DRB4 as a genetic risk factor for Churg-Strauss syndrome. Arthritis Rheum 2007; 56:3159.
  24. Wieczorek S, Hellmich B, Arning L, et al. Functionally relevant variations of the interleukin-10 gene associated with antineutrophil cytoplasmic antibody-negative Churg-Strauss syndrome, but not with Wegener's granulomatosis. Arthritis Rheum 2008; 58:1839.
  25. Wechsler ME, Garpestad E, Flier SR, et al. Pulmonary infiltrates, eosinophilia, and cardiomyopathy following corticosteroid withdrawal in patients with asthma receiving zafirlukast. JAMA 1998; 279:455.
  26. Franco J, Artés MJ. Pulmonary eosinophilia associated with montelukast. Thorax 1999; 54:558.
  27. Green RL, Vayonis AG. Churg-Strauss syndrome after zafirlukast in two patients not receiving systemic steroid treatment. Lancet 1999; 353:725.
  28. Kinoshita M, Shiraishi T, Koga T, et al. Churg-Strauss syndrome after corticosteroid withdrawal in an asthmatic patient treated with pranlukast. J Allergy Clin Immunol 1999; 103:534.
  29. Martin RM, Wilton LV, Mann RD. Prevalence of Churg-Strauss syndrome, vasculitis, eosinophilia and associated conditions: retrospective analysis of 58 prescription-event monitoring cohort studies. Pharmacoepidemiol Drug Saf 1999; 8:179.
  30. Wechsler ME, Finn D, Gunawardena D, et al. Churg-Strauss syndrome in patients receiving montelukast as treatment for asthma. Chest 2000; 117:708.
  31. Hauser T, Mahr A, Metzler C, et al. The leucotriene receptor antagonist montelukast and the risk of Churg-Strauss syndrome: a case-crossover study. Thorax 2008; 63:677.
  32. D'Cruz DP, Barnes NC, Lockwood CM. Difficult asthma or Churg-Strauss syndrome? BMJ 1999; 318:475.
  33. Le Gall C, Pham S, Vignes S, et al. Inhaled corticosteroids and Churg-Strauss syndrome: a report of five cases. Eur Respir J 2000; 15:978.
  34. Cooper SM, Libman BS, Lazarovich M. Churg-strauss syndrome in a group of patients receiving fluticasone for asthma. J Rheumatol 2002; 29:2651.
  35. Tuggey JM, Hosker HS. Churg-Strauss syndrome associated with montelukast therapy. Thorax 2000; 55:805.
  36. Katsura T, Yoshida F, Takinishi Y. The Churg-Strauss syndrome after pranlukast treatment in a patient not receiving corticosteroids. Ann Intern Med 2003; 139:386.
  37. Nathani N, Little MA, Kunst H, et al. Churg-Strauss syndrome and leukotriene antagonist use: a respiratory perspective. Thorax 2008; 63:883.
  38. Keogh KA, Specks U. Churg-Strauss syndrome: clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists. Am J Med 2003; 115:284.
  39. Specks, U. Pulmonary vasculitis. In: Interstitial Lung Disease, 5th, Schwarz, MI, King, TE Jr (Eds), People's Medical Publishing House, Shelton, CT, USA 2011. p.765.
  40. Keogh KA. Leukotriene receptor antagonists and Churg-Strauss syndrome: cause, trigger or merely an association? Drug Saf 2007; 30:837.
  41. Bibby S, Healy B, Steele R, et al. Association between leukotriene receptor antagonist therapy and Churg-Strauss syndrome: an analysis of the FDA AERS database. Thorax 2010; 65:132.
  42. Wechsler ME, Wong DA, Miller MK, Lawrence-Miyasaki L. Churg-strauss syndrome in patients treated with omalizumab. Chest 2009; 136:507.
  43. Ruppert AM, Averous G, Stanciu D, et al. Development of Churg-Strauss syndrome with controlled asthma during omalizumab treatment. J Allergy Clin Immunol 2008; 121:253.
  44. Orriols R, Muñoz X, Ferrer J, et al. Cocaine-induced Churg-Strauss vasculitis. Eur Respir J 1996; 9:175.
  45. Wiesner O, Russell KA, Lee AS, et al. Antineutrophil cytoplasmic antibodies reacting with human neutrophil elastase as a diagnostic marker for cocaine-induced midline destructive lesions but not autoimmune vasculitis. Arthritis Rheum 2004; 50:2954.
  46. Peikert T, Finkielman JD, Hummel AM, et al. Functional characterization of antineutrophil cytoplasmic antibodies in patients with cocaine-induced midline destructive lesions. Arthritis Rheum 2008; 58:1546.
  47. Churg A. Recent advances in the diagnosis of Churg-Strauss syndrome. Mod Pathol 2001; 14:1284.
  48. Katzenstein AL. Diagnostic features and differential diagnosis of Churg-Strauss syndrome in the lung. A review. Am J Clin Pathol 2000; 114:767.
  49. Lie JT. Illustrated histopathologic classification criteria for selected vasculitis syndromes. American College of Rheumatology Subcommittee on Classification of Vasculitis. Arthritis Rheum 1990; 33:1074.
  50. Sinico RA, Di Toma L, Maggiore U, et al. Renal involvement in Churg-Strauss syndrome. Am J Kidney Dis 2006; 47:770.
  51. Clutterbuck EJ, Evans DJ, Pusey CD. Renal involvement in Churg-Strauss syndrome. Nephrol Dial Transplant 1990; 5:161.
  52. Neumann T, Manger B, Schmid M, et al. Cardiac involvement in Churg-Strauss syndrome: impact of endomyocarditis. Medicine (Baltimore) 2009; 88:236.
  53. Kawakami T, Soma Y, Kawasaki K, et al. Initial cutaneous manifestations consistent with mononeuropathy multiplex in Churg-Strauss syndrome. Arch Dermatol 2005; 141:873.
  54. Churg A, Brallas M, Cronin SR, Churg J. Formes frustes of Churg-Strauss syndrome. Chest 1995; 108:320.
  55. Hueto-Perez-de-Heredia JJ, Dominguez-del-Valle FJ, Garcia E, et al. Chronic eosinophilic pneumonia as a presenting feature of Churg-Strauss syndrome. Eur Respir J 1994; 7:1006.