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Clinical features and diagnosis of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

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

INTRODUCTION

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss), abbreviated EGPA, which was previously called the Churg-Strauss syndrome (CSS) or allergic granulomatosis and angiitis, is a multisystem disorder characterized by chronic rhinosinusitis, asthma, and prominent peripheral blood eosinophilia [1-7]. EGPA is classified as a vasculitis of the small and medium sized arteries, although the vasculitis is often not apparent in the initial phases of the disease.

The most commonly involved organ is the lung, followed by the skin. EGPA, however, can affect any organ system, including the cardiovascular, gastrointestinal, renal, and central nervous systems. Vasculitis of extrapulmonary organs is largely responsible for the morbidity and mortality associated with EGPA.

The clinical features and diagnosis of EGPA will be reviewed here. The epidemiology, pathogenesis, treatment, and prognosis of this disorder, as well as the approach to patients with vasculitis and/or eosinophilia are discussed separately. (See "Epidemiology, pathogenesis, and pathology of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)" and "Treatment and prognosis of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)" and "Overview of and approach to the vasculitides in adults" and "Approach to the patient with unexplained eosinophilia".)

CLINICAL FEATURES

Phases of disease — The clinical features of EGPA typically develop in several sequential phases, although these phases are not always clearly distinguishable [8,9]:

Prodromal phase – The prodromal phase occurs among individuals in the second and third decades of life and is characterized by atopic disease, allergic rhinitis, and asthma.

                         

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Literature review current through: Nov 2016. | This topic last updated: Fri Jul 08 00:00:00 GMT+00:00 2016.
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References
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  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. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65:1.
  8. 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.
  9. Pagnoux C, Guillevin L. Churg-Strauss syndrome: evidence for disease subtypes? Curr Opin Rheumatol 2010; 22:21.
  10. 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; 65:270.
  11. Cottin V, Khouatra C, Dubost R, et al. Persistent airflow obstruction in asthma of patients with Churg-Strauss syndrome and long-term follow-up. Allergy 2009; 64:589.
  12. 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.
  13. Churg A, Brallas M, Cronin SR, Churg J. Formes frustes of Churg-Strauss syndrome. Chest 1995; 108:320.
  14. Chumbley LC, Harrison EG Jr, DeRemee RA. Allergic granulomatosis and angiitis (Churg-Strauss syndrome). Report and analysis of 30 cases. Mayo Clin Proc 1977; 52:477.
  15. Bacciu A, Buzio C, Giordano D, et al. Nasal polyposis in Churg-Strauss syndrome. Laryngoscope 2008; 118:325.
  16. Bacciu A, Bacciu S, Mercante G, et al. Ear, nose and throat manifestations of Churg-Strauss syndrome. Acta Otolaryngol 2006; 126:503.
  17. Ishiyama A, Canalis RF. Otological manifestations of Churg-Strauss syndrome. Laryngoscope 2001; 111:1619.
  18. Nakamaru Y, Takagi D, Suzuki M, et al. Otologic and Rhinologic Manifestations of Eosinophilic Granulomatosis with Polyangiitis. Audiol Neurootol 2016; 21:45.
  19. Schwartz RA, Churg J. Churg-Strauss syndrome. Br J Dermatol 1992; 127:199.
  20. Kawakami T, Soma Y, Kawasaki K, et al. Initial cutaneous manifestations consistent with mononeuropathy multiplex in Churg-Strauss syndrome. Arch Dermatol 2005; 141:873.
  21. Neumann T, Manger B, Schmid M, et al. Cardiac involvement in Churg-Strauss syndrome: impact of endomyocarditis. Medicine (Baltimore) 2009; 88:236.
  22. Corradi D, Maestri R, Facchetti F. Postpartum Churg-Strauss syndrome with severe cardiac involvement: description of a case and review of the literature. Clin Rheumatol 2009; 28:739.
  23. Dennert RM, van Paassen P, Schalla S, et al. Cardiac involvement in Churg-Strauss syndrome. Arthritis Rheum 2010; 62:627.
  24. 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; 72:1011.
  25. Mehta AK, Langford CA, Taylor DO, et al. A 39-Year-Old Postpartum Woman With Foot Drop and Shortness of Breath. Chest 2016; 149:e61.
  26. Szczeklik W, Miszalski-Jamka T, Mastalerz L, et al. Multimodality assessment of cardiac involvement in Churg-Strauss syndrome patients in clinical remission. Circ J 2011; 75:649.
  27. Allenbach Y, Seror R, Pagnoux C, et al. High frequency of venous thromboembolic events in Churg-Strauss syndrome, Wegener's granulomatosis and microscopic polyangiitis but not polyarteritis nodosa: a systematic retrospective study on 1130 patients. Ann Rheum Dis 2009; 68:564.
  28. Della Rossa A, Baldini C, Tavoni A, et al. Churg-Strauss syndrome: clinical and serological features of 19 patients from a single Italian centre. Rheumatology (Oxford) 2002; 41:1286.
  29. Solans R, Bosch JA, Pérez-Bocanegra C, et al. Churg-Strauss syndrome: outcome and long-term follow-up of 32 patients. Rheumatology (Oxford) 2001; 40:763.
  30. Wolf J, Bergner R, Mutallib S, et al. Neurologic complications of Churg-Strauss syndrome--a prospective monocentric study. Eur J Neurol 2010; 17:582.
  31. 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.
  32. Hattori N, Ichimura M, Nagamatsu M, et al. Clinicopathological features of Churg-Strauss syndrome-associated neuropathy. Brain 1999; 122 ( Pt 3):427.
  33. Liou HH, Liu HM, Chiang IP, et al. Churg-Strauss syndrome presented as multiple intracerebral hemorrhage. Lupus 1997; 6:279.
  34. Sheerin UM, Barreto J, Brown MM, et al. Subarachnoid haemorrhage as the first clinical manifestation of Churg-Strauss syndrome. J Neurol 2008; 255:607.
  35. Clutterbuck EJ, Evans DJ, Pusey CD. Renal involvement in Churg-Strauss syndrome. Nephrol Dial Transplant 1990; 5:161.
  36. Sinico RA, Di Toma L, Maggiore U, et al. Renal involvement in Churg-Strauss syndrome. Am J Kidney Dis 2006; 47:770.
  37. Churg A. Recent advances in the diagnosis of Churg-Strauss syndrome. Mod Pathol 2001; 14:1284.
  38. Noth I, Strek ME, Leff AR. Churg-Strauss syndrome. Lancet 2003; 361:587.
  39. 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.
  40. Schmitt WH, Csernok E, Kobayashi S, et al. Churg-Strauss syndrome: serum markers of lymphocyte activation and endothelial damage. Arthritis Rheum 1998; 41:445.
  41. Reid AJ, Harrison BD, Watts RA, et al. Churg-Strauss syndrome in a district hospital. QJM 1998; 91:219.
  42. Sablé-Fourtassou R, Cohen P, Mahr A, et al. Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Intern Med 2005; 143:632.
  43. Nathani N, Little MA, Kunst H, et al. Churg-Strauss syndrome and leukotriene antagonist use: a respiratory perspective. Thorax 2008; 63:883.
  44. Keogh KA, Specks U. Churg-Strauss syndrome: clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists. Am J Med 2003; 115:284.
  45. Sinico RA, Di Toma L, Maggiore U, et al. Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome. Arthritis Rheum 2005; 52:2926.
  46. Sidhu B, Nanda U, Abbas S. Is this an exacerbation of asthma? A cautionary tale. BMJ Case Rep 2013; 2013.
  47. McAleavey N, Millar A, Pendleton A. Cardiac involvement as the main presenting feature in eosinophilic granulomatosis with polyangiitis. BMJ Case Rep 2013; 2013.
  48. Hara T, Yamaguchi K, Iwase T, et al. Eosinophilic myocarditis due to Churg-Strauss syndrome with markedly elevated eosinophil cationic protein. Int Heart J 2013; 54:51.
  49. Kakouros N, Bastiaenen R, Kourliouros A, Anderson L. Churg-Strauss presenting as acute coronary syndrome: sometimes it's zebras. BMJ Case Rep 2011; 2011.
  50. Choi YH, Im JG, Han BK, et al. Thoracic manifestation of Churg-Strauss syndrome: radiologic and clinical findings. Chest 2000; 117:117.
  51. Erzurum SC, Underwood GA, Hamilos DL, Waldron JA. Pleural effusion in Churg-Strauss syndrome. Chest 1989; 95:1357.
  52. Buschman DL, Waldron JA Jr, King TE Jr. Churg-Strauss pulmonary vasculitis. High-resolution computed tomography scanning and pathologic findings. Am Rev Respir Dis 1990; 142:458.
  53. Worthy SA, Müller NL, Hansell DM, Flower CD. Churg-Strauss syndrome: the spectrum of pulmonary CT findings in 17 patients. AJR Am J Roentgenol 1998; 170:297.
  54. Allen JN, Davis WB. Eosinophilic lung diseases. Am J Respir Crit Care Med 1994; 150:1423.
  55. Sauvetre G, Fares J, Caudron J, et al. [Usefulness of magnetic resonance imaging in Churg-Strauss syndrome related cardiac involvement. A case series of three patients and literature review]. Rev Med Interne 2010; 31:600.
  56. Marmursztejn J, Vignaux O, Cohen P, et al. Impact of cardiac magnetic resonance imaging for assessment of Churg-Strauss syndrome: a cross-sectional study in 20 patients. Clin Exp Rheumatol 2009; 27:S70.
  57. Baccouche H, Yilmaz A, Alscher D, et al. Images in cardiovascular medicine. Magnetic resonance assessment and therapy monitoring of cardiac involvement in Churg-Strauss syndrome. Circulation 2008; 117:1745.
  58. Mavrogeni S, Manoussakis MN, Karagiorga TC, et al. Detection of coronary artery lesions and myocardial necrosis by magnetic resonance in systemic necrotizing vasculitides. Arthritis Rheum 2009; 61:1121.
  59. Mavrogeni S, Karabela G, Gialafos E, et al. Cardiac involvement in ANCA (+) and ANCA (-) Churg-Strauss syndrome evaluated by cardiovascular magnetic resonance. Inflamm Allergy Drug Targets 2013; 12:322.
  60. Miszalski-Jamka T, Szczeklik W, Sokołowska B, et al. Standard and feature tracking magnetic resonance evidence of myocardial involvement in Churg-Strauss syndrome and granulomatosis with polyangiitis (Wegener's) in patients with normal electrocardiograms and transthoracic echocardiography. Int J Cardiovasc Imaging 2013; 29:843.
  61. Marmursztejn J, Guillevin L, Trebossen R, et al. Churg-Strauss syndrome cardiac involvement evaluated by cardiac magnetic resonance imaging and positron-emission tomography: a prospective study on 20 patients. Rheumatology (Oxford) 2013; 52:642.
  62. Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 1994; 37:187.
  63. 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.
  64. Steinfeld S, Golstein M, De Vuyst P. Chronic eosinophilic pneumonia (CEP) as a presenting feature of Churg-Strauss syndrome (CSS). Eur Respir J 1994; 7:2098.