RESUMEN

La granulomatosis eosinofílica con poliangitis (GEPA), anteriormente denominado síndrome de Churg-Strauss (SCS), es un trastorno poco frecuente, de etiología desconocida, caracterizada por una vasculitis necrosante que afecta a vasos de pequeño a mediano calibre. Los pacientes presentan como rasgo característico antecedentes de asma, rinitis alérgica y eosinofilia en sangre periférica. La GEPA, antes denominado SCS es poco común en la infancia. Aunque la GEPA es una vasculitis asociada a la presencia de anticuerpos anticitoplasma de neutrófilo (ANCA), en niños sólo se encuentran en menos del 40% de los casos. La detección temprana de la enfermedad es importante ya que un retraso en el diagnóstico puede llevar a la afectación orgánica de grado variable con resultados fatales. La GEPA de inicio en la infancia representa menos del 2% de los casos de vasculitis en edad pediátrica, y sólo 50 casos de GEPA en menores de 18 años se habían publicado hasta el año 2013. Presentamos el caso de un niño de 13 años de edad con asma de difícil control y deterioro de su estado general, que finalmente fue diagnosticado con GEPA-ANCA negativo.

PALABRAS CLAVE

Granulomatosis eosinofílica con poliangitis, GEPA, asma, Churg-Strauss, vasculitis.

REFERENCIAS

1. 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.

2. Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 1951;27(2):277-301.

3. Mukhtyar C, Guillevin L, Cid MC, et al.; European Vasculitis Study Group. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis 2009;68(3):310-317. doi: 10.1136/ard.2008.088096.

4. Comarmond C, Pagnoux C, Khellaf M, et al.; French Vasculitis Study Group. 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):270-281. doi: 10.1002/art.37721.

5. 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):2926-2935.

6. Sablé-Fourtassou R, Cohen P, Mahr A, et al.; French Vasculitis Study Group. Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Intern Med 2005;143(9):632-638.

7. Zwerina J, Eger G, Englbrecht M, Manger B, Schett G. Churg-Strauss syndrome in childhood: a systematic literature review and clinical comparison with adult patients. Semin Arthritis Rheum 2009;39(2):108-15. doi: 10.1016/j.semarthrit.2008.05.004.

8. 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):571-576.e1-6. doi: 10.1016/j.jaci.2012.05.058.

9. Keogh KA, Specks U. Churg–Strauss syndrome: clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists. Am J Med 2003; 115(4):284-290.

10. Klion AD, Bochner BS, Gleich GJ, et al.; The Hypereosinophilic Syndromes Working Group. Approaches to the treatment of hypereosinophilic syndromes: a workshop summary report. J Allergy Clin Immunol 2006;117(6):1292-1302.

11. Tomac N, Yuksek M, Kunak B, Ertan U, Igde M. Churg-Strauss syndrome: a patient report in infancy. Clin Pediatr (Philadelphia) 2003;42(4):367-370.

12. Haugeberg G, Bie R, Bendvold A, Larsen AS, Johnsen V. Primary vasculitis in a Norwegian community hospital: a retrospective study. Clin Rheumatol 1998;17(5):364-368.

13. Mahr A, Guillevin L, Poissonnet M, Aymé S. Prevalences of polyarteritis nodosa, microscopic polyangiitis, Wegener’s granulomatosis, and Churg–Strauss syndrome in a French urban multiethnic population in 2000: a capture–recapture estimate. Arthritis Rheum 2004;51(1):92-99.

14. Mohammad AJ, Jacobsson LT, Mahr AD, Sturfelt G, Segelmark M. Prevalence of Wegener’s granulomatosis, microscopic polyangiitis, polyarteritis nodosa and Churg-Strauss syndrome within a defined population in southern Sweden. Rheumatology (Oxford) 2007;46(8):1329-1337.

15. Watts RA, Lane S, Scott DG. What is known about the epidemiology of the vasculitides? Best Pract Res Clin Rheumatol 2005;19(2):191-207.

16. Herlyn K, Hellmich B, Gross WL, Reinhold-Keller E. Stable incidence of systemic vasculitides in Schleswig-Holstein, Germany. Dtsch Arztebl Int 2008;105(19):355-361. doi: 10.3238/arztebl.2008.0355.

17. 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(3):179-189.

18. 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(6):1076-1080.

19. 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):1011-1017. doi: 10.1136/annrheumdis-2012-201531.

20. Roufosse F, Weller PF. Practical approach to the patient with hypereosinophilia. J Allergy Clin Immunol 2010;126(1):39-44. doi: 10.1016/j.jaci.2010.04.011.

21. Zwerina J, Strehl JD, Beyer C, Schett G. Can ANCA differentiate eosinophilic granulomatosis with polyangiitis (Churg-Strauss) from idiopathic hypereosinophilic syndrome? Clin Exp Rheumatol 2013;31(6):989-990.

22. Taylor MR, Keane CT, O’Connor P, Mulvihill E, Holland C. The expanded spectrum of toxocaral disease. Lancet 1988;1(8587):692-695.

23. Siddiqui AA, Berk SL. Diagnosis of Strongyloides stercoralis infection. Clin Infect Dis 2001;33(7):1040-1047.

24. Agarwal R. Allergic bronchopulmonary aspergillosis. Chest 2009;135(3):805-826. doi: 10.1378/chest.08-2586.

25. Cogan E, Schandené L, Crusiaux A, Cochaux P, Velu T, Goldman M. Brief report: clonal proliferation of type 2 helper T cells in a man with the hypereosinophilic syndrome. N Engl J Med 1994;330(8):535-538.

26. de Lavareille A, Roufosse F, Schmid-Grendelmeier P, et al. High serum thymus and activation-regulated chemokine levels in the lymphocytic variant of the hypereosinophilic syndrome. J Allergy Clin Immunol 2002;110(3):476-479.

27. Ogbogu PU, Bochner BS, Butterfield JH, et al. Hypereosinophilic syndrome: a multicenter, retrospective analysis of clinical characteristics and response to therapy. J Allergy Clin Immunol 2009;124(6):1319-1325. e3. doi: 10.1016/j.jaci.2009.09.022.

28. Legrand F, Renneville A, Macintyre E, et al.; on behalf of the French Eosinophil Network. The spectrum of FIP1L1–PDGFRA-associated chronic eosinophilic leukemia: new insights based on a survey of 44 cases. Medicine (Baltimore) 2013; 92(5): e1-e9.

29. Marchand E, Reynaud-Gaubert M, Lauque D, Durieu J, Tonnel AB, Cordier JF. Idiopathic chronic eosinophilic pneumonia. A clinical and follow-up study of 62 cases. The Groupe d’Études et de Recherche sur les Maladies “Orphelines” Pulmonaires (GERM“O”P). Medicine (Baltimore) 1998;77(5):299-312.

30. Choi HK, Liu S, Merkel PA, Colditz GA, Niles JL. Diagnostic performance of antineutrophil cytoplasmic antibody tests for idiopathic vasculitides: metaanalysis with a focus on antimyeloperoxidase antibodies. J Rheumatol 2001;28(7):1584-1590.

31. Cordier JF, Cottin V, Guillevin L, et al. L5. Eosinophilic granulomatosis with polyangiitis (Churg–Strauss). Presse Med 2013;42(4 Pt 2):507-510. doi: 10.1016/j.lpm.2013.02.308.

32. Groh M, Masciocco G, Kirchner E, et al. Heart transplantation in patients with eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome). J Heart Lung Transplant 2014;33(8):842-850. doi: 10.1016/j.healun.2014.02.023

33. Samson M, Puéchal X, Devilliers H, et al.; French Vasculitis Study Group. Long-term outcomes of 118 patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) enrolled in two prospective trials. J Autoimmun 2013; 43:60-69. doi: 10.1016/j.jaut.2013.03.003

34. Grayson PC, Monach PA, Pagnoux C, et al.; Vasculitis Clinical Research Consortium. Value of commonly measured laboratory tests as biomarkers of disease activity and predictors of relapse in eosinophilic granulomatosis with polyangiitis. Rheumatology (Oxford). 2015;54(8):1351-1359. doi: 10.1093/rheumatology/keu427.

35. Guilpain P, Auclair J-F, Tamby MC, et al. Serum eosinophil cationic protein: a marker of disease activity in Churg–Strauss syndrome. Ann N Y Acad Sci 2007; 1107:392-399.

36. Hurst S, Chizzolini C, Dayer JM, Olivieri J, Roux-Lombard P. Usefulness of serum eosinophil cationic protein (ECP) in predicting relapse of Churg and Strauss vasculitis. Clin Exp Rheumatol 2000;18(6):784-785.

37. Herrmann K, Gross WL, Moosig F. Extended follow-up after stopping mepolizumab in relapsing/refractory Churg–Strauss syndrome. Clin Exp Rheumatol 2012;30(1 Suppl 70):S62-S65.

38. De Vries M, Drent M, Cohen TJ-W. Vasculitis induced by drugs. In: Camus P, Rosenow EC, editors. Drug-induced and iatrogenic respiratory disease. London: Hodder Education; 2010. p. 308-317.

39. Boyer D, Vargas SO, Slattery D, Rivera-Sanchez YM, Colin AA. Churg-Strauss syndrome in children: a clinical and pathologic review. Pediatrics 2006;118(3):e914-e920.

40. Zwerina J, Bach C, Martorana D, et al. Eotaxin-3 in Churg–Strauss syndrome: a clinical and immunogenetic study. Rheumatology (Oxford) 2011;50(10):1823-1827. doi: 10.1093/rheumatology/keq445.

41. Choi YH, Im JG, Han BK, Kim JH, Lee KY, Myoung NH. Thoracic Manifestation of Churg-Strauss syndrome: radiologic and clinical findings. Chest 2000;117(1):117-124.

42. Lu S, Zhou WY, Zhou W, et al. Churg-Strauss syndrome in children: a case evolving in endomyocardiopathy. Zhongguo Dang Dai Er Ke Za Zhi 2008;10(5):625-628.

43. Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Le Toumelin P.; French Vasculitis Study Group (FVSG). 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.

44. Razenberg FG, Heynens JW, Jan de Vries G, et al. Clinical presentation of Churg-Strauss syndrome in children: A 12-year-old-boy with ANCA-negative Churg- Strauss syndrome. Respir Med Case Rep 2012;7:4-7. doi: 10.1016/j.rmcr.2012.09.004.

45. Groh M, Pagnoux C, Baldini C, et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA). Consensus Task Force recommendations for evaluation and management. Eur J Intern Med 2015;26(7):545-553. doi: 10.1016/j.ejim.2015.04.022.

46. Bouldouyre MA, Cohen P, Guillevin L. Severe bronchospasm associated with rituximab for refractory Churg–Strauss syndrome. Ann Rheum Dis 2009;68(4):606. doi: 10.1136/ard.2008.093773.

47. Pepper RJ, Fabre MA, Pavesio C, et al. Rituximab is effective in the treatment of refractory Churg-Strauss syndrome and is associated with diminished T-cell interleukin-5 production. Rheumatology (Oxford) 2008;47(7):1104-1105. doi: 10.1093/rheumatology/ken175.

48. 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(6):929-935. doi: 10.3899/jrheum.120808.

49. 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(2):65-81.