Estridor, punta del iceberg en granulomatosis con poliangeítis: presentación de un caso
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jun 17, 2024
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Greis Patricia Espitia Caro
https://orcid.org/0000-0002-6332-661X
Myriam Lorena Martín M.
https://orcid.org/0000-0002-4770-6882
Felipe Aluja Jaramillo
https://orcid.org/0000-0002-3093-2509
María Camila Villegas
https://orcid.org/0000-0002-8921-8764
https://orcid.org/0000-0002-6332-661X
Myriam Lorena Martín M.
https://orcid.org/0000-0002-4770-6882
Felipe Aluja Jaramillo
https://orcid.org/0000-0002-3093-2509
María Camila Villegas
https://orcid.org/0000-0002-8921-8764
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Resumen
Introducción: La granulomatosis con poliangeítis es una vasculitis autoinmune, necrosante y granulomatosa asociada con la presencia de anticuerpos anticitoplasma de neutrófilo. Afecta vasos de pequeño calibre y afecta especialmente la vía área superior e inferior, y en menor frecuencia otros órganos como el riñón. Objetivo: Reseñar el caso de una paciente pediátrica de 10 años de edad con granulomatosis con poliangeítis y su sustancial respuestas clínica y paraclínica luego del tratamiento. Presentación del caso: paciente con un cuadro de 6 meses de estridor, tos seca y disnea de grandes esfuerzos, manejada con corticoide sistémico y nebulizado. A partir de los estudios realizados se investigó el diagnóstico de granulomatosis con poliangeítis, cuyo resultado de anticuerpos anticitoplasma de neutrófilo fue positivo por inmunofluorescencia: p-ANCA 1:320, ELISA: antimieloperoxidasa positivo. Ello confirmo la sospecha diagnóstica. Conclusión: la granulomatosis con poliangeítis es una rara enfermedad en pediatría, y se requiere una alta sospecha diagnóstica y un tratamiento temprano para mejorar el pronóstico de los pacientes.
Keywords
Granulomatosis con poliangeítis, estridor, estenosis subglótica, Vasculitis autoinmune
References
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16. Monteiro M, Domingos R, Rocha S, Miranda I. Granulomatosis with polyangiitis: the complexity of clinical manifestations, therapeutic challenges, and complications of a severe multisystemic case. Cureus. 2023;15(10):e47031. https://doi.org/10.7759/cureus.47031
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18. Hirano D, Ishikawa T, Inaba A, Sato M, Shinozaki T, Iijima K, et al. Epidemiology and clinical features of childhood-onset anti-neutrophil cytoplasmic antibody-associated vasculitis: a clinicopathological analysis. Pediatr Nephrol. 2019;34(8):1425-33. https://doi.org/10.1007/s00467-019-04228-4
19. Higgins GC. Complications of treatments for pediatric rheumatic diseases. Pediatr Clin North Am. 2018;65(4):827-54. https://doi.org/10.1016/j.pcl.2018.04.008
2. Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, et al. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis. 2010;69(5):798-806. https://doi.org/10.1136/ard.2009.116657
3. Jariwala M, Laxer RM. Childhood GPA, EGPA, and MPA. Clin Immunol. 2020;211:108325. https://doi.org/10.1016/j.clim.2019.108325
4. Filocamo G, Torreggiani S, Agostoni C, Esposito S. Lung involvement in childhood onset granulomatosis with polyangiitis. Pediatr Rheumatol. 2017;15(1):1-9. https://doi.org/10.1186/s12969-017-0150-8
5. Ludici M, Quartier P, Terrier B, Mouthon L, Guillevin L, Puéchal X. Childhood-onset granulomatosis with polyangiitis and microscopic polyangiitis: systematic review and meta-analysis. Orphanet J Rare Dis. 2016;11(1):1-12. http://dx.doi.org/10.1186/s13023-016-0523-y
6. Herrera CN, Barreno V ME, Quintana Vega VJ, Suquilanda Feria DB. Hipoacusia y vasculitis cutánea, manifestación inicial de granulomatosis con poliangeítis en una niña de 14 años. Rev Colomb Reumatol. 2019;26(3):216-9. https://doi.org/10.1016/j.rcreu.2018.09.002
7. Ludici M, Puéchal X, Pagnoux C, Quartier P, Agard C, Aouba A, et al. Brief report: childhood-onset systemic necrotizing vasculitides. Long-term data from the French vasculitis study group registry. Arthritis Rheumatol. 2015;67(7):1959-65. https://doi.org/10.1002/art.39122
8. Comarmond C, Cacoub P. Granulomatosis with polyangiitis (Wegener): clinical aspects and treatment. Autoimmun Rev. 2014;13(11):1121-5. https://doi.org/10.1016/j.autrev.2014.08.017
9. Greco A, Marinelli C, Fusconi M, Macri GF, Gallo A, de Virgilio A, et al. Clinic manifestations in granulomatosis with polyangiitis. Int J Immunopathol Pharmacol. 2016;29(2):151-9. https://doi.org/10.1177/0394632015617063
10. Sullivan KE. Pathogenesis of pediatric rheumatologic diseases. Pediatr Clin North Am. 2018;65(4):639-55. https://doi.org/10.1016/j.pcl.2018.03.004
11. James KE, Xiao R, Merkel PA, Weiss PF. Clinical course and outcomes of childhood-onset granulomatosis with polyangiitis. Clin Exp Rheumatol [internet]. 2017;35(1):202-8. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5382123/
12. Jariwala MP, Laxer RM. Primary vasculitis in childhood: GPA and MPA in childhood. Front Pediatr. 2018;6(August):1-11. https://doi.org/10.3389/fped.2018.00226
13. Peraza Labrador AJ, Valdez LHM, González Marín NR, Ibazetta KAR, Chacón JAL, Fernández AJV, et al. Oral granulomatosis with polyangiitis a systematic review. Clin Exp Dent Res. 2023;9:100-11. https://doi.org/10.1002/cre2.706
14. De Graeff N, Groot N, Brogan P, Ozen S, Avcin T, Bader-Meunier B, et al. European consensus-based recommendations for the diagnosis and treatment of rare paediatric vasculitides-the SHARE initiative. Rheumatology (United Kingdom). 2019;58(4):656-71. https://doi.org/10.1093/rheumatology/key322
15. Robson JC, Grayson PC, Ponte C, Suppiah R, Craven A, Judge A, et al. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis. Ann Rheum Dis. 2022 Mar;81(3):315-20. https://doi.org/10.1136/annrheumdis-2021-221795
16. Monteiro M, Domingos R, Rocha S, Miranda I. Granulomatosis with polyangiitis: the complexity of clinical manifestations, therapeutic challenges, and complications of a severe multisystemic case. Cureus. 2023;15(10):e47031. https://doi.org/10.7759/cureus.47031
17. Sacri AS, Chambaraud T, Ranchin B, Florkin B, Sée H, Decramer S, et al. Clinical characteristics and outcomes of childhood-onset ANCA-associated vasculitis: a French nationwide study. Nephrol Dial Transplant. 2015;30(February):i104-12. https://doi.org/10.1093/ndt/gfv011
18. Hirano D, Ishikawa T, Inaba A, Sato M, Shinozaki T, Iijima K, et al. Epidemiology and clinical features of childhood-onset anti-neutrophil cytoplasmic antibody-associated vasculitis: a clinicopathological analysis. Pediatr Nephrol. 2019;34(8):1425-33. https://doi.org/10.1007/s00467-019-04228-4
19. Higgins GC. Complications of treatments for pediatric rheumatic diseases. Pediatr Clin North Am. 2018;65(4):827-54. https://doi.org/10.1016/j.pcl.2018.04.008
Cómo citar
Espitia Caro, G. P., Martín M., M. L., Aluja Jaramillo, F., & Villegas, M. C. (2024). Estridor, punta del iceberg en granulomatosis con poliangeítis: presentación de un caso. Universitas Medica, 64(3). https://doi.org/10.11144/Javeriana.umed64-3.iceb
Número
Sección
Suplemento Especial de Pedriatría
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