Estridor, punta del iceberg en granulomatosis con poliangeítis: presentación de un caso
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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Apresentação do caso: Adolescente do sexo feminino, 10 anos, com 6 meses de estridor, tosse seca e dispneia aos esforços. Tratada repetidamente com corticosteroides sistêmicos e nebulizados. Nos últimos 2 anos, ela apresentou rinorreia espessa e crostosa, halitose, epistaxe e 8 episódios de otite média aguda.
O estudo do estridor começou com uma nasofibrolaringoscopia com suspeita de laringomalácia tipo 3 (deslocamento posterior da epiglote), seguida de um exame das vias aéreas com broncoscopia rígida, que revelou estenose subglótica grau II (obstrução das vias aéreas entre 51% e 70%). A tomografia computadorizada do pescoço mostrou uma diminuição de 72% no diâmetro transversal da traqueia devido à estenose subglótica e nódulos pulmonares disseminados no tórax. Portanto, o diagnóstico de granulomatose com poliangiite foi investigado, o resultado dos anticorpos citoplasmáticos anti-neutrófilos foi positivo por imunofluorescência: p-ANCA 1:320, ELISA: anti-mieloperoxidase positivo; o que confirmou a suspeita diagnóstica. O tratamento imunossupressor foi iniciado com esteroide e ciclofosfamida mensalmente por 6 doses, com resposta clínica adequada, redução da estenose subglótica e resolução dos nódulos pulmonares, e manutenção com metotrexato.
Conclusão: A granulomatose com poliangiite é uma doença rara em pediatria, sendo necessária alta suspeita diagnóstica e tratamento precoce para melhorar o prognóstico dos pacientes.
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