{"id":10569,"date":"2018-09-13T17:51:19","date_gmt":"2018-09-13T17:51:19","guid":{"rendered":"https:\/\/sbpt.org.br\/portal\/?p=10569"},"modified":"2026-03-04T22:14:27","modified_gmt":"2026-03-05T01:14:27","slug":"desafios-no-manejo-da-fibrose-cistica-em-adultos","status":"publish","type":"post","link":"https:\/\/sbpt.org.br\/portal\/desafios-no-manejo-da-fibrose-cistica-em-adultos\/","title":{"rendered":"Desafios no manejo da fibrose c\u00edstica em adultos"},"content":{"rendered":"<p><strong>Desafios no manejo da fibrose c\u00edstica em adultos<\/strong><\/p>\n<p>Autora: Samia Zahi Rached<\/p>\n<p>M\u00e9dica da Divis\u00e3o de Pneumologia do Instituto do Cora\u00e7\u00e3o do Hospital das Cl\u00ednicas da Faculdade de Medicina da Universidade de S\u00e3o Paulo<\/p>\n<p>Caso Cl\u00ednico:<\/p>\n<p>Paciente 21 anos, sexo masculino, casado, desempregado, natural e procedente de S\u00e3o Paulo<\/p>\n<p>Queixa Principal: Exacerba\u00e7\u00f5es pulmonares frequentes<\/p>\n<p>Hist\u00f3ria Mol\u00e9stia Atual:<\/p>\n<ul>\n<li>Paciente portador de Fibrose C\u00edstica (FC), refere que nos \u00faltimos 6 meses apresentou 4 exacerba\u00e7\u00f5es, sendo 1 interna\u00e7\u00e3o.<\/li>\n<li>Exacerba\u00e7\u00f5es caracterizadas por piora da tosse e aumento da expectora\u00e7\u00e3o, com colora\u00e7\u00e3o amarela \/ esverdeada. Piora da dispneia associada com alguns epis\u00f3dios de chiado.<\/li>\n<li>Entre crises, mantendo mMRC 0 e tosse produtiva em moderada quantidade. Perdeu 2 kg em 6 meses.<\/li>\n<\/ul>\n<p>Antecedentes Pessoais:<\/p>\n<ul>\n<li>Diagn\u00f3stico de fibrose c\u00edstica (FC) ao nascimento (muta\u00e7\u00f5es F508del e S4X), transferido da equipe pedi\u00e1trica aos 18 anos.<\/li>\n<li>Acometimento pulmonar, sinusopatia, insufici\u00eancia pancre\u00e1tica ex\u00f3crina<\/li>\n<li>Infec\u00e7\u00e3o cr\u00f4nica das vias a\u00e9reas por <em>Pseud\u00f4monas aeruginosa<\/em> e <em>Staphylococcus aureus<\/em> sens\u00edvel \u00e0 meticilina .<\/li>\n<li>Tratou Aspergilose broncopulmonar al\u00e9rgica (ABPA) dos 17 aos 18 anos com prednisona.<\/li>\n<li>Epilepsia<\/li>\n<li>Nega tabagismo, etilismo ou uso de drogas il\u00edcitas<\/li>\n<\/ul>\n<p>Medica\u00e7\u00f5es em uso: dornase alfa, tobramicina inalat\u00f3ria em meses alternados, enzimas pancre\u00e1ticas, vitaminas ADEK, suplementos nutricionais. Faz higiene br\u00f4nquica diariamente.<\/p>\n<p>Exame F\u00edsico: Bom estado geral, corado, hidratado, eupneico e afebril.<\/p>\n<p>PA = 110x60mmHg FC = 96bpm FR = 30ipm SpO2 = 96% &nbsp;em ar ambiente, Peso 54 kg, altura 1,57m, IMC 21,9 kg\/m<sup>2<\/sup><br \/>\nMurm\u00fario vesicular presente bilateralmente com roncos &nbsp;e estertores crepitantes difusos.<br \/>\nBulhas card\u00edacas r\u00edtmicas, normofon\u00e9ticas, sem sopros.<br \/>\nAbdome fl\u00e1cido, escavado, indolor a palpa\u00e7\u00e3o, sem visceromegalias palp\u00e1veis. Membros inferiores sem edema ou empastamento de panturrilhas.<br \/>\nPresen\u00e7a de baqueteamento digital.<\/p>\n<table style=\"width: 100%;\" border=\"1\" cellspacing=\"1\" cellpadding=\"1\" align=\"center\">\n<tbody>\n<tr>\n<td width=\"92\"><strong>&nbsp;<\/strong><\/td>\n<td width=\"142\"><strong>CVF (%predito)<\/strong><\/td>\n<td width=\"198\"><strong>VEF1 (% predito)<\/strong><\/td>\n<td width=\"149\"><strong>VEF1\/CVF<\/strong><\/td>\n<\/tr>\n<tr>\n<td width=\"92\">&nbsp;Out\/ 2014<\/td>\n<td width=\"142\">2,65 (74%)<\/td>\n<td width=\"198\">2,36 (70%)<\/td>\n<td width=\"149\">0,89<\/td>\n<\/tr>\n<tr>\n<td width=\"92\">Jun\/ 2015<\/td>\n<td width=\"142\">1,78 (48%)<\/td>\n<td width=\"198\">1,54(45%)<\/td>\n<td width=\"149\">0,87<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<table style=\"width: 600px;\" border=\"0\" cellspacing=\"0\" cellpadding=\"0\" align=\"center\">\n<tbody>\n<tr>\n<td style=\"text-align: center;\"><a href=\"https:\/\/d1xe7tfg0uwul9.cloudfront.net\/sbpt-portal\/wp-content\/uploads\/2018\/09\/figura-1-julho-1.jpg\"><img loading=\"lazy\" decoding=\"async\" class=\"wp-image-10571 alignleft\" src=\"https:\/\/d1xe7tfg0uwul9.cloudfront.net\/sbpt-portal\/wp-content\/uploads\/2018\/09\/figura-1-julho-1.jpg\" alt=\"\" width=\"548\" height=\"548\" srcset=\"https:\/\/d1xe7tfg0uwul9.cloudfront.net\/sbpt-portal\/wp-content\/uploads\/2018\/09\/figura-1-julho-1.jpg 800w, https:\/\/d1xe7tfg0uwul9.cloudfront.net\/sbpt-portal\/wp-content\/uploads\/2018\/09\/figura-1-julho-1-300x300.jpg 300w, https:\/\/d1xe7tfg0uwul9.cloudfront.net\/sbpt-portal\/wp-content\/uploads\/2018\/09\/figura-1-julho-1-150x150.jpg 150w, https:\/\/d1xe7tfg0uwul9.cloudfront.net\/sbpt-portal\/wp-content\/uploads\/2018\/09\/figura-1-julho-1-768x768.jpg 768w\" sizes=\"auto, (max-width: 548px) 100vw, 548px\" \/><\/a><\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<p><strong>Quest\u00e3o 1:&nbsp;<\/strong>Considerando a hist\u00f3ria cl\u00ednica, exame f\u00edsico e altera\u00e7\u00f5es funcionais e tomogr\u00e1ficas, qual a principal hip\u00f3tese diagn\u00f3stica?<\/p>\n<ol>\n<li>Progress\u00e3o de doen\u00e7a<\/li>\n<li>Micobacteriose at\u00edpica<\/li>\n<li>Desnutri\u00e7\u00e3o<\/li>\n<li>Aspergilose broncopulmonar al\u00e9rgica<\/li>\n<\/ol>\n<p><strong>Coment\u00e1rio\/justificativa: <\/strong><\/p>\n<p>Diante de exacerba\u00e7\u00f5es freq\u00fcentes&nbsp; em paciente com FC devemos sempre investigar por poss\u00edveis novas infec\u00e7\u00f5es, ABPA, diabetes, desnutri\u00e7\u00e3o, m\u00e1 ades\u00e3o, entre outros.<\/p>\n<p>A ABPA tem alta preval\u00eancia em FC e cursa com quadro cl\u00ednico de chiado, dispn\u00e9ia e exacerba\u00e7\u00f5es. Considerando o quadro cl\u00ednico de nosso paciente e o passado recente de ABPA, devemos iniciar nossa investiga\u00e7\u00e3o por esta hip\u00f3tese solicitando inicialmente nova dosagem de Imunoglobulina (Ig) E.<\/p>\n<table style=\"width: 100%;\" border=\"1\" cellspacing=\"1\" cellpadding=\"1\" align=\"center\">\n<tbody>\n<tr>\n<td width=\"576\"><strong>ABPA em FIbrose C\u00edstica<\/strong><\/td>\n<\/tr>\n<tr>\n<td width=\"576\"><strong>Crit\u00e9rio Diagn\u00f3stico Cl\u00e1ssico: <\/strong><\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<p><strong>&nbsp;\u2013 Deteriora\u00e7\u00e3o cl\u00ednica<\/strong><\/p>\n<p><strong>&nbsp;\u2013 RAST ou prick test positivo <\/strong><\/p>\n<p><strong>\u2013 IgE &gt; 1000 kU\/I <\/strong><\/p>\n<p><strong>\u2013 Sorologia ou preceptinas positivas <\/strong><\/p>\n<p><strong>\u2013 Rx de t\u00f3rax alterado<\/strong><strong>Rastreamento: dosagem de IgE anual<\/strong><\/p>\n<p><strong>\u2013 IgE &gt; 500 kU\/I:&nbsp; RAST ou prick test <\/strong><\/p>\n<p><strong>\u2013 IgE &lt; 500 kU\/I: repetir IgE a depender da cl\u00ednica<\/strong><\/p>\n<p><strong>SEGUIMENTO DO CASO: <\/strong><\/p>\n<p>INVESTIGA\u00c7\u00c3O REALIZADA PARA ABPA:<\/p>\n<table style=\"width: 100%;\" border=\"1\" width=\"0\" cellspacing=\"1\" cellpadding=\"1\" align=\"center\">\n<tbody>\n<tr>\n<td width=\"208\"><strong>Crit\u00e9rios para ABPA<\/strong><\/td>\n<td width=\"300\"><strong>&nbsp;<\/strong><\/td>\n<\/tr>\n<tr>\n<td width=\"208\">Imunoglobulina E<\/td>\n<td width=\"300\">828 UI\/ mL (dosagem h\u00e1 10 meses: 269 UI\/mL)<\/td>\n<\/tr>\n<tr>\n<td width=\"208\">Eosin\u00f3filos<\/td>\n<td width=\"300\">625 \/mm3<\/td>\n<\/tr>\n<tr>\n<td width=\"208\">Cultura de escarro<\/td>\n<td width=\"300\">Positiva para <em>Aspergillus fumigatus<\/em><\/td>\n<\/tr>\n<tr>\n<td width=\"208\">IgE espec\u00edfica para Aspergillus<\/td>\n<td width=\"300\">Classe 3 (alto)<\/td>\n<\/tr>\n<tr>\n<td width=\"208\">Sorologia para Aspergillus<\/td>\n<td width=\"300\">Positiva<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<p><strong>Quest\u00e3o 2:<\/strong> Qual a principal medica\u00e7\u00e3o a ser consideradas no tratamento da ABPA?<\/p>\n<ol>\n<li>a) Itraconazol<\/li>\n<li>b) Corticoide inalat\u00f3rio<\/li>\n<li>c) Prednisona oral<\/li>\n<li>d) Omalizumabe<\/li>\n<li>e) Anfotericina inalat\u00f3ria<\/li>\n<\/ol>\n<p><strong>Coment\u00e1rio\/justificativa: <\/strong><\/p>\n<p>O principal tratamento para ABPA \u00e9 corticoester\u00f3ides. O objetivo \u00e9 suprimir a resposta inflamat\u00f3ria provocada pelo fungo. A Cystic Fibrosis Foundation considera como primeira linha o uso de prednisona 0,5 \u2013 2mg\/kg\/dia por 1 a 2 semanas, com desmame progressivo por 2 a 3 meses.<\/p>\n<p>Diversos estudos avaliaram a associa\u00e7\u00e3o de antif\u00fangico para diminui\u00e7\u00e3o da coloniza\u00e7\u00e3o pelo <em>Aspergillus<\/em>, com redu\u00e7\u00e3o do uso de cortic\u00f3ide. \u00c9 recomendado para pacientes dependentes de corticoester\u00f3ides ou que apresentam relapso. O principal utilizado \u00e9 o itraconazol.<\/p>\n<p>Em casos com resist\u00eancia ao tratamento mesmo com anti f\u00fangico, pode-se considerar terapias adjuvantes como pulsoterapia com corticoester\u00f3ides, corticoide inalat\u00f3rio, omalizumab ou ainda anfotericina inalat\u00f3ria.<\/p>\n<p><strong>SEGUIMENTO DO CASO: <\/strong><\/p>\n<p>Considerado a hip\u00f3tese de aspergilose broncopulmonar al\u00e9rgica (ABPA), iniciado prednisona 40mg\/dia e itraconazol 400mg\/dia com melhora cl\u00ednica e redu\u00e7\u00e3o do n\u00famero de exacerba\u00e7\u00f5es.<\/p>\n<table style=\"width: 100%;\" border=\"1\" width=\"0\" cellspacing=\"1\" cellpadding=\"1\" align=\"center\">\n<tbody>\n<tr>\n<td width=\"155\">&nbsp;<\/td>\n<td width=\"140\">CVF (%predito)<\/td>\n<td width=\"138\">VEF1 (% predito)<\/td>\n<td width=\"134\">CVF\/VEF1<\/td>\n<\/tr>\n<tr>\n<td width=\"155\">DIAGN\u00d3STICO DE ABPA<\/td>\n<td width=\"140\">1,78 (48%)<\/td>\n<td width=\"138\">1,54(45%)<\/td>\n<td width=\"134\">0,87<\/td>\n<\/tr>\n<tr>\n<td width=\"155\">Ap\u00f3s 6 meses de tratamento<\/td>\n<td width=\"140\">2,53 (66%)<\/td>\n<td width=\"138\">2,51 (71%)<\/td>\n<td width=\"134\">0,99<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<p>Ap\u00f3s 8 meses de tratamento do in\u00edcio do tratamento da ABPA, cultura de vigil\u00e2ncia de escarro revelou crescimento de <em>Mycobacterium abscessus<\/em>. Paciente apresentava-se est\u00e1vel, sem novas queixas cl\u00ednicas. Repetida fun\u00e7\u00e3o pulmonar com nova queda dos volumes pulmonares.<\/p>\n<table style=\"width: 100%;\" border=\"1\" width=\"0\" cellspacing=\"1\" cellpadding=\"1\" align=\"center\">\n<tbody>\n<tr>\n<td width=\"139\">&nbsp;<\/td>\n<td width=\"139\">CVF (%predito)<\/td>\n<td width=\"147\">VEF1<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<p>(% predito)CVF\/VEF1Apos 6 meses de tratamento para ABPA2,53 (66%)2,51 (71%)0,99Surgimento de MNT1,40 (36%)1,40 (39%)1,00<\/p>\n<p><strong>Quest\u00e3o 3:<\/strong> Qual a conduta a ser tomada neste momento?<\/p>\n<ol>\n<li>Iniciar tratamento para <em>Mycobacterium abscessus<\/em><\/li>\n<li>Como paciente assintom\u00e1tico, apenas observa\u00e7\u00e3o cl\u00ednica.<\/li>\n<li>Nova tomografia e colher novos escarros para cultura de micobact\u00e9rias.<\/li>\n<li>Encaminhar o paciente para transplante pulmonar.<\/li>\n<\/ol>\n<p><strong>&nbsp;<\/strong><strong>Coment\u00e1rio\/justificativa: <\/strong><\/p>\n<p>Micobacteriose n\u00e3o tuberculosa \u00e9 uma complica\u00e7\u00e3o frequente na FC, e pode ser predisposta por terapia imunossupressora como a prednisona utilizada para a ABPA. O Mycobacterium abscessus \u00e9 potencialmente patog\u00eanico e deve ser investigado. Apesar de nosso paciente n\u00e3o relatar piora cl\u00ednica, houve queda de fun\u00e7\u00e3o pulmonar. A investiga\u00e7\u00e3o deve incluir nova tomografia (imagem abaixo \u2013 houve piora tomogr\u00e1fica neste caso tamb\u00e9m), al\u00e9m de novos escarros. Caso n\u00e3o se confirme com nova amostra de escarro, considerar broncoscopia com lavado broncoalveolar e bi\u00f3psia.<\/p>\n<p>Tomografia ao diagn\u00f3stico da ABPA:<\/p>\n<table style=\"width: 600px;\" border=\"1\" cellspacing=\"1\" cellpadding=\"1\" align=\"center\">\n<tbody>\n<tr>\n<td><a style=\"text-align: center;\" href=\"https:\/\/d1xe7tfg0uwul9.cloudfront.net\/sbpt-portal\/wp-content\/uploads\/2018\/09\/figura-2-julho.jpg\"><img decoding=\"async\" class=\"wp-image-10572 alignleft\" style=\"width: 600px; height: 297px;\" src=\"https:\/\/d1xe7tfg0uwul9.cloudfront.net\/sbpt-portal\/wp-content\/uploads\/2021\/10\/figura-2-julho.png\" alt=\"\"><\/a><\/td>\n<\/tr>\n<tr>\n<td><a style=\"text-align: center;\" href=\"https:\/\/d1xe7tfg0uwul9.cloudfront.net\/sbpt-portal\/wp-content\/uploads\/2018\/09\/figura-3-julho.jpg\"><img decoding=\"async\" class=\"wp-image-10573 alignleft\" style=\"width: 600px; height: 296px;\" src=\"https:\/\/d1xe7tfg0uwul9.cloudfront.net\/sbpt-portal\/wp-content\/uploads\/2021\/10\/figura-3-julho.png\" alt=\"\"><\/a><\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<p style=\"text-align: center;\">Tomografia quando surge a cultura positiva para micobact\u00e9ra:<\/p>\n<table style=\"width: 100%;\" border=\"1\" cellspacing=\"1\" cellpadding=\"1\" align=\"center\">\n<tbody>\n<tr>\n<td width=\"576\"><strong>Crit\u00e9rios diagn\u00f3sticos para Micobact\u00e9rias n\u00e3o tuberculose<\/strong><\/td>\n<\/tr>\n<tr>\n<td width=\"576\">Cl\u00ednicos (obrigat\u00f3rio a presen\u00e7a dos 2 crit\u00e9rios)<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<p>&#8211; Sintomas respirat\u00f3rios, cavidades \/n\u00f3dulos\/ bronquiectasias em radiologia;<\/p>\n<p>&#8211; Exclus\u00e3o adequada de outros diagn\u00f3sticosMicrobiol\u00f3gicos (apenas 1 crit\u00e9rio)<\/p>\n<p>&#8211; Ao menos 2 culturas de escarro positivas;<\/p>\n<p>&#8211; Cultura de lavado broncoalveolar positiva;<\/p>\n<p>&#8211; Cultura de material de bi\u00f3psia positiva<\/p>\n<p><strong>SEGUIMENTO DO CASO: <\/strong><\/p>\n<p>Realizado lavado broncoalveolar por broncoscopia que confirmou o achado de <em>Mycobacterium abscessus<\/em>. Iniciado tratamento ambulatorial com claritromicina, levofloxacino e tobramicina inalada sem sucesso tendo sido optado por antibioticoterapia prolongada em n\u00edvel domiciliar com gentamicina endovenosa, levofloxacino e claritromicina.<\/p>\n<p><strong>Discuss\u00e3o:<\/strong> A popula\u00e7\u00e3o adulta com FC apresenta alguns desafios no seu manejo tais como complica\u00e7\u00f5es mais graves e associa\u00e7\u00f5es de comordidades. Apresentamos um caso com diagn\u00f3stico definido de ABPA e boa resposta terap\u00eautica ao tratamento, por\u00e9m com depend\u00eancia de prednisona em doses altas. Durante sua evolu\u00e7\u00e3o, houve concord\u00e2ncia da piora cl\u00ednica e funcional com a identifica\u00e7\u00e3o de <em>M. abscessus<\/em>. A micobacteriose n\u00e3o tuberculosa \u00e9 uma complica\u00e7\u00e3o frequente na FC, por\u00e9m a terapia imunossupressora associada a altera\u00e7\u00f5es estruturais das vias a\u00e9reas decorrentes da ABPA devem ser considerados fatores predisponentes neste caso. Desta forma, salientamos a import\u00e2ncia do atendimento sistem\u00e1tico do paciente com FC adulto, enfatizando a necessidade de realiza\u00e7\u00e3o de exames de vigil\u00e2ncia assim como aten\u00e7\u00e3o para as poss\u00edveis complica\u00e7\u00f5es inerentes a esta faixa et\u00e1ria e respectivos tratamentos.<\/p>\n<p>1 Griffith, D. E.<em>&nbsp;et al.<\/em>&nbsp;An official ATS\/IDSA statement: diagnosis, treatment, and prevention of nontuberculous mycobacterial diseases.&nbsp;<em>Am J Respir Crit Care Med<\/em>&nbsp;175, 367-416, doi:10.1164\/rccm.200604-571ST (2007).<\/p>\n<p>2 Kousha, M., Tadi, R. &amp; Soubani, A. O. Pulmonary aspergillosis: a clinical review.&nbsp;<em>Eur Respir Rev<\/em>&nbsp;20, 156-174, doi:10.1183\/09059180.00001011 (2011).<\/p>\n<p>3 Stevens, D. A.<em>&nbsp;et al.<\/em>&nbsp;Allergic bronchopulmonary aspergillosis in cystic fibrosis&#8211;state of the art: Cystic Fibrosis Foundation Consensus Conference.&nbsp;<em>Clin Infect Dis<\/em>&nbsp;37 Suppl 3, S225-264, doi:10.1086\/376525 (2003).<\/p>\n<!-- AddThis Advanced Settings generic via filter on the_content --><!-- AddThis Share Buttons generic via filter on the_content -->","protected":false},"excerpt":{"rendered":"<p>Desafios no manejo da fibrose c\u00edstica em adultos Autora: Samia Zahi Rached M\u00e9dica da Divis\u00e3o de Pneumologia do Instituto do Cora\u00e7\u00e3o do Hospital das Cl\u00ednicas da Faculdade de Medicina da Universidade de S\u00e3o Paulo Caso Cl\u00ednico: Paciente 21 anos, sexo masculino, casado, desempregado, natural e procedente de S\u00e3o Paulo Queixa Principal: Exacerba\u00e7\u00f5es pulmonares frequentes Hist\u00f3ria [&hellip;]<!-- AddThis Advanced Settings generic via filter on get_the_excerpt --><!-- AddThis Share Buttons generic via filter on get_the_excerpt --><\/p>\n","protected":false},"author":2,"featured_media":0,"comment_status":"closed","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_acf_changed":false,"ngg_post_thumbnail":0,"footnotes":""},"categories":[15,217],"tags":[],"class_list":["post-10569","post","type-post","status-publish","format-standard","hentry","category-acesso-restrito","category-casos-clinicos-2018"],"acf":[],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v28.6 - 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