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junto al infiltrado linfoplasmoc&#237;tico y la congesti&#243;n vascular &#40;<a class="elsevierStyleCrossRef" href="#fig0005">fig&#46; 1</a>c&#41;&#46; El paciente fue diagnosticado de vasculitis tipo PAM microsc&#243;pica con p-ANCA&#44; hemorragia alveolar y fallo renal agudo&#44; as&#237; como enfermedad por IgG4 &#40;ER-IgG4&#41;&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">El tratamiento con ciclofosfamida&#44; prednisona&#58; 70<span class="elsevierStyleHsp" style=""></span>mg&#47;kg&#47;d&#237;a&#44; plasmaf&#233;resis &#40;alterna&#41; y hemodi&#225;lisis&#44; tuvo evoluci&#243;n satisfactoria&#44; sin recidivas de hemorragia alveolar&#44; con negativizaci&#243;n de p-ANCA&#46; El deterioro de la funci&#243;n renal oblig&#243; a mantener la hemodi&#225;lisis&#46; A los cuatro a&#241;os del diagn&#243;stico&#44; recibi&#243; un trasplante renal con evoluci&#243;n favorable al a&#241;o&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Discusi&#243;n</span><p id="par0035" class="elsevierStylePara elsevierViewall">El s&#237;ndrome de asociaci&#243;n &#40;VAA- ER-IgG4&#41; es una nueva entidad reconocida en 2017<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a>&#46; Estos hallazgos son conocidos desde 2011 en pacientes con nefritis t&#250;bulointersticial con ER-IgG4 y vasculitis<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a>&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">El estudio de Danlos F-X et al&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> incluy&#243; a 18 pacientes de edad media de 55 a&#241;os&#44; con VAA y ER-IgG4&#44; seg&#250;n criterios de Chapel Hill 2013<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a>&#44; y seg&#250;n los criterios internacionales de CDC para ER-IgG4 &#40;posible&#44; probable y definitiva&#41;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a>&#46; En el 72&#37; el diagn&#243;stico fue concomitante&#59; un 17&#37; la VAA precedi&#243; a la ER-IgG4 y en el 11&#37; la ER-IgG4 precedi&#243; a la VAA&#46; El diagn&#243;stico de ER-IgG4 se estableci&#243; en un 28&#37; definitivo&#44; probable en un 28&#37; y posible en un 44&#37;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1&#44;2</span></a>&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">El recambio plasm&#225;tico m&#225;s glucocorticoides fue cuestionado en el estudio PEXIVAS<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a>&#44; por escasa efectividad en reducir la mortalidad y en preservar la funci&#243;n renal&#46; El consenso de la ACR 2021 para manejo de la VAA-ANCAS recomienda su uso si hay alto riesgo de insuficiencia renal severa&#46; Rituximab puede ser un tratamiento de primera l&#237;nea en ambas entidades<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a>&#46; Son deseables estudios multic&#233;ntricos para un mejor conocimiento y comprensi&#243;n de esta nueva entidad&#46;</p></span></span>"
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