array:24 [
  "pii" => "S0300289622003301"
  "issn" => "03002896"
  "doi" => "10.1016/j.arbres.2022.02.018"
  "estado" => "S300"
  "fechaPublicacion" => "2022-05-01"
  "aid" => "3106"
  "copyright" => "SEPAR"
  "copyrightAnyo" => "2022"
  "documento" => "simple-article"
  "crossmark" => 1
  "subdocumento" => "crp"
  "cita" => "Arch Bronconeumol. 2022;58:T431-T432"
  "abierto" => array:3 [
    "ES" => true
    "ES2" => true
    "LATM" => true
  ]
  "gratuito" => true
  "lecturas" => array:1 [
    "total" => 0
  ]
  "itemSiguiente" => array:18 [
    "pii" => "S0300289621001241"
    "issn" => "03002896"
    "doi" => "10.1016/j.arbres.2021.04.004"
    "estado" => "S300"
    "fechaPublicacion" => "2022-05-01"
    "aid" => "2808"
    "copyright" => "SEPAR"
    "documento" => "article"
    "crossmark" => 1
    "subdocumento" => "sco"
    "cita" => "Arch Bronconeumol. 2022;58:433"
    "abierto" => array:3 [
      "ES" => true
      "ES2" => true
      "LATM" => true
    ]
    "gratuito" => true
    "lecturas" => array:1 [
      "total" => 0
    ]
    "es" => array:11 [
      "idiomaDefecto" => true
      "cabecera" => "<span class="elsevierStyleTextfn">Imagen cl&#237;nica</span>"
      "titulo" => "Transmogrificaci&#243;n placentaria del pulm&#243;n"
      "tienePdf" => "es"
      "tieneTextoCompleto" => "es"
      "paginas" => array:1 [
        0 => array:1 [
          "paginaInicial" => "433"
        ]
      ]
      "titulosAlternativos" => array:1 [
        "en" => array:1 [
          "titulo" => "Placental Transmogrification of the Lung"
        ]
      ]
      "contieneTextoCompleto" => array:1 [
        "es" => true
      ]
      "contienePdf" => array:1 [
        "es" => true
      ]
      "resumenGrafico" => array:2 [
        "original" => 0
        "multimedia" => array:7 [
          "identificador" => "fig0005"
          "etiqueta" => "Figura 1"
          "tipo" => "MULTIMEDIAFIGURA"
          "mostrarFloat" => true
          "mostrarDisplay" => false
          "figura" => array:1 [
            0 => array:4 [
              "imagen" => "gr1.jpeg"
              "Alto" => 950
              "Ancho" => 950
              "Tamanyo" => 272836
            ]
          ]
          "descripcion" => array:1 [
            "es" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Histolog&#237;a y radiolog&#237;a&#46; A&#41; Microscop&#237;a &#243;ptica &#40;MO&#41;&#58; lesi&#243;n no encapsulada compuesta de estructuras papilares dentro de espacios qu&#237;sticos hiperaireados y presencia significativa de tejido adiposo con focos ocasionales de metaplasia &#243;sea &#40;flecha&#41; &#40;H&#38;E 2&#215;&#41;&#46; B&#41; MO&#58; en las estructuras papilares &#40;flecha&#41; se observa una proliferaci&#243;n de capilares en el interior y abundantes mastocitos &#40;H&#38;E 10&#215;&#41;&#46; C&#41; Corte axial de TC&#58; opacidad en vidrio deslustrado de 19<span class="elsevierStyleHsp" style=""></span>mm en su di&#225;metro m&#225;ximo&#44; localizada en el l&#243;bulo inferior izquierdo&#46; D&#41; Corte sagital de TC&#58; la lesi&#243;n asocia un componente s&#243;lido&#44; qu&#237;stico y trabeculado&#46;</p>"
          ]
        ]
      ]
      "autores" => array:1 [
        0 => array:2 [
          "autoresLista" => "Giovanna Foschini, Carmen M&#46; Rodr&#237;guez, Matilde M&#46; Rubio, Xavier Baldo"
          "autores" => array:4 [
            0 => array:2 [
              "nombre" => "Giovanna"
              "apellidos" => "Foschini"
            ]
            1 => array:2 [
              "nombre" => "Carmen M&#46;"
              "apellidos" => "Rodr&#237;guez"
            ]
            2 => array:2 [
              "nombre" => "Matilde M&#46;"
              "apellidos" => "Rubio"
            ]
            3 => array:2 [
              "nombre" => "Xavier"
              "apellidos" => "Baldo"
            ]
          ]
        ]
      ]
    ]
    "idiomaDefecto" => "es"
    "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0300289621001241?idApp=UINPBA00003Z"
    "url" => "/03002896/0000005800000005/v2_202212170819/S0300289621001241/v2_202212170819/es/main.assets"
  ]
  "itemAnterior" => array:18 [
    "pii" => "S0300289622003258"
    "issn" => "03002896"
    "doi" => "10.1016/j.arbres.2022.02.017"
    "estado" => "S300"
    "fechaPublicacion" => "2022-05-01"
    "aid" => "3102"
    "copyright" => "SEPAR"
    "documento" => "simple-article"
    "crossmark" => 1
    "subdocumento" => "crp"
    "cita" => "Arch Bronconeumol. 2022;58:431-2"
    "abierto" => array:3 [
      "ES" => true
      "ES2" => true
      "LATM" => true
    ]
    "gratuito" => true
    "lecturas" => array:1 [
      "total" => 0
    ]
    "es" => array:11 [
      "idiomaDefecto" => true
      "cabecera" => "<span class="elsevierStyleTextfn">Caso cl&#237;nico</span>"
      "titulo" => "Vasculitis &#40;pANCA&#41; asociada con hemorragia alveolar y afectaci&#243;n renal y enfermedad relacionada con IgG4&#58; un nuevo s&#237;ndrome de solapamiento"
      "tienePdf" => "es"
      "tieneTextoCompleto" => "es"
      "paginas" => array:1 [
        0 => array:2 [
          "paginaInicial" => "431"
          "paginaFinal" => "432"
        ]
      ]
      "titulosAlternativos" => array:1 [
        "en" => array:1 [
          "titulo" => "ANCA-Associated Vasculitis Presenting With Alveolar Hemorrhage and Renal Involvement and IgG4-Related Disease&#58; A New Overlap Syndrome"
        ]
      ]
      "contieneTextoCompleto" => array:1 [
        "es" => true
      ]
      "contienePdf" => array:1 [
        "es" => true
      ]
      "resumenGrafico" => array:2 [
        "original" => 0
        "multimedia" => array:7 [
          "identificador" => "fig0005"
          "etiqueta" => "Figura 1"
          "tipo" => "MULTIMEDIAFIGURA"
          "mostrarFloat" => true
          "mostrarDisplay" => false
          "figura" => array:1 [
            0 => array:4 [
              "imagen" => "gr1.jpeg"
              "Alto" => 801
              "Ancho" => 2825
              "Tamanyo" => 452291
            ]
          ]
          "descripcion" => array:1 [
            "es" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A&#46; TAC tor&#225;cico con cortes coronales con presencia de opacidades bilaterales asociadas con hemorragia alveolar&#46; Modificada&#46; B&#46; Biopsia nasal con afectaci&#243;n de la pared vascular con presencia de vasculitis&#46; C&#46; Tinci&#243;n inmunohistoqu&#237;mica espec&#237;fica positiva para IgG4 &#40;25 c&#233;lulas plasm&#225;ticas IgG4 &#43;<span class="elsevierStyleHsp" style=""></span> por campo de gran aumento&#41; junto al infiltrado linfoplasmoc&#237;tico e histiocitos&#46; Presencia de congesti&#243;n vascular&#46;</p>"
          ]
        ]
      ]
      "autores" => array:1 [
        0 => array:2 [
          "autoresLista" => "Soledad Torres Tienza, Francisco Rueda Correa, Sergio Campos T&#233;llez, Jos&#233; Javier Jare&#241;o Esteban"
          "autores" => array:4 [
            0 => array:2 [
              "nombre" => "Soledad"
              "apellidos" => "Torres Tienza"
            ]
            1 => array:2 [
              "nombre" => "Francisco"
              "apellidos" => "Rueda Correa"
            ]
            2 => array:2 [
              "nombre" => "Sergio"
              "apellidos" => "Campos T&#233;llez"
            ]
            3 => array:2 [
              "nombre" => "Jos&#233; Javier"
              "apellidos" => "Jare&#241;o Esteban"
            ]
          ]
        ]
      ]
    ]
    "idiomaDefecto" => "es"
    "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0300289622003258?idApp=UINPBA00003Z"
    "url" => "/03002896/0000005800000005/v2_202212170819/S0300289622003258/v2_202212170819/es/main.assets"
  ]
  "asociados" => array:1 [
    0 => array:18 [
      "pii" => "S0300289622003258"
      "issn" => "03002896"
      "doi" => "10.1016/j.arbres.2022.02.017"
      "estado" => "S300"
      "fechaPublicacion" => "2022-05-01"
      "aid" => "3102"
      "copyright" => "SEPAR"
      "documento" => "simple-article"
      "crossmark" => 1
      "subdocumento" => "crp"
      "cita" => "Arch Bronconeumol. 2022;58:431-2"
      "abierto" => array:3 [
        "ES" => true
        "ES2" => true
        "LATM" => true
      ]
      "gratuito" => true
      "lecturas" => array:1 [
        "total" => 0
      ]
      "es" => array:11 [
        "idiomaDefecto" => true
        "cabecera" => "<span class="elsevierStyleTextfn">Caso cl&#237;nico</span>"
        "titulo" => "Vasculitis &#40;pANCA&#41; asociada con hemorragia alveolar y afectaci&#243;n renal y enfermedad relacionada con IgG4&#58; un nuevo s&#237;ndrome de solapamiento"
        "tienePdf" => "es"
        "tieneTextoCompleto" => "es"
        "paginas" => array:1 [
          0 => array:2 [
            "paginaInicial" => "431"
            "paginaFinal" => "432"
          ]
        ]
        "titulosAlternativos" => array:1 [
          "en" => array:1 [
            "titulo" => "ANCA-Associated Vasculitis Presenting With Alveolar Hemorrhage and Renal Involvement and IgG4-Related Disease&#58; A New Overlap Syndrome"
          ]
        ]
        "contieneTextoCompleto" => array:1 [
          "es" => true
        ]
        "contienePdf" => array:1 [
          "es" => true
        ]
        "resumenGrafico" => array:2 [
          "original" => 0
          "multimedia" => array:7 [
            "identificador" => "fig0005"
            "etiqueta" => "Figura 1"
            "tipo" => "MULTIMEDIAFIGURA"
            "mostrarFloat" => true
            "mostrarDisplay" => false
            "figura" => array:1 [
              0 => array:4 [
                "imagen" => "gr1.jpeg"
                "Alto" => 801
                "Ancho" => 2825
                "Tamanyo" => 452291
              ]
            ]
            "descripcion" => array:1 [
              "es" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A&#46; TAC tor&#225;cico con cortes coronales con presencia de opacidades bilaterales asociadas con hemorragia alveolar&#46; Modificada&#46; B&#46; Biopsia nasal con afectaci&#243;n de la pared vascular con presencia de vasculitis&#46; C&#46; Tinci&#243;n inmunohistoqu&#237;mica espec&#237;fica positiva para IgG4 &#40;25 c&#233;lulas plasm&#225;ticas IgG4 &#43;<span class="elsevierStyleHsp" style=""></span> por campo de gran aumento&#41; junto al infiltrado linfoplasmoc&#237;tico e histiocitos&#46; Presencia de congesti&#243;n vascular&#46;</p>"
            ]
          ]
        ]
        "autores" => array:1 [
          0 => array:2 [
            "autoresLista" => "Soledad Torres Tienza, Francisco Rueda Correa, Sergio Campos T&#233;llez, Jos&#233; Javier Jare&#241;o Esteban"
            "autores" => array:4 [
              0 => array:2 [
                "nombre" => "Soledad"
                "apellidos" => "Torres Tienza"
              ]
              1 => array:2 [
                "nombre" => "Francisco"
                "apellidos" => "Rueda Correa"
              ]
              2 => array:2 [
                "nombre" => "Sergio"
                "apellidos" => "Campos T&#233;llez"
              ]
              3 => array:2 [
                "nombre" => "Jos&#233; Javier"
                "apellidos" => "Jare&#241;o Esteban"
              ]
            ]
          ]
        ]
      ]
      "idiomaDefecto" => "es"
      "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0300289622003258?idApp=UINPBA00003Z"
      "url" => "/03002896/0000005800000005/v2_202212170819/S0300289622003258/v2_202212170819/es/main.assets"
    ]
  ]
  "en" => array:14 [
    "idiomaDefecto" => true
    "cabecera" => "<span class="elsevierStyleTextfn">Case Report</span>"
    "titulo" => " ANCA-Associated Vasculitis Presenting With Alveolar Hemorrhage and Renal Involvement and IgG4-Related Disease&#58; A New Overlap Syndrome"
    "tieneTextoCompleto" => true
    "paginas" => array:1 [
      0 => array:2 [
        "paginaInicial" => "T431"
        "paginaFinal" => "T432"
      ]
    ]
    "autores" => array:1 [
      0 => array:4 [
        "autoresLista" => "Soledad Torres Tienza, Francisco Rueda Correa, Sergio Campos T&#233;llez, Jos&#233; Javier Jare&#241;o Esteban"
        "autores" => array:4 [
          0 => array:3 [
            "nombre" => "Soledad"
            "apellidos" => "Torres Tienza"
            "referencia" => array:1 [
              0 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">a</span>"
                "identificador" => "aff0005"
              ]
            ]
          ]
          1 => array:3 [
            "nombre" => "Francisco"
            "apellidos" => "Rueda Correa"
            "referencia" => array:1 [
              0 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">b</span>"
                "identificador" => "aff0010"
              ]
            ]
          ]
          2 => array:3 [
            "nombre" => "Sergio"
            "apellidos" => "Campos T&#233;llez"
            "referencia" => array:1 [
              0 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">a</span>"
                "identificador" => "aff0005"
              ]
            ]
          ]
          3 => array:4 [
            "nombre" => "Jos&#233; Javier"
            "apellidos" => "Jare&#241;o Esteban"
            "email" => array:1 [
              0 => "jjjarenoesteban@yahoo.es"
            ]
            "referencia" => array:2 [
              0 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">a</span>"
                "identificador" => "aff0005"
              ]
              1 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">&#42;</span>"
                "identificador" => "cor0005"
              ]
            ]
          ]
        ]
        "afiliaciones" => array:2 [
          0 => array:3 [
            "entidad" => "Servicio de Neumolog&#237;a&#44; Hospital Central de la Defensa &#8220;G&#243;mez Ulla&#8221;&#44; Spain"
            "etiqueta" => "a"
            "identificador" => "aff0005"
          ]
          1 => array:3 [
            "entidad" => "Servicio de Anatom&#237;a Patol&#243;gica Neumolog&#237;a&#44; Hospital Central de la Defensa &#8220;G&#243;mez Ulla&#8221;&#44; Spain"
            "etiqueta" => "b"
            "identificador" => "aff0010"
          ]
        ]
        "correspondencia" => array:1 [
          0 => array:3 [
            "identificador" => "cor0005"
            "etiqueta" => "&#8270;"
            "correspondencia" => "Corresponding author&#46;"
          ]
        ]
      ]
    ]
    "titulosAlternativos" => array:1 [
      "es" => array:1 [
        "titulo" => "Vasculitis &#40;pANCA&#41; asociada con hemorragia alveolar y afectaci&#243;n renal y enfermedad relacionada con IgG4&#58; un nuevo s&#237;ndrome de solapamiento"
      ]
    ]
    "resumenGrafico" => array:2 [
      "original" => 0
      "multimedia" => array:7 [
        "identificador" => "fig0005"
        "etiqueta" => "Fig&#46; 1"
        "tipo" => "MULTIMEDIAFIGURA"
        "mostrarFloat" => true
        "mostrarDisplay" => false
        "figura" => array:1 [
          0 => array:4 [
            "imagen" => "gr1.jpeg"
            "Alto" => 808
            "Ancho" => 2825
            "Tamanyo" => 432416
          ]
        ]
        "descripcion" => array:1 [
          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">&#40;a&#41; Chest CT with coronal slices showing bilateral opacities associated with alveolar hemorrhage&#46; Modified&#46; &#40;b&#41; Nasal biopsy with vascular wall involvement showing vasculitis&#46; &#40;c&#41; Specific immunohistochemical staining positive for IgG4 &#40;25 IgG4-positive plasma cells per high power field&#41; together with lymphoplasmocytic infiltrate and vascular congestion &#40;c&#41;&#46; Presence of vascular congestion&#46;</p>"
        ]
      ]
    ]
    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">We report the case of a 57-year-old man with cough&#44; dyspnea&#44; hemoptysis&#44; and epistaxis&#44; and no history of exposure to drugs&#44; toxic substances or chemicals&#46; He had previously been diagnosed with IgG4-related disease &#40;IgG4-RD&#41; by lacrimal gland biopsy&#44; and also had chronic gastritis&#44; autoimmune hypothyroidism&#44; hypergonadotropic hypogonadism&#44; chronic anemia&#44; and chronic kidney disease stage V&#46; On physical examination&#44; he was afebrile&#44; tachypneic&#44; and bleeding from the nose&#46; Pulmonary auscultation revealed bilateral wet crackles&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">Labs&#58; RBC 3&#46;12&#47;mm<span class="elsevierStyleSup">3</span>&#44; Hct&#58; 29&#46;5&#37;&#44; Hb&#58; 9&#46;1<span class="elsevierStyleHsp" style=""></span>g&#47;dL&#44; WBC&#58; 15&#44;360&#47;mm<span class="elsevierStyleSup">3</span> &#40;82&#37; neutrophils&#41;&#44; Platelets&#58; 277&#44;000&#44; Glucose 109<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#44; Creatinine&#58; 12&#46;46<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#44; Urea&#58; 161<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#44; CPK&#58; 72<span class="elsevierStyleHsp" style=""></span>U&#47;L&#44; Na&#58; 142<span class="elsevierStyleHsp" style=""></span>mEquiv&#46;&#47;L&#44; K&#58; 5&#46;6<span class="elsevierStyleHsp" style=""></span>mEquiv&#46;&#47;L&#44; PRO-BNP&#58; 22&#44;644<span class="elsevierStyleHsp" style=""></span>pg&#47;mL&#44; CRP&#58; 20&#46;91<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#44; PCT&#58; 0&#46;929<span class="elsevierStyleHsp" style=""></span>ng&#47;dL&#46; Arterial blood gases &#40;FiO<span class="elsevierStyleInf">2</span>&#58; 0&#46;21&#41;&#58; pH 7&#46;47&#44; pCO<span class="elsevierStyleInf">2</span>&#58; 34&#46;4<span class="elsevierStyleHsp" style=""></span>mmHg&#44; pO<span class="elsevierStyleInf">2</span>&#58; 59<span class="elsevierStyleHsp" style=""></span>mmHg&#44; HCO<span class="elsevierStyleInf">3</span><span class="elsevierStyleSup">&#8722;</span> 25&#46;4&#46; Normal liver function and coagulation&#46; Chest X-ray with bilateral alveolar pattern&#46; Chest&#44; cranial&#44; and upper airway CT confirmed alveolar consolidation&#44; with thickening of left maxillary sinus mucosa &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>a&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Fiberoptic bronchoscopy revealed active bleeding in the left bronchial tree and bronchoalveolar lavage showed more than 15&#37; hemosiderophages&#46; DLCO&#58; 120&#37; KCO&#58; 154&#37;&#44; elevated&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">Autoimmune testing positive for p-ANCA &#40;Anti-MPO&#58; 33<span class="elsevierStyleHsp" style=""></span>IU&#47;mL&#41; and negative for ANAs&#44; ENAs&#44; C3&#58; 83<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#44; C4&#58; 30<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#44; AMA&#44; AML&#44; Anti-MBG and cryoglobulins&#46; Serum IgG&#58; 934<span class="elsevierStyleHsp" style=""></span>mg&#47;dL and IgG4&#58; 38<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#44; normal&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">Nasal biopsy &#40;inferior and middle turbinate&#41; showed 2 medium-caliber vascular structures with walls occupied by polymorphonuclear neutrophils&#44; showing fibrinoid degeneration&#44; consistent with a diagnosis of leukocytoclastic vasculitis &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>b&#41;&#46; Specific immunohistochemical staining was positive for IgG4 &#40;25 IgG4-positive plasma cells per high power field&#41; together with lymphoplasmocytic infiltrate and vascular congestion &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>c&#41;&#46; The patient was diagnosed with microscopic polyangitis-type vasculitis with p-ANCA&#44; alveolar hemorrhage and acute renal failure&#44; and IgG4-RD&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">Treatment consisted of cyclophosphamide&#44; prednisone &#40;70<span class="elsevierStyleHsp" style=""></span>mg&#47;kg&#47;day&#41;&#44; plasmapheresis &#40;alternating days&#41; and hemodialysis&#46; The patient&#39;s progress was satisfactory&#44; with no recurrences of alveolar hemorrhage and negativization of p-ANCA&#46; Renal function deterioration required continued hemodialysis&#46; Four years after diagnosis&#44; he received a renal transplant and has shown favorable progress at 1 year&#46;</p><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Discussion</span><p id="par0035" class="elsevierStylePara elsevierViewall">ANCA-associated vasculitis &#40;AAV&#41;-IgG4-RD overlap syndrome is a new entity that was first identified in 2017&#44;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> although these findings were described in 2011 in patients with tubulointerstitial nephritis with IgG4-RD and vasculitis&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">The study by Danlos F-X et al&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> included 18 patients with a mean age of 55 years&#44; with AAV and IgG4-RD according to Chapel Hill criteria &#40;2013&#41;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a> and the CDC international criteria for IgG4-RD &#40;possible&#44; probable&#44; and definitive&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a> The diagnosis was concomitant in 72&#37; of the series&#59; in 17&#37;&#44; AAV preceded IgG4-RD and in 11&#37;&#44; IgG4-RD preceded AAV&#46; The diagnosis of IgG4-RD was established as definitive in 28&#37;&#44; probable in 28&#37;&#44; and possible in 44&#37;&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1&#44;2</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">Plasma replacement plus glucocorticoids was questioned in the PEXIVAS study&#44;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> because of its lack of effectiveness in reducing mortality and preserving renal function&#44; but the ACR 2021 consensus statement on the management of AAV recommends its use if there is a high risk of severe renal failure&#46; Rituximab may be used as a first-line treatment in both entities&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a> Multicenter studies are needed to improve knowledge and understanding of this new entity&#46;</p></span></span>"
    "textoCompletoSecciones" => array:1 [
      "secciones" => array:2 [
        0 => array:2 [
          "identificador" => "sec0010"
          "titulo" => "Discussion"
        ]
        1 => array:1 [
          "titulo" => "References"
        ]
      ]
    ]
    "pdfFichero" => "main.pdf"
    "tienePdf" => true
    "multimedia" => array:1 [
      0 => array:7 [
        "identificador" => "fig0005"
        "etiqueta" => "Fig&#46; 1"
        "tipo" => "MULTIMEDIAFIGURA"
        "mostrarFloat" => true
        "mostrarDisplay" => false
        "figura" => array:1 [
          0 => array:4 [
            "imagen" => "gr1.jpeg"
            "Alto" => 808
            "Ancho" => 2825
            "Tamanyo" => 432416
          ]
        ]
        "descripcion" => array:1 [
          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">&#40;a&#41; Chest CT with coronal slices showing bilateral opacities associated with alveolar hemorrhage&#46; Modified&#46; &#40;b&#41; Nasal biopsy with vascular wall involvement showing vasculitis&#46; &#40;c&#41; Specific immunohistochemical staining positive for IgG4 &#40;25 IgG4-positive plasma cells per high power field&#41; together with lymphoplasmocytic infiltrate and vascular congestion &#40;c&#41;&#46; Presence of vascular congestion&#46;</p>"
        ]
      ]
    ]
    "bibliografia" => array:2 [
      "titulo" => "References"
      "seccion" => array:1 [
        0 => array:2 [
          "identificador" => "bibs0015"
          "bibliografiaReferencia" => array:5 [
            0 => array:3 [
              "identificador" => "bib0030"
              "etiqueta" => "1"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Antineutrophil cytoplasmic antibody-associated vasculitides and IgG4-related disease&#58; a new overlap syndrome"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "F&#46;-X&#46; Danlos"
                            1 => "G&#46;M&#46; Rossi"
                            2 => "D&#46; Blockmans"
                            3 => "G&#46; Emmi"
                            4 => "A&#46; Kronbichler"
                            5 => "S&#46; Durupt"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:5 [
                        "tituloSerie" => "Autoimmun Rev"
                        "fecha" => "2017"
                        "volumen" => "16"
                        "paginaInicial" => "1036"
                        "paginaFinal" => "1043"
                      ]
                    ]
                  ]
                ]
              ]
            ]
            1 => array:3 [
              "identificador" => "bib0035"
              "etiqueta" => "2"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Comprehensive diagnostic criteria for IgG4-related disease &#40;IgG4-RD&#41;&#44; 2011"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "H&#46; Umehara"
                            1 => "K&#46; Okazaki"
                            2 => "Y&#46; Masaki"
                            3 => "M&#46; Kawano"
                            4 => "M&#46; Yamamoto"
                            5 => "T&#46; Saeki"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1007/s10165-011-0571-z"
                      "Revista" => array:6 [
                        "tituloSerie" => "Mod Rheumatol"
                        "fecha" => "2012"
                        "volumen" => "22"
                        "paginaInicial" => "21"
                        "paginaFinal" => "30"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/22218969"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            2 => array:3 [
              "identificador" => "bib0040"
              "etiqueta" => "3"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "2012 Revised International Chapel Hill consensus conference nomenclature of vasculitides"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "J&#46;C&#46; Jennette"
                            1 => "R&#46;J&#46; Falk"
                            2 => "P&#46;A&#46; Bacon"
                            3 => "N&#46; Basu"
                            4 => "M&#46;C&#46; Cid"
                            5 => "F&#46; Ferrario"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1002/art.37715"
                      "Revista" => array:6 [
                        "tituloSerie" => "Arthritis Rheum"
                        "fecha" => "2013"
                        "volumen" => "65"
                        "paginaInicial" => "1"
                        "paginaFinal" => "11"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/23045170"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            3 => array:3 [
              "identificador" => "bib0045"
              "etiqueta" => "4"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:1 [
                      "autores" => array:1 [
                        0 => array:3 [
                          "colaboracion" => "for the PEXIVAS Investigator"
                          "etal" => true
                          "autores" => array:6 [
                            0 => "M&#46; Walsh"
                            1 => "P&#46;A&#46; Merkel"
                            2 => "C&#46;A&#46; Peh"
                            3 => "W&#46;M&#46; Szpirt"
                            4 => "X&#46; Pu&#233;chal"
                            5 => "S&#46; Fuyimoto"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1056/NEJMoa1803537"
                      "Revista" => array:6 [
                        "tituloSerie" => "N Engl J Med"
                        "fecha" => "2020"
                        "volumen" => "382"
                        "paginaInicial" => "622"
                        "paginaFinal" => "631"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/32053298"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            4 => array:3 [
              "identificador" => "bib0050"
              "etiqueta" => "5"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "2021 American College of Rheumatology&#47;Vasculitis Foundation Guideline for the management of Antineutrophil Cytoplasmic Antibody &#8211; Associated Vasculitis&#46; American College of Rheumatology 2021"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:3 [
                            0 => "S&#46;A&#46; Chung"
                            1 => "C&#46;A&#46; Langford"
                            2 => "M&#46; Maz"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1002/acr 4634"
                      "Revista" => array:5 [
                        "tituloSerie" => "Arthritis Care Res"
                        "fecha" => "2021"
                        "volumen" => "73"
                        "paginaInicial" => "1088"
                        "paginaFinal" => "1105"
                      ]
                    ]
                  ]
                ]
              ]
            ]
          ]
        ]
      ]
    ]
  ]
  "idiomaDefecto" => "en"
  "url" => "/03002896/0000005800000005/v2_202212170819/S0300289622003301/v2_202212170819/en/main.assets"
  "Apartado" => array:4 [
    "identificador" => "93649"
    "tipo" => "SECCION"
    "en" => array:2 [
      "titulo" => "Case reports&#47;Casos cl&#237;nicos"
      "idiomaDefecto" => true
    ]
    "idiomaDefecto" => "en"
  ]
  "PDF" => "https://static.elsevier.es/multimedia/03002896/0000005800000005/v2_202212170819/S0300289622003301/v2_202212170819/en/main.pdf?idApp=UINPBA00003Z&text.app=https://archbronconeumol.org/"
  "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0300289622003301?idApp=UINPBA00003Z"
]
Share
Journal Information
Vol. 58. Issue 5.
Pages T431-T432 (May 2022)
Share
Share
Download PDF
More article options
Vol. 58. Issue 5.
Pages T431-T432 (May 2022)
Case Report
Full text access
ANCA-Associated Vasculitis Presenting With Alveolar Hemorrhage and Renal Involvement and IgG4-Related Disease: A New Overlap Syndrome
Vasculitis (pANCA) asociada con hemorragia alveolar y afectación renal y enfermedad relacionada con IgG4: un nuevo síndrome de solapamiento
Visits
2551
Soledad Torres Tienzaa, Francisco Rueda Correab, Sergio Campos Télleza, José Javier Jareño Estebana,
Corresponding author
jjjarenoesteban@yahoo.es

Corresponding author.
a Servicio de Neumología, Hospital Central de la Defensa “Gómez Ulla”, Spain
b Servicio de Anatomía Patológica Neumología, Hospital Central de la Defensa “Gómez Ulla”, Spain
Related content
Soledad Torres Tienza, Francisco Rueda Correa, Sergio Campos Téllez, José Javier Jareño Esteban
This item has received
Article information
Full Text
Bibliography
Download PDF
Statistics
Figures (1)
Full Text

We report the case of a 57-year-old man with cough, dyspnea, hemoptysis, and epistaxis, and no history of exposure to drugs, toxic substances or chemicals. He had previously been diagnosed with IgG4-related disease (IgG4-RD) by lacrimal gland biopsy, and also had chronic gastritis, autoimmune hypothyroidism, hypergonadotropic hypogonadism, chronic anemia, and chronic kidney disease stage V. On physical examination, he was afebrile, tachypneic, and bleeding from the nose. Pulmonary auscultation revealed bilateral wet crackles.

Labs: RBC 3.12/mm3, Hct: 29.5%, Hb: 9.1g/dL, WBC: 15,360/mm3 (82% neutrophils), Platelets: 277,000, Glucose 109mg/dL, Creatinine: 12.46mg/dL, Urea: 161mg/dL, CPK: 72U/L, Na: 142mEquiv./L, K: 5.6mEquiv./L, PRO-BNP: 22,644pg/mL, CRP: 20.91mg/dL, PCT: 0.929ng/dL. Arterial blood gases (FiO2: 0.21): pH 7.47, pCO2: 34.4mmHg, pO2: 59mmHg, HCO3 25.4. Normal liver function and coagulation. Chest X-ray with bilateral alveolar pattern. Chest, cranial, and upper airway CT confirmed alveolar consolidation, with thickening of left maxillary sinus mucosa (Fig. 1a).

Fig. 1.

(a) Chest CT with coronal slices showing bilateral opacities associated with alveolar hemorrhage. Modified. (b) Nasal biopsy with vascular wall involvement showing vasculitis. (c) Specific immunohistochemical staining positive for IgG4 (25 IgG4-positive plasma cells per high power field) together with lymphoplasmocytic infiltrate and vascular congestion (c). Presence of vascular congestion.

(0.41MB).

Fiberoptic bronchoscopy revealed active bleeding in the left bronchial tree and bronchoalveolar lavage showed more than 15% hemosiderophages. DLCO: 120% KCO: 154%, elevated.

Autoimmune testing positive for p-ANCA (Anti-MPO: 33IU/mL) and negative for ANAs, ENAs, C3: 83mg/dL, C4: 30mg/dL, AMA, AML, Anti-MBG and cryoglobulins. Serum IgG: 934mg/dL and IgG4: 38mg/dL, normal.

Nasal biopsy (inferior and middle turbinate) showed 2 medium-caliber vascular structures with walls occupied by polymorphonuclear neutrophils, showing fibrinoid degeneration, consistent with a diagnosis of leukocytoclastic vasculitis (Fig. 1b). Specific immunohistochemical staining was positive for IgG4 (25 IgG4-positive plasma cells per high power field) together with lymphoplasmocytic infiltrate and vascular congestion (Fig. 1c). The patient was diagnosed with microscopic polyangitis-type vasculitis with p-ANCA, alveolar hemorrhage and acute renal failure, and IgG4-RD.

Treatment consisted of cyclophosphamide, prednisone (70mg/kg/day), plasmapheresis (alternating days) and hemodialysis. The patient's progress was satisfactory, with no recurrences of alveolar hemorrhage and negativization of p-ANCA. Renal function deterioration required continued hemodialysis. Four years after diagnosis, he received a renal transplant and has shown favorable progress at 1 year.

Discussion

ANCA-associated vasculitis (AAV)-IgG4-RD overlap syndrome is a new entity that was first identified in 2017,1 although these findings were described in 2011 in patients with tubulointerstitial nephritis with IgG4-RD and vasculitis.2

The study by Danlos F-X et al.1 included 18 patients with a mean age of 55 years, with AAV and IgG4-RD according to Chapel Hill criteria (2013)3 and the CDC international criteria for IgG4-RD (possible, probable, and definitive).2 The diagnosis was concomitant in 72% of the series; in 17%, AAV preceded IgG4-RD and in 11%, IgG4-RD preceded AAV. The diagnosis of IgG4-RD was established as definitive in 28%, probable in 28%, and possible in 44%.1,2

Plasma replacement plus glucocorticoids was questioned in the PEXIVAS study,4 because of its lack of effectiveness in reducing mortality and preserving renal function, but the ACR 2021 consensus statement on the management of AAV recommends its use if there is a high risk of severe renal failure. Rituximab may be used as a first-line treatment in both entities.5 Multicenter studies are needed to improve knowledge and understanding of this new entity.

References
[1]
F.-X. Danlos, G.M. Rossi, D. Blockmans, G. Emmi, A. Kronbichler, S. Durupt, et al.
Antineutrophil cytoplasmic antibody-associated vasculitides and IgG4-related disease: a new overlap syndrome.
Autoimmun Rev, 16 (2017), pp. 1036-1043
[2]
H. Umehara, K. Okazaki, Y. Masaki, M. Kawano, M. Yamamoto, T. Saeki, et al.
Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011.
Mod Rheumatol, 22 (2012), pp. 21-30
[3]
J.C. Jennette, R.J. Falk, P.A. Bacon, N. Basu, M.C. Cid, F. Ferrario, et al.
2012 Revised International Chapel Hill consensus conference nomenclature of vasculitides.
Arthritis Rheum, 65 (2013), pp. 1-11
[4]
M. Walsh, P.A. Merkel, C.A. Peh, W.M. Szpirt, X. Puéchal, S. Fuyimoto, for the PEXIVAS Investigator, et al.
N Engl J Med, 382 (2020), pp. 622-631
[5]
S.A. Chung, C.A. Langford, M. Maz, et al.
2021 American College of Rheumatology/Vasculitis Foundation Guideline for the management of Antineutrophil Cytoplasmic Antibody – Associated Vasculitis. American College of Rheumatology 2021.
Arthritis Care Res, 73 (2021), pp. 1088-1105
Copyright © 2022. SEPAR
Archivos de Bronconeumología
Article options
Tools

Are you a health professional able to prescribe or dispense drugs?