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"documento" => "simple-article" "crossmark" => 1 "subdocumento" => "cor" "cita" => "Med Clin. 2021;156:94-5" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "en" => array:11 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Letter to the Editor</span>" "titulo" => "A new mutation in the SEPSECS gene related to pontocerebellar hypoplasia type 2D" "tienePdf" => "en" "tieneTextoCompleto" => "en" "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "94" "paginaFinal" => "95" ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Hipoplasia pontocerebelosa tipo 2D: una nueva mutación en el gen SEPSECS" ] ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1051 "Ancho" => 905 "Tamanyo" => 96550 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Brain MRI, axial and sagittal plane. (A) 14 months. (B) 4 years. Hipoplasic cerebellar vermis and hemispheres, “butterfly-like” pattern.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Marta Arrudi-Moreno, Alba Fernández-Gómez, José L. Peña-Segura" "autores" => array:3 [ 0 => array:2 [ "nombre" => "Marta" "apellidos" => "Arrudi-Moreno" ] 1 => array:2 [ "nombre" => "Alba" "apellidos" => "Fernández-Gómez" ] 2 => array:2 [ "nombre" => "José L." "apellidos" => "Peña-Segura" ] ] ] ] ] "idiomaDefecto" => "en" "Traduccion" => array:1 [ "en" => array:9 [ "pii" => "S0025775319305925" "doi" => "10.1016/j.medcli.2019.10.005" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0025775319305925?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2387020620306082?idApp=UINPBA00004N" "url" => "/23870206/0000015600000002/v2_202102250946/S2387020620306082/v2_202102250946/en/main.assets" ] "en" => array:13 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Letter to the Editor</span>" "titulo" => "Gastrointestinal tract diseases related to Ig G4" "tieneTextoCompleto" => true "saludo" => "To the Editor:" "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "95" "paginaFinal" => "96" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Joaquim Torné Cachot, Javier García Pont" "autores" => array:2 [ 0 => array:4 [ "nombre" => "Joaquim" "apellidos" => "Torné Cachot" "email" => array:1 [ 0 => "18112jtc@comb.cat" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "Javier" "apellidos" => "García Pont" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Servicio de Medicina Interna, Hospital Sant Camil, Consorci Sanitari de l’Alt Penedès-Garraf, Sant Pere de Ribes, Barcelona, Spain" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Enfermedad del tracto gastrointestinal relacionada con la Ig G4" ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">We have read with interest the article by Lorente et al.,<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> on the variability of the clinical spectrum of immunoglobulin G4-related disease (IgG4-RD). The authors present three clinical observations, one of them with gastrointestinal tract (GIT) involvement in the form of an inflammatory pseudotumor of the colon. The limited discussion of this particular site has motivated us to make various contributions in relation to our experience and the review carried out, highlighting the clinical peculiarities and the diagnostic difficulty when involvement is isolated.</p><p id="par0010" class="elsevierStylePara elsevierViewall">In our case series of 7 patients, we had the opportunity to diagnose a case of inflammatory descending colon stenosis related to Ig G4 in a patient hospitalized in the surgery department due to abdominal pain. Like Lorente et al.,<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> it was an isolated involvement of the disease and the diagnosis was unfortunately made after a hemicolectomy due to the suspicion of colon cancer. Despite the normal results of the postoperative study using <span class="elsevierStyleSup">18</span>F-FDG-PET/CT and the determination of Ig G4, steroids therapy was administered for 6 months. This possibly questionable therapeutic decision was justified by the lack of available evidence, the fact that it is a systemic disease with a high potential for multi-organ involvement, the frequent presence of subclinical sites, and its recurrent nature.</p><p id="par0015" class="elsevierStylePara elsevierViewall">The GIT is one of the sites with the fewest descriptions and its knowledge is based on clinical observations and review articles. Out of a total of 736 patients included in three large English-language cohorts of IgG4-RD, only 2 were localized in the GIT.<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2–4</span></a> GIT IgG4-RD affects glandular tissues and the vascular system. The stomach is the most commonly affected organ. In most cases, the identified GIT disease constitutes the only manifestation of IgG4-RD. In a physiopathological sense, the disease causes two types of lesions, wall thickening that can manifest itself in the form of organ dysfunction, stenosis and ulcerations, and the formation of pseudotumors (polyps and submucosal masses). Pseudotumoral lesions are usually prevalent in the stomach and colon, while dysfunction and obstructive clinical features are found in any segment of the GIT.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> In IgC4-related pseudotumor processes of the pancreas and mesentery, GIT involvement due to contiguity has been described. A high number of patients with IgG4-related GIT disease, especially pseudotumor forms, are diagnosed after surgery due to suspected malignancy. A common finding, although of uncertain significance, is the asymptomatic infiltration of Ig G4+ plasma cells in the gastric mucosa (33–47%), papilla of Vater (55–80%) and, less frequently, in the colon of patients with type 1 Ig G4-related autoimmune pancreatitis.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Ig G4 plasma cells infiltration is not specific to IgG4-RD and has been observed in various GIT processes such as eosinophilic esophagitis, ulcerative colitis, diverticulitis, and colorectal cancer.</p><p id="par0020" class="elsevierStylePara elsevierViewall">IgG4-RD is not without diagnostic difficulty, requiring the combination of laboratory and histological criteria and imaging tests. However, study findings often require a differential diagnosis with inflammatory, infectious, and malignant diseases. Unusual sites, isolated involvement, and poor knowledge of the disease are other factors that make diagnosis difficult. In the absence of known IgG4-RD and/or manifestations suggestive of the disease, the lack of suspicion is acceptable in view of the endoscopic finding of a polypoid lesion. On the contrary, in the presence of stenosing lesions of uncertain origin and especially submucosal tumours whose differential diagnosis includes neuroendocrine tumours, gastrointestinal stromal and muscle layer tumours, IgG4-RD should be considered.</p><p id="par0025" class="elsevierStylePara elsevierViewall">Every new disease implies a reasonable period of learning. Possibly GIT IgG4-RD, despite its uncommon detection, has a sufficiently well-defined clinical spectrum to be considered in the differential diagnosis of various GIT processes so as to avoid diagnostic errors and unnecessary surgical procedures which are not free of morbidity. The knowledge of this systemic disease, basic competence of the Internal Medicine department, should be extended to various specialties such as surgery, digestive and anatomical pathology, considering the diagnosis in the light of the different clinical forms described and/or inconclusive histological anomalies, but with the presence of any of the pathologic peculiarities of the disease.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Torné Cachot J, García Pont J. Enfermedad del tracto gastrointestinal relacionada con la Ig G4. Med Clin (Barc). 2021;156:95–96.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Enfermedad relacionada con la inmunoglobulina G4: variabilidad del espectro clínico" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "O. Lorente" 1 => "A. Rueda" 2 => "C. Campos" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.medcli.2018.10.032" "Revista" => array:6 [ "tituloSerie" => "Med Clin (Barc)." 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"fecha" => "2013" "volumen" => "19" "paginaInicial" => "5769" "paginaFinal" => "5774" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/24124321" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/23870206/0000015600000002/v2_202102250946/S2387020620306239/v2_202102250946/en/main.assets" "Apartado" => array:4 [ "identificador" => "43309" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Letters to the Editor" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/23870206/0000015600000002/v2_202102250946/S2387020620306239/v2_202102250946/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2387020620306239?idApp=UINPBA00004N" ]
