Granulomatous–lymphocytic interstitial lung disease: A pulmonary complication of common variable immunodeficiency

Matesanz López, Cristina; Raboso Moreno, Beatriz; Río Ramírez, María Teresa

doi:10.1016/j.medcli.2023.01.016

array:23 ["pii" => "S0025775323000337" "issn" => "00257753" "doi" => "10.1016/j.medcli.2023.01.016" "estado" => "S300" "fechaPublicacion" => "2023-06-09" "aid" => "6186" "copyright" => "Elsevier España, S.L.U.. All rights reserved" "copyrightAnyo" => "2023" "documento" => "simple-article" "crossmark" => 1 "subdocumento" => "cor" "cita" => "Med Clin. 2023;160:e1-e2" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "itemSiguiente" => array:19 [ "pii" => "S0025775323000362" "issn" => "00257753" "doi" => "10.1016/j.medcli.2023.01.019" "estado" => "S300" "fechaPublicacion" => "2023-06-09" "aid" => "6189" "copyright" => "Elsevier España, S.L.U." "documento" => "simple-article" "crossmark" => 1 "subdocumento" => "cor" "cita" => "Med Clin. 2023;160:e3-e4" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "es" => array:10 [ "idiomaDefecto" => true "cabecera" => "Carta al Editor" "titulo" => "Ictus y apneas del sueño en una familia con arteriopatía cerebral autosómica dominante con infartos subcorticales y leucoencefalopatía (CADASIL)" "tienePdf" => "es" "tieneTextoCompleto" => "es" "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "e3" "paginaFinal" => "e4" ] ] "titulosAlternativos" => array:1 [ "en" => array:1 [ "titulo" => "Stroke and sleep apnea in a family with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)" ] ] "contieneTextoCompleto" => array:1 [ "es" => true ] "contienePdf" => array:1 [ "es" => true ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Ana Domínguez Mayoral, Soledad Pérez Sánchez, Joan Montaner Villalonga" "autores" => array:3 [ 0 => array:2 [ "nombre" => "Ana" "apellidos" => "Domínguez Mayoral" ] 1 => array:2 [ "nombre" => "Soledad" "apellidos" => "Pérez Sánchez" ] 2 => array:2 [ "nombre" => "Joan" "apellidos" => "Montaner Villalonga" ] ] ] ] ] "idiomaDefecto" => "es" "Traduccion" => array:1 [ "en" => array:9 [ "pii" => "S2387020623001833" "doi" => "10.1016/j.medcle.2023.05.004" "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/S2387020623001833?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0025775323000362?idApp=UINPBA00004N" "url" => "/00257753/0000016000000011/v1_202306061651/S0025775323000362/v1_202306061651/es/main.assets" ] "itemAnterior" => array:19 [ "pii" => "S0025775323000933" "issn" => "00257753" "doi" => "10.1016/j.medcli.2023.02.006" "estado" => "S300" "fechaPublicacion" => "2023-06-09" "aid" => "6199" "copyright" => "Elsevier España, S.L.U." "documento" => "article" "crossmark" => 1 "subdocumento" => "rev" "cita" => "Med Clin. 2023;160:501-16" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "es" => array:13 [ "idiomaDefecto" => true "cabecera" => "Revisión" "titulo" => "Antagonistas del factor de necrosis tumoral (TNF) en la enfermedad inflamatoria intestinal pediátrica: Una revisión sistemática" "tienePdf" => "es" "tieneTextoCompleto" => "es" "tieneResumen" => array:2 [ 0 => "es" 1 => "en" ] "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "501" "paginaFinal" => "516" ] ] "titulosAlternativos" => array:1 [ "en" => array:1 [ "titulo" => "Tumour necrosis factor (TNF) antagonist therapy for paediatric inflammatory bowel disease: A systematic review" ] ] "contieneResumen" => array:2 [ "es" => true "en" => true ] "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" => 970 "Ancho" => 2508 "Tamanyo" => 279881 ] ] "descripcion" => array:1 [ "es" => "Remisión y respuesta clínica del tratamiento de mantenimiento con anti-TNF en enfermedad de Crohn." ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Paula Martín-García, Adolfo Alonso-Arroyo, Ferrán Catalá-López" "autores" => array:3 [ 0 => array:2 [ "nombre" => "Paula" "apellidos" => "Martín-García" ] 1 => array:2 [ "nombre" => "Adolfo" "apellidos" => "Alonso-Arroyo" ] 2 => array:2 [ "nombre" => "Ferrán" "apellidos" => "Catalá-López" ] ] ] ] ] "idiomaDefecto" => "es" "Traduccion" => array:1 [ "en" => array:9 [ "pii" => "S2387020623001857" "doi" => "10.1016/j.medcle.2023.02.001" "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/S2387020623001857?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0025775323000933?idApp=UINPBA00004N" "url" => "/00257753/0000016000000011/v1_202306061651/S0025775323000933/v1_202306061651/es/main.assets" ] "en" => array:15 [ "idiomaDefecto" => true "cabecera" => "Letter to the Editor" "titulo" => "Granulomatous–lymphocytic interstitial lung disease: A pulmonary complication of common variable immunodeficiency" "tieneTextoCompleto" => true "saludo" => "Dear Editor," "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "e1" "paginaFinal" => "e2" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Cristina Matesanz López, Beatriz Raboso Moreno, María Teresa Río Ramírez" "autores" => array:3 [ 0 => array:4 [ "nombre" => "Cristina" "apellidos" => "Matesanz López" "email" => array:1 [ 0 => "Cristina.matesanz@salud.madrid.org" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "*" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "Beatriz" "apellidos" => "Raboso Moreno" ] 2 => array:2 [ "nombre" => "María Teresa" "apellidos" => "Río Ramírez" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Servicio de Neumología, Hospital Universitario de Getafe, Madrid, Spain" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Enfermedad pulmonar intersticial linfocítica granulomatosa: una complicación pulmonar de la inmunodeficiencia común variable" ] ] "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" => 373 "Ancho" => 1005 "Tamanyo" => 94561 ] ] "descripcion" => array:1 [ "en" => "The first picture shows a dense interstitial-septal lymphoplasmacytic infiltrate surrounding alveoli (some alveoli are marked with yellow stars) (HE, 20×). The second picture shows a chest CT with nodules (yellow arrow), lymphadenopathies (red arrow) and small bronchiectasis (blue triangle)." ] ] ] "textoCompleto" => "Common variable immunodeficiency (CVID) is a primary immunodeficiency disorder characterized by loss of B-cell function and defective immunoglobulin production.Pulmonary complication is one of the most frequent. The most common finding is bronchiectasis, although granulomatous–lymphocytic interstitial lung disease (GLILD) is also possible.<a class="elsevierStyleCrossRef" href="#bib0030">1</a>Case 1A 26-year-old male with a history of CVID and recurrent respiratory infections, presented with pulmonary bilateral nodules, interstitial thickening, and lymphadenopathy. A core needle biopsy was carried out, showing a lymphoid infiltrate with a CD3-CD4 predominance, as also a minimally granulomatous reaction.During the first years of following-up, a decline in lung function and worsen of symptoms were observed, what motivated the initiation of treatment with prednisone 0.5mg/kg. There was a significant radiological and clinical improvement, which allowed a dose reduction. Complete withdrawal of glucocorticoesteroids was not possible.In addition, he contracted COVID-19 with a favorable evolution.Case 2A 22-year-old male, with a CVID diagnosis, had several respiratory and ear, nose and throat (ENT) infections. Due to this, a thorax high-resolution computed tomography (HRCT) was carried out, which showed hilar and mediastinal lymph nodes with basal and peribronchial nodular opacities. Considering these findings, a core needle biopsy was performed with the initial diagnosis of organizing pneumonia.However, the evolution of the organizing pneumonia was not typical, so a second core needle biopsy was considered. On this occasion, lung parenchima showed an interstitial inflammatory infiltrate with T cell, predominantly CD3+ and CD4+, apart from patchy areas of collagenized fibrosis. Masson bodies weren’t observed. GLILD diagnosis was established (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>).<elsevierMultimedia ident="fig0005"></elsevierMultimedia>Case 3A 31-year-old woman was diagnosed with CVID due to recurrent sinusitis and mums. A chest HRCT was carried out. It showed hilar and mediastinal adenopathies, bronchiectasis and small nodules. A bronchoscopy was subsequently performed. The BAL was normal but the BTT was also described as non-necrotizing granulomatosis.With these findings, she was diagnosed with GLILD and corticosteroid treatment was started. The lung parenchima improved and she remained asymptomatic.However, after 12 years of follow-up, she died due to a non-Hodgkin's lymphoma.DiscussionIn 2017, the British Lung Foundation/United Kingdom Primary Immunodeficiency Network made a consensus statement<a class="elsevierStyleCrossRef" href="#bib0035">2</a> where GLILD was defined as “a distinct clinico-radio-pathological ILD occurring in patients with CVID, associated with a lymphocytic infiltrate and/or granuloma in the lung, and in whom other conditions have been considered and, where possible, excluded.”<a class="elsevierStyleCrossRef" href="#bib0040">3</a>GLILD is described in 10–30% of patients with CVID, without differences between gender, and it's less common in Blacks.It is complex to reach the diagnosis, since symptoms are non-specific and it is only suspected in the presence of radiological alterations. This happened in the three cases that we present.HRCT is the gold standard imaging technique. Sometimes, making the differential diagnosis between GLILD and sarcoidosis can be a challenge. Nodules in GLILD are usually bigger (>1cm) with random or predominantly basal distribution. Hilar lymph nodes can be present in both diseases. The role of PET-CT is being studied to monitor response to treatment and to rule out other organ involvement.<a class="elsevierStyleCrossRef" href="#bib0045">4</a>The utility of trans-bronchial biopsy or cryobiopsy is not well defined, although one of our patients was diagnosed thanks to it.Histology of GLILD is very heterogeneous. Findings include lymphoid hyperplasia, granulomata, organizing pneumonia and pulmonary fibrosis, as described in the cases presented.An increased risk of malignancies is also reported in CVID. Mainly Non-Hodgkin lymphomas, followed by gastric cancers.The decision about treatment generally relies upon a combination of clinical and functional parameters. If the patient is asymptomatic and lung function is normal, specific treatment is not recommended.When a worsening is observed, it is complex to establish a therapeutic algorithm due to a lack of studies. IgG replacement seems to be the first approach, followed by corticosteroids. Biologicals have also been used in this pathology. However, there is still a lack of solid evidence in order to choose the best option.<a class="elsevierStyleCrossRef" href="#bib0050">5</a>We present three different cases with unlike evolution so far. It is, therefore, a compilation of three cases of a rare disease. The main objective is to highlight the importance of early diagnosis and the need to carry out more studies.Informed consentInformed consent has not been collected for this article, since the personal data of our patients is not included.FinancingThere was no funding for this article.Conflict of interestThere are no conflicts of interest." "textoCompletoSecciones" => array:1 [ "secciones" => array:8 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Case 1" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Case 2" ] 2 => array:2 [ "identificador" => "sec0015" "titulo" => "Case 3" ] 3 => array:2 [ "identificador" => "sec0020" "titulo" => "Discussion" ] 4 => array:2 [ "identificador" => "sec0025" "titulo" => "Informed consent" ] 5 => array:2 [ "identificador" => "sec0030" "titulo" => "Financing" ] 6 => array:2 [ "identificador" => "sec0035" "titulo" => "Conflict of interest" ] 7 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "multimedia" => array:1 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 373 "Ancho" => 1005 "Tamanyo" => 94561 ] ] "descripcion" => array:1 [ "en" => "The first picture shows a dense interstitial-septal lymphoplasmacytic infiltrate surrounding alveoli (some alveoli are marked with yellow stars) (HE, 20×). The second picture shows a chest CT with nodules (yellow arrow), lymphadenopathies (red arrow) and small bronchiectasis (blue triangle)." ] ] ] "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" => "Progression of common variable immunodeficiency interstitial lung disease accompanies distinct pulmonary and laboratory findings" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "P.J. Maglione" 1 => "J.R. Overbey" 2 => "C. Cunningham-Rundles" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.jaip.2015.07.004" "Revista" => array:6 [ "tituloSerie" => "J Allergy Clin Immunol Pract" "fecha" => "2015" "volumen" => "3" "paginaInicial" => "941" "paginaFinal" => "950" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/26372540" "web" => "Medline" ] ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0035" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "British Lung Foundation/United Kingdom primary immunodeficiency network consensus statement on the definition, diagnosis, and management of granulomatous–lymphocytic interstitial lung disease in common variable immunodeficiency disorders" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "J.R. Hurst" 1 => "N. Verma" 2 => "D. Lowe" 3 => "H.E. Baxendale" 4 => "S. Jolles" 5 => "P. Kelleher" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.jaip.2017.01.021" "Revista" => array:5 [ "tituloSerie" => "J Allergy Clin Immunol Pract" "fecha" => "2017" "volumen" => "5" "paginaInicial" => "938" "paginaFinal" => "945" ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0040" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Granulomatouslymphocytic lung disease shortens survival in common variable immunodeficiency" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "C.A. Bates" 1 => "M.C. Ellison" 2 => "D.A. Lynch" 3 => "C.D. Cool" 4 => "K.K. Brown" 5 => "J.M. Routes" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.jaci.2004.05.057" "Revista" => array:6 [ "tituloSerie" => "J Allergy Clin Immunol" "fecha" => "2004" "volumen" => "114" "paginaInicial" => "415" "paginaFinal" => "421" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/15316526" "web" => "Medline" ] ] ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0045" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "FDG PET-CT imaging of therapeutic response in granulomatous lymphocytic interstitial lung disease (GLILD) in common variable immunodeficiency (CVID)" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "S. Jolles" 1 => "E. Carne" 2 => "M. Brouns" 3 => "T. El-Shanawany" 4 => "P. Williams" 5 => "C. Marshall" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1111/cei.12856" "Revista" => array:7 [ "tituloSerie" => "Clin Exp Immunol" "fecha" => "2017" "volumen" => "187" "paginaInicial" => "138" "paginaFinal" => "145" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/27896807" "web" => "Medline" ] ] "itemHostRev" => array:3 [ "pii" => "S0732889315001510" "estado" => "S300" "issn" => "07328893" ] ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib0050" "etiqueta" => "5" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Treatment strategies for GLILD in common variable immunodeficiency: a systematic review" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "O.A.C. Lamers" 1 => "B.M. Smits" 2 => "H.L. Leavis" 3 => "G.J. de Bree" 4 => "C. Cunningham-Rundles" 5 => "V.A.S.H. Dalm" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.3389/fimmu.2021.606099" "Revista" => array:4 [ "tituloSerie" => "Front Immunol" "fecha" => "2021" "volumen" => "15" "paginaInicial" => "606099" ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/00257753/0000016000000011/v1_202306061651/S0025775323000337/v1_202306061651/en/main.assets" "Apartado" => array:4 [ "identificador" => "66430" "tipo" => "SECCION" "es" => array:2 [ "titulo" => "Cartas al Editor" "idiomaDefecto" => true ] "idiomaDefecto" => "es" ] "PDF" => "https://static.elsevier.es/multimedia/00257753/0000016000000011/v1_202306061651/S0025775323000337/v1_202306061651/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0025775323000337?idApp=UINPBA00004N"]

ISSN: 0025-7753

Medicina Clínica, fundada en 1943, es la única publicación semanal de contenido clínico que se edita en España y constituye el máximo exponente de la calidad y pujanza de la medicina española. Son características fundamentales de esta publicación el rigor científico y metodológico de sus artículos, la actualidad de los temas y, sobre todo, su sentido práctico, buscando siempre que la información sea de la mayor utilidad en la práctica clínica. Los contenidos de Medicina Clínica abarcan dos frentes: trabajos de investigación original rigurosamente seleccionados atendiendo a su calidad, originalidad e interés, y trabajos orientados a la formación continuada, encomendados por la revista a autores relevantes (Editoriales, Revisiones, Conferencias clínicas y clínico-patológicas, Diagnóstico y Tratamiento). En estos artículos se ponen al día aspectos de destacado interés clínico o conceptual en la medicina actual. Medicina Clínica es un vehículo de información científica de reconocida calidad, como demuestra su inclusión en los más prestigiosos y selectivos índices bibliográficos del mundo.

Ver más Opción Open Access

Indexada en:

Current Contents/Clinical Medicine, Journal Citation Reports, SCI-Expanded, Index Medicus/Medline, Excerpta Medica/EMBASE, IBECS, IME, MEDES, PASCAL, SCOPUS, ScienceDirect