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A propósito de un caso" ] ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:8 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1712 "Ancho" => 2500 "Tamanyo" => 275610 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">a) Bilateral, diffuse, micronodular pulmonary pattern, with some perilymphatic nodules; b) mediastinal adenopathies; and c) homogeneous hepatomegaly, splenomegaly, and pathological adenopathies in the hepatic hilum and the gastrohepatic ligament.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Diana Esteller, Laura Llansó, Ona Escoda" "autores" => array:3 [ 0 => array:2 [ "nombre" => "Diana" "apellidos" => "Esteller" ] 1 => array:2 [ "nombre" => "Laura" "apellidos" => "Llansó" ] 2 => array:2 [ "nombre" => "Ona" "apellidos" => "Escoda" ] ] ] ] ] "idiomaDefecto" => "en" "Traduccion" => array:1 [ "es" => array:9 [ "pii" => "S002577532030107X" "doi" => "10.1016/j.medcli.2020.01.014" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "es" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S002577532030107X?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S238702062100139X?idApp=UINPBA00004N" "url" => "/23870206/0000015600000008/v1_202104180843/S238702062100139X/v1_202104180843/en/main.assets" ] "en" => array:16 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Letter to the Editor</span>" "titulo" => "Rapidly progressive interstitial lung disease without cutaneous involvement associated to anti-MDA5 antibodies" "tieneTextoCompleto" => true "saludo" => "Dear Editor:" "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "413" "paginaFinal" => "414" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Marta Chicot, Claudia Valenzuela, Diego A. Rodríguez" "autores" => array:3 [ 0 => array:4 [ "nombre" => "Marta" "apellidos" => "Chicot" "email" => array:1 [ 0 => "martachis@yahoo.es" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "*" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "Claudia" "apellidos" => "Valenzuela" "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] ] 2 => array:3 [ "nombre" => "Diego A." "apellidos" => "Rodríguez" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Unidad de Cuidados Intensivos, Hospital de La Princesa, IIS-Princesa, Madrid, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Neumología, Hospital de La Princesa, IIS-Princesa, Madrid, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Cátedra ROCHE-UAM, EPID-Futuro, Universidad Autónoma de Madrid (UAM), Madrid, Spain" "etiqueta" => "c" "identificador" => "aff0015" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Enfermedad pulmonar intersticial rápidamente progresiva sin afectación cutánea asociada a anticuerpos anti-MDA5" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:8 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 975 "Ancho" => 2500 "Tamanyo" => 148693 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Pulmonary progress during the patient’s stay at the hospitalization ward prior to her admission to the ICU: (A) chest X-ray (posteroanterior projection) showing a diffuse ground-glass interstitial infiltrate, predominantly in the right hemithorax; (B) thoracic CT images revealing the same type of infiltrate, predominantly on the right side.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Acute respiratory failure is a frequent cause of admission to the intensive care unit (ICU) whose etiology must be determined in order to treat it. Autoantibodies against the melanoma differentiation-associated gene 5 (MDA5) have been described in several cohorts of patients with dermatomyositis (DM) and clinically amyopathic dermatomyositis (CADM) associated with a rapidly progressive interstitial lung disease (RP-ILD).<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1–3</span></a> We hereby present a case of RP-ILD due to anti-MDA5 antibodies (Abs) without signs of DM/CADM, concerning which there are very few publications in the scientific literature.<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4,5</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">This case corresponded to a 58-year-old Asian woman who was admitted to the Pulmonology Department with respiratory failure after a one-month history of cough, dyspnea, and weight loss. Empirical antibiotic therapy with ceftriaxone and levofloxacin was started, with imipenem subsequently being added due to the patient’s poor clinical and radiological evolution. Her levels of acute-phase reactants (C-reactive protein [CRP] and procalcitonin) were slightly elevated. The first thoracoabdominal computed tomography (CT) scan performed showed images of bilateral, diffuse, ground-glass infiltrates. A fibrobronchoscopy with a bronchoalveolar lavage (BAL) and bronchial aspirate (BAS) was also performed, detecting a slightly inverted CD4/CD8 ratio and an absence of cells suggestive of malignancy. All virus, bacteria, and fungi cultures, serologies, and polymerase chain reaction (PCR) tests performed were negative. An echocardiogram and abdominal ultrasound also revealed normal findings. Because an autoimmune disorder was suspected, immunological tests were requested and treatment with 6-methylprednisolone (MP) at a dosage of 2 mg/kg/24 h was prescribed. A transbronchial biopsy was also performed, observing an alveolar parenchyma without histological alterations. In addition, a follow-up pulmonary CT scan revealed images of an extensive subcutaneous emphysema associated with pneumomediastinum, possibly related to the transbronchial biopsy performed, as well as increased ground-glass infiltrates, predominantly in the right hemithorax, and small consolidations in the right lung base (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Twenty-one days later, she had to be admitted to the ICU with orotracheal intubation and invasive mechanical ventilation. She subsequently also developed pneumothorax, because of which she had to have a thoracic tube placed on the right side of her chest. Boluses of 1 g of 6-MP were prescribed for three consecutive days, followed by a dosage of 1.5 mg/kg/24 h, without achieving a clinical or radiological improvement.</p><p id="par0020" class="elsevierStylePara elsevierViewall">The patient’s condition continued to fail to improve despite the administration of respiratory optimization maneuvers, and therapy with venovenous extracorporeal membrane oxygenation (vv-ECMO) and ultra-protective mechanical ventilation was consequently started. Moreover, the immunological study was further expanded with a myositis Immunoblot assay. Given the patient’s poor clinical evolution, her immunosuppressive treatment was intensified with intravenous (i.v.) cyclophosphamide 1 g on the fifth day of her stay at the ICU. In spite of this, she developed left-sided pneumothorax and rapidly progressive pulmonary fibrosis. The patient passed away after spending 21 days in the ICU. We subsequently received the results of the follow-up immunological tests, which revealed positivity for anti-MDA5 Abs, a frequent finding in this type of process.</p><p id="par0025" class="elsevierStylePara elsevierViewall">The analysis of these anti-MDA5 antibodies is useful in the diagnosis of myositis, as they are specific to CADM and DM associated with RP-ILD. Interstitial lung disease (ILD) as an initial manifestation of this condition constitutes a diagnostic challenge, particularly in the absence of cutaneous or muscular involvement and in its rapidly progressive form. There are few reports worldwide of this condition in which no manifestations other than pulmonary ones have been observed. Rapidly progressive ILD with positivity for anti-MDA5 antibodies is associated with a mortality rate of approximately 30%–60%.<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4,5</span></a> Biopsy specimens obtained from patients with ILD exhibit an acute and organized form of diffuse alveolar damage. The lung biopsy performed in our case provided little information, possibly due to the fact that it was obtained from a relatively healthy area of the lung.</p><p id="par0030" class="elsevierStylePara elsevierViewall">Even in the absence of previous cutaneous, muscular, or joint involvement, the rapidly progressive evolution of this condition should lead us to suspect this entity and request testing for anti-MDA5 antibodies in the event of any bilateral interstitial pattern with suspected autoimmunity. Rapidly progressive ILD associated with positivity for anti-MDA5 antibodies is more frequent and severe in Asian patients such as ours.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1–3</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">The use of an intensive immunosuppressive (IS) treatment regimen with high doses of glucocorticoids, oral ciclosporin, and/or pulse therapy with i.v. cyclophosphamide has been reported as possibly effective.<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3,4</span></a> Additional IS therapy with tofacitinib and rituximab, or hemoperfusion with polymyxin B, have also been described as useful for the treatment of refractory lung involvement in patients with DM associated with positivity for anti-MDA5 Abs.<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4,5</span></a> Although the efficacy of these therapies must be confirmed in prospective trials, early detection of anti-MDA5 Abs in patients with a similar clinical picture could promote the early use of intensive IS therapy and, therefore, improve its prognosis.</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: Chicot M, Valenzuela C, Rodríguez DA. Enfermedad pulmonar intersticial rápidamente progresiva sin afectación cutánea asociada a anticuerpos anti-MDA5. Med Clin (Barc). 2021;156:413–414.</p>" ] ] "multimedia" => array:1 [ 0 => array:8 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 975 "Ancho" => 2500 "Tamanyo" => 148693 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Pulmonary progress during the patient’s stay at the hospitalization ward prior to her admission to the ICU: (A) chest X-ray (posteroanterior projection) showing a diffuse ground-glass interstitial infiltrate, predominantly in the right hemithorax; (B) thoracic CT images revealing the same type of infiltrate, predominantly on the right side.</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" => "Factors associated with interstitial lung disease in patients with polymyositis and dermatomyositis: a systematic review and meta-analysis" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "L. 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Kashiwada" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.rmcr.2019.01.012" "Revista" => array:6 [ "tituloSerie" => "Respir Med Case Rep" "fecha" => "2019" "volumen" => "26" "paginaInicial" => "193" "paginaFinal" => "196" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/30723666" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] "agradecimientos" => array:1 [ 0 => array:4 [ "identificador" => "xack524776" "titulo" => "Acknowledgments" "texto" => "<p id="par0040" class="elsevierStylePara elsevierViewall">We would like to thank Dr. Santos Castañeda.</p>" "vista" => "all" ] ] ] "idiomaDefecto" => "en" "url" => "/23870206/0000015600000008/v1_202104180843/S2387020621001418/v1_202104180843/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/23870206/0000015600000008/v1_202104180843/S2387020621001418/v1_202104180843/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2387020621001418?idApp=UINPBA00004N" ]
Journal Information
Vol. 156. Issue 8.
Pages 413-414 (April 2021)
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Vol. 156. Issue 8.
Pages 413-414 (April 2021)
Letter to the Editor
Rapidly progressive interstitial lung disease without cutaneous involvement associated to anti-MDA5 antibodies
Enfermedad pulmonar intersticial rápidamente progresiva sin afectación cutánea asociada a anticuerpos anti-MDA5
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