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Se aprecian además bronquiectasias de tracción y bronquiolectasias (c). En el momento actual no presenta panalización.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Ester Zamarrón-de Lucas, Luis Gómez Carrera, Gema Bonilla, Dessiree Petit, Alberto Mangas, Rodolfo Álvarez-Sala" "autores" => array:6 [ 0 => array:2 [ "nombre" => "Ester" "apellidos" => "Zamarrón-de Lucas" ] 1 => array:2 [ "nombre" => "Luis" "apellidos" => "Gómez Carrera" ] 2 => array:2 [ "nombre" => "Gema" "apellidos" => "Bonilla" ] 3 => array:2 [ "nombre" => "Dessiree" "apellidos" => "Petit" ] 4 => array:2 [ "nombre" => "Alberto" "apellidos" => "Mangas" ] 5 => array:2 [ "nombre" => "Rodolfo" "apellidos" => "Álvarez-Sala" ] ] ] ] ] "idiomaDefecto" => "es" "Traduccion" => array:1 [ "en" => array:9 [ "pii" => "S2387020617301316" "doi" => "10.1016/j.medcle.2017.02.018" "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/S2387020617301316?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0025775316306340?idApp=UINPBA00004N" "url" => "/00257753/0000014800000004/v1_201702040018/S0025775316306340/v1_201702040018/es/main.assets" ] ] "itemSiguiente" => array:19 [ "pii" => "S2387020617301286" "issn" => "23870206" "doi" => "10.1016/j.medcle.2016.11.035" "estado" => "S300" "fechaPublicacion" => "2017-02-23" "aid" => "3851" "copyright" => "Elsevier España, S.L.U." "documento" => "article" "crossmark" => 1 "subdocumento" => "rev" "cita" => "Med Clin. 2017;148:170-5" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:2 [ "total" => 2 "HTML" => 2 ] "en" => array:13 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Review</span>" "titulo" => "Idiopathic pulmonary fibrosis" "tienePdf" => "en" "tieneTextoCompleto" => "en" "tieneResumen" => array:2 [ 0 => "en" 1 => "es" ] "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "170" "paginaFinal" => "175" ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Fibrosis pulmonar idiopática" ] ] "contieneResumen" => array:2 [ "en" => true "es" => true ] "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" => 713 "Ancho" => 950 "Tamanyo" => 157289 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">HRCT. Pattern of usual interstitial pneumonia. Reticular images and subpleural, bibasilar and peripheral honeycomb changes.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Antoni Xaubet, Julio Ancochea, María Molina-Molina" "autores" => array:3 [ 0 => array:2 [ "nombre" => "Antoni" "apellidos" => "Xaubet" ] 1 => array:2 [ "nombre" => "Julio" "apellidos" => "Ancochea" ] 2 => array:2 [ "nombre" => "María" "apellidos" => "Molina-Molina" ] ] ] ] ] "idiomaDefecto" => "en" "Traduccion" => array:1 [ "es" => array:9 [ "pii" => "S0025775316305772" "doi" => "10.1016/j.medcli.2016.11.004" "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/S0025775316305772?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2387020617301286?idApp=UINPBA00004N" "url" => "/23870206/0000014800000004/v1_201703290102/S2387020617301286/v1_201703290102/en/main.assets" ] "itemAnterior" => array:19 [ "pii" => "S2387020617301353" "issn" => "23870206" "doi" => "10.1016/j.medcle.2017.02.021" "estado" => "S300" "fechaPublicacion" => "2017-02-23" "aid" => "3889" "copyright" => "Elsevier España, S.L.U." 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Traction bronchiectasis and bronchiolectasis (c) are also observed. No honeycombing is observed at present.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">The antisynthetase syndrome (ASS), first described by Marguerie et al. in 1990,<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">1</span></a> is an autoimmune-origin clinical entity belonging to the group of inflammatory myopathies. The annual incidence of inflammatory myopathies in Spain is only 8–9 cases/million inhabitants, and ASS represents 30% of these.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">2</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">It is characterized by a series of clinical manifestations such as myositis, fever, mechanic's hands and interstitial lung disease (ILD), all associated with positivity to antisynthetase antibodies (ASA). The presence of ASA is related to a higher frequency of ILD (up to 90%) and it is the presence of these that will largely determine the prognosis and treatment for these patients.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">3</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">Pulmonary involvement usually occurs in a subacute-chronic degree, as in cryptogenic organizing pneumonia or, more frequently, nonspecific interstitial pneumonia (NSIP) pneumonia. In addition, it may precede myopathy or it may occur after it or even develop without it; hence the importance of ASA determination in the differential diagnosis of ILD, since its positivity can predict the onset of other ASS-specific clinical characteristics during follow-up.<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">4</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">In the medical literature, there are few reported cases of pulmonary involvement in patients with ASS.<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">5</span></a> Therefore, we report a series of 11 patients with ASS under follow-up by the Pneumology service of a tertiary hospital.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">Clinical observation</span><p id="par0025" class="elsevierStylePara elsevierViewall">11 patients diagnosed of ASS with lung involvement, with an average age of 59 years and female prevalence (81%). Follow-up time includes from 2000 to 2015, inclusive.</p><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0075">Symptoms onset</span><p id="par0030" class="elsevierStylePara elsevierViewall">Seven patients were diagnosed with ASS during the study of systemic symptoms (arthralgia, myopathy and/or mechanic's hands). Three began as febrile syndrome of unknown origin. Only one patient started with respiratory symptoms (chronic cough). A radiological pattern of interstitial lung disease was observed in all of them during the study of the said symptoms by high-resolution computed tomography (HRCT), meeting the criteria for requesting anti-Jo antibodies. If there is a high suspicion of negative anti-Jo ASS, it is expanded to anti-PL12 and PL7. Given the findings, the patient is referred to the monographic interstitial lung disease and collagen disease service.</p><p id="par0035" class="elsevierStylePara elsevierViewall">From a systemic point of view, 6 (55%) patients had joint involvement (arthralgia, mainly proximal), 6 (55%), muscle (weakness or muscle pain, 2 of them diagnosed with dermatomyositis), 5 (45%), mechanic's hands, and 3 (27%), Raynaud's phenomenon.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0080">Antisynthetase antibodies</span><p id="par0040" class="elsevierStylePara elsevierViewall">9 (81%) were positive for anti-Jo and 2 (19%) for anti-PL12. The most frequent non-antisynthetase antibodies were antinuclear antibodies (ANA) in 7 patients, rheumatoid factor in 3 patients and anti-Ro/SSA in 2 patients. Other immune-origin pathologies were ruled out in all of them, as well as drug-induced causes or exposure to organic and inorganic powders.</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0085">Radiology</span><p id="par0045" class="elsevierStylePara elsevierViewall">All showed bilateral involvement, apicobasal gradient and subpleural predominance with intralobular interstitial thickening on HRCT. 73% were accompanied by traction bronchiectasis and bronchiolectasis and 27% had signs of honeycombing. A pattern consistent with NSIP (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>) was observed in 4 patients (36%), with usual interstitial pneumonia in 4 (36%), (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>), with cryptogenic obliterans pneumonia in one (10%), (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>), and with ground-glass opacities in 2, not currently compatible with a specific radiological pattern.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0090">Lung function and echocardiographic examination</span><p id="par0050" class="elsevierStylePara elsevierViewall">Functionally, the involvement of the <span class="elsevierStyleItalic">diffusing capacity of the lungs for carbon monoxide</span> (DLCO) stands out in 54% of patients: 3 with mild decrease, 2 moderate and one severe. 45% had a positive 6-minute walk test (all with decreased DLCO except one, who was normal), which measures the maximum distance the patient can walk in 6<span class="elsevierStyleHsp" style=""></span>min. It is evaluated using reference equations, dyspnoea, heart rate and blood oxygen saturation. It is positive if there is a decrease of 4% in SpO<span class="elsevierStyleInf">2</span>, with the final being below 93%.<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">6</span></a> In 2 patients, mild pulmonary hypertension was observed by echocardiography, stable in clinical follow-up. Both patients had a UIP pattern, one with DLCO of 43% and a positive 6MWT and the other with DLCO of 75%, with the other functional tests within normal limits.</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0095">Treatment</span><p id="par0055" class="elsevierStylePara elsevierViewall">Eight patients continued active chronic steroid therapy, with or without immunosuppressant agent, azathioprine being the most frequent (72%). Followed, in order of frequency, by hydroxychloroquine, cyclophosphamide, rituximab (27% each), mycophenolate mofetil and methotrexate (18% each).</p><p id="par0060" class="elsevierStylePara elsevierViewall">Intravenous immunoglobulins were only used in one patient due to poor response to prior therapy (azathioprine, rituximab and cyclophosphamide), with good response, which made possible to down-titrate the medication.</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0100">Prognosis</span><p id="par0065" class="elsevierStylePara elsevierViewall">All patients maintained a stable or slowly progressive clinical course (radiologic or functional) with the treatment. Periods of exacerbation were treated by up-titration of immunosuppressive therapy, with good response. No patient has died, neither has anyone developed neoplasia or required Intensive Care Unit admission or ventilatory support to date.</p></span></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0105">Discussion</span><p id="par0070" class="elsevierStylePara elsevierViewall">The relationship between ASS and lung disease has been described in several series.<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">3,7</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">Our series included 11 cases of ASS, all with ILD. Pulmonary involvement in these patients has developed both before and after myopathy, and even without it, hence the need for ASA determination in the differential diagnosis of ILD. In addition, ILD is a finding in the study of systemic symptoms in the case of 7 patients without respiratory symptoms.</p><p id="par0080" class="elsevierStylePara elsevierViewall">Fever is frequently the only sign of disease, plays the main role in exacerbations or even at the onset (3 patients in this series). It is an expression of the disease that usually responds to increased immunosuppressive therapy, especially glucocorticoids. On the other hand, it represents a challenge for clinicians to differentiate it from infection, as these patients are usually under immunosuppressive therapy.</p><p id="par0085" class="elsevierStylePara elsevierViewall">Eight types of different ASA have been described so far.<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">4</span></a> We have only found 2 types: anti-Jo (81%), the most common in the literature, and anti-PL12 (19%). We cannot compare the frequency of pulmonary involvement described according to anti-Jo and anti-PL12 positivity, since we must keep in mind that all our patients are from a cohort selected from the monographic “interstitial lung disease and collagen disease” service. However, there is no clear difference regarding lung involvement positivity between the two.</p><p id="par0090" class="elsevierStylePara elsevierViewall">Hervier et al.,<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">8</span></a> in a multicenter study of 233 patients divided according to ASA subtypes, found that patients with anti-PL17 and anti-PL12, compared with those with anti-Jo, had ILD more often (80 and 87% versus 67%, respectively) and less frequently myositis (44 and 47% versus 74%). According to these data, there would be a higher frequency of pulmonary involvement in patients with positive anti-PL17 and anti-PL12, compared to those with positive anti-Jo.</p><p id="par0095" class="elsevierStylePara elsevierViewall">ANA-negative results are possible given the cytoplasmic location of these autoantibodies, so, in some cases, the initial ANA screening is insufficient for diagnosis. In anti-Jo-1 positive patients it is important to apply the anti-Ro antibodies determination (60/52<span class="elsevierStyleHsp" style=""></span>kDa).<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">9</span></a> With some frequency (up to 58 and 72%), both coexist (Jo-Ro52 complex) and their presence seems to confer a worse prognosis in connection with pulmonary interstitium involvement. In our series, 2 patients had the said complex, without this resulting in a worse prognosis compared to the rest.</p><p id="par0100" class="elsevierStylePara elsevierViewall">It should be noted that the evaluation of these ASA will help identifying cases of patients incorrectly diagnosed with idiopathic ILD, which clearly have a worse prognosis, especially those with idiopathic pulmonary fibrosis.<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">7</span></a></p><p id="par0105" class="elsevierStylePara elsevierViewall">Unlike other collagen diseases in which pulmonary hypertension are rare, ASS is not a common finding.<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">10</span></a> The cause of the association of pulmonary hypertension and ASS is not well defined. In patients with advanced lung disease, hypoxaemia could be its cause due to pulmonary hypoxic vasoconstriction and vascular remodelling. But this could also occur independently of the occurrence of hypoxaemia and the severity of the same; therefore, we must investigate its presence in case of breathlessness which does not correspond to the data in the respiratory function tests.</p><p id="par0110" class="elsevierStylePara elsevierViewall">As for radiology, we observe a high presence of both bronchiectasis/bronchiolectasis as honeycombing with respect to the data published in other series.<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">10</span></a></p><p id="par0115" class="elsevierStylePara elsevierViewall">A functional examination of these patients is needed for their monitoring and adjustment of immunosuppressive therapy; therefore, we recommended periodic lung function monitoring by DLCO and forced vital capacity (FVC). Both variables correlate with radiographic involvement, so we believe it is appropriate to consider both for monitoring, although FVC is considered a parameter with less variability between different determinations.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">3</span></a></p><p id="par0120" class="elsevierStylePara elsevierViewall">The first line treatment is corticosteroids at doses of 1<span class="elsevierStyleHsp" style=""></span>mg/kg/day.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">3</span></a> The corticodependence of these patients is noteworthy, which suggests that, frequently coinciding with a dose reduction as well as during worsening periods, the association of other immunosuppressant drugs such as azathioprine, methotrexate, cyclophosphamide, cyclosporine, mycophenolate mofetil, rituximab or tacrolimus could be necessary. The response to immunomodulatory treatment will vary and its choice will vary depending on the systemic involvement, side effects, centre's experience and response to the previously prescribed treatment.</p><p id="par0125" class="elsevierStylePara elsevierViewall">We consider it necessary to stress the lack of studies specifically evaluating treatment of this disease, which complicates the feasibility of a standardized treatment algorithm.</p><p id="par0130" class="elsevierStylePara elsevierViewall">In our experience, patients maintained clinical stability or slow progression with treatment, presenting a good prognosis, better than in other series,<a class="elsevierStyleCrossRefs" href="#bib0080"><span class="elsevierStyleSup">5,11</span></a> probably because of the short period of disease in many of our patients (7 had been diagnosed less than 10 years before).</p><p id="par0135" class="elsevierStylePara elsevierViewall">This contrasts with the chronic course of idiopathic pulmonary fibrosis, where evolution is progressive, with a short life expectancy.</p><p id="par0140" class="elsevierStylePara elsevierViewall">We can conclude by highlighting the need for ASA screening in patients with ILD given the different prognosis and treatment. More subtypes of ASA could be discovered in the future.</p><p id="par0145" class="elsevierStylePara elsevierViewall">More studies are needed to better understand the relationship between histological, radiological and response-to-treatment patterns in order to optimize and standardized these findings.</p></span><span id="sec0050" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0110">Conflict of interests</span><p id="par0150" class="elsevierStylePara elsevierViewall">The authors declare no conflict of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:9 [ 0 => array:3 [ "identificador" => "xres821199" "titulo" => "Abstract" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0005" "titulo" => "Introduction" ] 1 => array:2 [ "identificador" => "abst0010" "titulo" => "Patients and methods" ] 2 => array:2 [ "identificador" => "abst0015" "titulo" => "Results" ] 3 => array:2 [ "identificador" => "abst0020" "titulo" => "Conclusions" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec818200" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres821198" "titulo" => "Resumen" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0025" "titulo" => "Introducción" ] 1 => array:2 [ "identificador" => "abst0030" "titulo" => "Pacientes y métodos" ] 2 => array:2 [ "identificador" => "abst0035" "titulo" => "Resultados" ] 3 => array:2 [ "identificador" => "abst0040" "titulo" => "Conclusiones" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec818201" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:3 [ "identificador" => "sec0010" "titulo" => "Clinical observation" "secciones" => array:6 [ 0 => array:2 [ "identificador" => "sec0015" "titulo" => "Symptoms onset" ] 1 => array:2 [ "identificador" => "sec0020" "titulo" => "Antisynthetase antibodies" ] 2 => array:2 [ "identificador" => "sec0025" "titulo" => "Radiology" ] 3 => array:2 [ "identificador" => "sec0030" "titulo" => "Lung function and echocardiographic examination" ] 4 => array:2 [ "identificador" => "sec0035" "titulo" => "Treatment" ] 5 => array:2 [ "identificador" => "sec0040" "titulo" => "Prognosis" ] ] ] 6 => array:2 [ "identificador" => "sec0045" "titulo" => "Discussion" ] 7 => array:2 [ "identificador" => "sec0050" "titulo" => "Conflict of interests" ] 8 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2016-06-15" "fechaAceptado" => "2016-11-03" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec818200" "palabras" => array:3 [ 0 => "Antisynthetase antibodies" 1 => "Lung interstitial disease" 2 => "Myositis" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec818201" "palabras" => array:3 [ 0 => "Anticuerpos antisintetasa" 1 => "Enfermedad pulmonar intersticial" 2 => "Miositis" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:3 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Introduction</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Antisynthetase syndrome (ASS) is characterized by a series of clinical manifestations such as myositis, fever, mechanic's hands and diffuse interstitial lung disease (ILD), all associated with positivity to antisynthetase antibodies. The presence of ILD will be that, to a great extent it will mark the response to treatment and prognosis.</p></span> <span id="abst0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Patients and methods</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Eleven cases of patients with ASS and pulmonary involvement in monitoring at a Pulmonary monographic consult in a third level hospital consult are described.</p></span> <span id="abst0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Results</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Nine patients presented positivity to anti-Jo antibody and 2 to anti-PL12. Four patients’ HRCT pattern showed NSIP, four UIP, one COP and 2 ground-glass opacity. A percentage of 73 were accompanied by bronchiectasis and bronchiolectasis and 27% honeycombing. Functional exploration was mainly affected by DLCO with up to 45% of the positive walking test. Cortico-dependence is highlighted, often requiring immunosuppressive treatment both chronically and in exacerbations. All patients maintain good prognosis so far.</p></span> <span id="abst0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Conclusions</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Patients with interstitial lung disease should have at least a determination of antisynthetase antibodies in order to identify this disease, better prognosis than other interstitial diseases such as idiopathic pulmonary fibrosis.</p></span>" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0005" "titulo" => "Introduction" ] 1 => array:2 [ "identificador" => "abst0010" "titulo" => "Patients and methods" ] 2 => array:2 [ "identificador" => "abst0015" "titulo" => "Results" ] 3 => array:2 [ "identificador" => "abst0020" "titulo" => "Conclusions" ] ] ] "es" => array:3 [ "titulo" => "Resumen" "resumen" => "<span id="abst0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Introducción</span><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">El síndrome antisintetasa (ASA) se caracteriza por manifestaciones clínicas como miositis, fiebre, manos de mecánico y enfermedad pulmonar intersticial (EPI) asociadas a la positividad ante anticuerpos antisintetasa. La presencia de EPI será la que, en gran medida, marcará la respuesta al tratamiento y el pronóstico.</p></span> <span id="abst0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Pacientes y métodos</span><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Se describen 11 pacientes con ASA y afectación pulmonar en seguimiento en una consulta monográfica de Neumología de un hospital de tercer nivel.</p></span> <span id="abst0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Resultados</span><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Nueve pacientes presentaron positividad para anticuerpos anti-Jo y 2 para anti-PL12. Cuatro pacientes mostraron en TCAR patrón NINE, 4 NIU, uno NOC y 2 en vidrio deslustrado. El 73% se acompañaron de bronquiectasias y bronquiolectasias y el 27%, de panalización. Funcionalmente resalta la DLCO, con hasta el 45% con un test de la marcha positivo. Destaca la corticodependencia, con necesidad en muchas ocasiones de tratamiento inmunodepresor, tanto en exacerbaciones como de forma crónica. Todos los pacientes mantuvieron buen pronóstico hasta el momento.</p></span> <span id="abst0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Conclusiones</span><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Los pacientes con EPI deberían tener al menos una determinación de anticuerpos antisintetasa para así identificar esta enfermedad, de mejor pronóstico que otras afecciones intersticiales como la fibrosis pulmonar idiopática.</p></span>" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0025" "titulo" => "Introducción" ] 1 => array:2 [ "identificador" => "abst0030" "titulo" => "Pacientes y métodos" ] 2 => array:2 [ "identificador" => "abst0035" "titulo" => "Resultados" ] 3 => array:2 [ "identificador" => "abst0040" "titulo" => "Conclusiones" ] ] ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Zamarrón-de Lucas E, Gómez Carrera L, Bonilla G, Petit D, Mangas A, Álvarez-Sala R. Síndrome antisintetasa: análisis de 11 casos. Med Clin (Barc). 2017;148:166–169.</p>" ] ] "multimedia" => array:3 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 512 "Ancho" => 1900 "Tamanyo" => 135305 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">Pattern compatible with nonspecific interstitial pneumonia. Predominantly subpleural, bilateral lung involvement is observed with thickening of intralobular interstitium (a), small traction bronchiectasis and bronchiolectasis (b) as well as small areas of ground-glass mainly affecting the lower lobes (c).</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Fig. 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 538 "Ancho" => 1500 "Tamanyo" => 109318 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">Findings indicative of usual interstitial pneumonia: patchy intralobular subpleural thickening (a), with apicobasal gradient and minimal subpleural ground-glass areas (b). Traction bronchiectasis and bronchiolectasis (c) are also observed. No honeycombing is observed at present.</p>" ] ] 2 => array:7 [ "identificador" => "fig0015" "etiqueta" => "Fig. 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 637 "Ancho" => 1894 "Tamanyo" => 147967 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0055" class="elsevierStyleSimplePara elsevierViewall">Likely cryptogenic organizing pneumonia pattern. Predominantly subpleural and peribronchovascular bilateral patchy ground-glass opacities, which do not cause disintegration of the adjacent lung parenchyma (a). Some images of nodular morphology (b) and <span class="elsevierStyleItalic">crazy-paving</span> pattern are also observed (c).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:11 [ 0 => array:3 [ "identificador" => "bib0060" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Polymyositis, pulmonary fibrosis and autoantibodies to aminoacyl-tRNA synthetase enzymes" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "C. Marguerie" 1 => "C.C. Bunn" 2 => "H.L. Beynon" 3 => "R.M. Bernstein" 4 => "J.M. Hughes" 5 => "A.K. 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2023 March | 3 | 0 | 3 |
2020 October | 1 | 1 | 2 |
2017 September | 0 | 1 | 1 |