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Inicio Medicina Clínica (English Edition) Antisynthetase syndrome: Analysis of 11 cases
Journal Information
Vol. 148. Issue 4.
Pages 166-169 (February 2017)
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Vol. 148. Issue 4.
Pages 166-169 (February 2017)
Clinical notes
Antisynthetase syndrome: Analysis of 11 cases
Síndrome antisintetasa: análisis de 11 casos
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6
Ester Zamarrón-de Lucasa,
Corresponding author
ester.zamarron@gmail.com

Corresponding author.
, Luis Gómez Carreraa, Gema Bonillab, Dessiree Petita, Alberto Mangasa, Rodolfo Álvarez-Salaa
a Servicio de Neumología, Hospital Universitario La Paz, Instituto de Investigación Hospital Universitario La Paz (IdiPAZ), Centro de Investigación Biomédica en Red de Enfermedades Respiratorias (CIBERES), Universidad Autónoma de Madrid, Madrid, Spain
b Servicio de Reumatología, Hospital Universitario La Paz, Instituto de Investigación Hospital Universitario La Paz (IdiPAZ), Centro de Investigación Biomédica en Red de Enfermedades Respiratorias (CIBERES), Universidad Autónoma de Madrid, Madrid, Spain
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Med Clin. 2017;148:164-510.1016/j.medcle.2017.02.021
Albert Selva-O’Callaghan, Ernesto Trallero-Araguás
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Abstract
Introduction

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.

Patients and methods

Eleven cases of patients with ASS and pulmonary involvement in monitoring at a Pulmonary monographic consult in a third level hospital consult are described.

Results

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.

Conclusions

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.

Keywords:
Antisynthetase antibodies
Lung interstitial disease
Myositis
Resumen
Introducción

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.

Pacientes y métodos

Se describen 11 pacientes con ASA y afectación pulmonar en seguimiento en una consulta monográfica de Neumología de un hospital de tercer nivel.

Resultados

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.

Conclusiones

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.

Palabras clave:
Anticuerpos antisintetasa
Enfermedad pulmonar intersticial
Miositis

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