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• W2178952813 abstract "Primary Sjögren syndrome (PSS) is a chronic inflammatory autoimmune disease. Interstitial lung disease (ILD) can be an extraglandular complication. To evaluate the clinical characteristics of patients diagnosed with PSS with ILD. Multicentre cohort study with 25 patients diagnosed with PSS and ILD. Data of PSS, prognostic factors, pulmonary involvement variables, complementary tests that suggest a worse diagnosis and treatment given were collected. EULAR index was measured for Sjögren's syndrome. We identified 25 patients. In 15/25 the diagnosis of ILD was done before the diagnosis of PSS. The histopathological patterns found were: 12 NSIP, 5 UIP, 4 OP, 2 LIP. PFRs showed restrictive pattern. The majority of the patients received glucocorticoid therapy, antimalarial or immunosuppressive treatment. Patients affected with PSS must be screened to catch a precocious diagnosis of ILD. The majority of the patients were diagnosed of ILD before being diagnosed of PSS. Multicenter cohorts are increasingly demanded and a multidisciplinary management is needed. El síndrome de Sjögren primario (SSP) es una enfermedad inflamatoria autoinmune. La enfermedad pulmonar intersticial (EPI) puede ser una complicación extraglandular. Evaluar las características clínicas de los pacientes diagnosticados de SSP con EPI. Estudio de cohortes multicéntrico con 25 pacientes diagnosticados de SSP y EPI. Se recopilaron datos propios del SSP, factores pronóstico, variables de medida de la afectación pulmonar, pruebas complementarias que sugieren un peor pronóstico, así como el tratamiento recibido. Se calculó el índice EULAR para el síndrome de Sjögren. Se identificaron 25 pacientes. Quince de ellos fueron diagnosticados de EPI antes que de SSP. Los patrones histopatológicos encontrados fueron 12 con neumonía intersticial inespecífica, 5 con neumonía intersticial común, 4 con neumonía organizada, 2 con neumonía intersticial linfocítica. Las pruebas de función respiratoria mostraron un patrón restrictivo. La mayoría de los pacientes recibió un tratamiento con glucocorticoides, antipalúdicos o inmunodepresores. Los pacientes afectados por SSP deben ser sometidos a pruebas para detectar un diagnóstico precoz de EPI. La mayoría de los pacientes fueron diagnosticados de EPI antes que de SSP. Los estudios de cohortes multicéntricos son cada vez más demandados y se precisa una gestión multidisciplinar." @default.
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• W2178952813 date "2016-07-01" @default.
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• W2178952813 title "Sjögren's syndrome-associated interstitial lung disease: A multicenter study" @default.
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