3 years ago

Antisynthetase Syndrome Complicating the Course of Established Case with Rheumatoid Arthritis: A Rare and Under-recognized Overlapping Disease

Yasser Emad, Yasser Ragab, Magdy Abd-elsalam, Johannes J. Rasker

Publication date: Available online 14 August 2018

Source: Reumatología Clínica

Author(s): Yasser Emad, Yasser Ragab, Magdy Abd-Elsalam, Johannes J. Rasker


A 52-year-old male patient developed RA in March 2009 at the age of 43, with symmetric polyarthritis and active synovitis affecting hands, knees, ankles and both feet without symptoms or signs suggestive of extra-articular features. Laboratory investigations showed negative RF and positive anti-CCP antibodies, negative ANA, negative anti-dsDNA antibodies; the X-rays of both hands showed typical erosive changes in RA and fulfilled the new ACR/EULAR (2010) criteria of RA. The patient achieved remission on a combination of DMARDs. He did well until January 2017 when he developed acute onset of progressive chest pain, dyspnea, and acute respiratory failure. High-resolution CT of the lung showed extensive areas of ground glass veiling, and interstitial subpleural infiltrates were found consistent with aggressive interstitial lung disease (ILD). Autoantibodies against extractable nuclear antigens were screened and showed positive results for anti-RO and anti-Jo1 autoantibodies. The positive anti-Jo1was an expression of anti-synthetase syndrome complicating the RA course and explained the rapidly aggressive course of ILD.


Un paciente de 52 años de edad desarrolló artritis reumatoide (AR) en marzo de 2009 a la edad de 43 años, con poliartritis simétrica y sinovitis activa que afecta manos, rodillas, tobillos y ambos pies, sin síntomas o signos sugestivos de características extraarticulares. Las investigaciones de laboratorio mostraron anticuerpos anti-CCP positivos, RF negativo, ANA negativo, anticuerpos anti-dsDNA negativos; los rayos X de ambas manos mostraron cambios erosivos típicos de la AR y cumplieron los nuevos criterios ACR/EULAR (2010) de AR. El paciente logró la remisión con una combinación de DMARD. Le fue bien hasta enero de 2017, cuando desarrolló una aparición aguda de dolor de pecho progresivo y disnea, e insuficiencia respiratoria aguda. La TC de pulmón de alta resolución mostró áreas extensas de velado de vidrio esmerilado y se encontraron infiltrados subpleurales intersticiales consistentes con enfermedad pulmonar intersticial (EPI) agresiva. Los autoanticuerpos contra antígenos nucleares extraíbles se cribaron y mostraron resultados positivos para autoanticuerpos anti-RO y anti-Jo1. El anti-Jo1 positivo fue una expresión del síndrome anti-sintetasa que complica el curso de la AR y explicó el curso rápidamente agresivo de EPI.

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