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Heterogeneous clinical spectrum of interstitial lung disease in patients with anti-EJ anti-synthetase syndrome: a case series

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Abstract

Auto-antibodies against aminoacyl-tRNA-synthetases (anti-ARS Abs) represent the hallmark of the anti-synthetase syndrome that is defined as the clinical association of fever, Raynaud’s phenomenon, myositis, interstitial lung disease (ILD), arthritis and mechanic’s hands. Recently, differences in clinical features depending on specific anti-ARS Abs have been reported. We describe three cases of anti-EJ (anti-glycyl) antibody-positive patients presenting with ILD as a common feature, but with heterogeneous histopathological and radiographic patterns and with different responses to treatment. Relapsing-remittent fever, refractory muscle involvement and seronegative arthritis were also striking clinical manifestations.

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    Authors and Affiliations

    1. Rheumatology Unit—DIM, University of Bari, P.zza G Cesare 11, 70124, Bari, Italy

      Margherita Giannini, Giuseppe Lopalco & Florenzo Iannone

    2. Rheumatology Unit, Department of Medicine, Solna, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden

      Antonella Notarnicola, Maryam Dastmalchi & Ingrid E. Lundberg

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    1. Margherita Giannini
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    2. Antonella Notarnicola
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    3. Maryam Dastmalchi
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    4. Ingrid E. Lundberg
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    5. Giuseppe Lopalco
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    6. Florenzo Iannone
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    Correspondence to Florenzo Iannone.

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    Margherita Giannini and Antonella Notarnicola contributed equally to this work.

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    Giannini, M., Notarnicola, A., Dastmalchi, M. et al. Heterogeneous clinical spectrum of interstitial lung disease in patients with anti-EJ anti-synthetase syndrome: a case series. Clin Rheumatol 35, 2363–2367 (2016). https://doi.org/10.1007/s10067-016-3258-1

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    • DOI: https://doi.org/10.1007/s10067-016-3258-1

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