title: Heterogeneous clinical spectrum of interstitial lung disease in patients with anti-EJ anti-synthetase syndrome: a case series.
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.
authors.affiliation: Rheumatology Unit-DIM, University of Bari, P.zza G Cesare 11, 70124, Bari, Italy.
authors.forename: Margherita
authors.initials: M
authors.lastname: Giannini
authors.affiliation: Rheumatology Unit, Department of Medicine, Solna, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden.
authors.forename: Antonella
authors.initials: A
authors.lastname: Notarnicola
authors.affiliation: Rheumatology Unit, Department of Medicine, Solna, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden.
authors.forename: Maryam
authors.initials: M
authors.lastname: Dastmalchi
authors.affiliation: Rheumatology Unit, Department of Medicine, Solna, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden.
authors.forename: Ingrid E
authors.initials: IE
authors.lastname: Lundberg
authors.affiliation: Rheumatology Unit-DIM, University of Bari, P.zza G Cesare 11, 70124, Bari, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Lopalco
authors.affiliation: Rheumatology Unit-DIM, University of Bari, P.zza G Cesare 11, 70124, Bari, Italy. florenzo.iannone@uniba.it.
authors.forename: Florenzo
authors.initials: F
authors.lastname: Iannone
mesh_terms: D000328:Adult; D000604:Amino Acyl-tRNA Synthetases / Q000276:immunology*; D001323:Autoantibodies / Q000276:immunology*; D005260:Female; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000150:complications / Q000276:immunology*; D008875:Middle Aged; D009220:Myositis / Q000150:complications / Q000276:immunology*; D055815:Young Adult
chemical_list: D001323:Autoantibodies; D000604:Amino Acyl-tRNA Synthetases
keywords: Anti-EJ; Anti-synthetase syndrome; Interstitial lung disease