title: Rituximab in the Treatment of Jo1 Antibody-associated Antisynthetase Syndrome: Anti-Ro52 Positivity as a Marker for Severity and Treatment Response.
abstract: Rituximab (RTX) has been used successfully for the treatment of severe Jo1 antibody-associated antisynthetase syndrome. The aim of this retrospective study was to evaluate the effect of RTX in severe Jo1 antisynthetase syndrome and determine predictive factors for response.
  
  
  
  There were 61 patients with Jo1 antisynthetase syndrome identified; 18 of these received RTX. One patient was lost to followup. The remaining 17 patients and 30 out of 43 patients who were treated with conventional immunosuppressive (IS) drugs were followed for a mean of 35 months and 84 months, respectively.
  
  
  
  Polymyositis/dermatomyositis (95%) and interstitial lung disease (ILD; 66%) were the dominant clinical manifestations. Detection of anti-Ro52 antibodies (43%) was significantly associated with acute-onset ILD (p = 0.016) with O2 dependency, and patients with high concentrations of anti-Ro52 (20%) had the highest risk (p = 0.0005). Sixteen out of 18 patients (89%) showed a fast and marked response to RTX. Among those patients who were highly positive for anti-Ro52, response to RTX was seen in 7 out of 7 cases (100%), but no response to cyclophosphamide (n = 4), cyclosporine A (n = 3), azathioprine (n = 9), methotrexate (n = 5), or leflunomide (n = 2) was observed. One patient treated with RTX died of pneumonia.
  
  
  
  RTX is effective in the treatment of severe forms of Jo1 antisynthetase syndrome. In our retrospective study, the presence of high anti-Ro52 antibody concentrations predicts severe acute-onset ILD and nonresponse to IS drugs. In contrast to conventional IS, RTX is equally effective in patients with Jo1 antisynthetase syndrome, independent of their anti-Ro52 antibody status.
authors.affiliation: From the ACURA Centre for Rheumatic Diseases, Baden-Baden; Department of Internal Medicine V, University of Heidelberg, Heidelberg; Department of Pneumology, Klinikum Löwenstein, Löwenstein; Department of Medical Informatics, Biometry and Epidemiology, Ruhr University Bochum, Bochum, Germany.J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Blank, MD, Department of Internal Medicine V, University of Heidelberg; R. Max, MD, Department of Internal Medicine V, University of Heidelberg; H.M. Lorenz, MD, ACURA Centre for Rheumatic Diseases, and Department of Internal Medicine V, University of Heidelberg; U. Wagner, MD, Department of Pneumology, Klinikum Löwenstein; D. Krause, MD, Department of Medical Informatics, Biometry and Epidemiology, Ruhr University Bochum; C. Fiehn, MD, ACURA Centre for Rheumatic Diseases.
authors.forename: Jutta
authors.initials: J
authors.lastname: Bauhammer
authors.affiliation: From the ACURA Centre for Rheumatic Diseases, Baden-Baden; Department of Internal Medicine V, University of Heidelberg, Heidelberg; Department of Pneumology, Klinikum Löwenstein, Löwenstein; Department of Medical Informatics, Biometry and Epidemiology, Ruhr University Bochum, Bochum, Germany.J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Blank, MD, Department of Internal Medicine V, University of Heidelberg; R. Max, MD, Department of Internal Medicine V, University of Heidelberg; H.M. Lorenz, MD, ACURA Centre for Rheumatic Diseases, and Department of Internal Medicine V, University of Heidelberg; U. Wagner, MD, Department of Pneumology, Klinikum Löwenstein; D. Krause, MD, Department of Medical Informatics, Biometry and Epidemiology, Ruhr University Bochum; C. Fiehn, MD, ACURA Centre for Rheumatic Diseases.
authors.forename: Norbert
authors.initials: N
authors.lastname: Blank
authors.affiliation: From the ACURA Centre for Rheumatic Diseases, Baden-Baden; Department of Internal Medicine V, University of Heidelberg, Heidelberg; Department of Pneumology, Klinikum Löwenstein, Löwenstein; Department of Medical Informatics, Biometry and Epidemiology, Ruhr University Bochum, Bochum, Germany.J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Blank, MD, Department of Internal Medicine V, University of Heidelberg; R. Max, MD, Department of Internal Medicine V, University of Heidelberg; H.M. Lorenz, MD, ACURA Centre for Rheumatic Diseases, and Department of Internal Medicine V, University of Heidelberg; U. Wagner, MD, Department of Pneumology, Klinikum Löwenstein; D. Krause, MD, Department of Medical Informatics, Biometry and Epidemiology, Ruhr University Bochum; C. Fiehn, MD, ACURA Centre for Rheumatic Diseases.
authors.forename: Regina
authors.initials: R
authors.lastname: Max
authors.affiliation: From the ACURA Centre for Rheumatic Diseases, Baden-Baden; Department of Internal Medicine V, University of Heidelberg, Heidelberg; Department of Pneumology, Klinikum Löwenstein, Löwenstein; Department of Medical Informatics, Biometry and Epidemiology, Ruhr University Bochum, Bochum, Germany.J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Blank, MD, Department of Internal Medicine V, University of Heidelberg; R. Max, MD, Department of Internal Medicine V, University of Heidelberg; H.M. Lorenz, MD, ACURA Centre for Rheumatic Diseases, and Department of Internal Medicine V, University of Heidelberg; U. Wagner, MD, Department of Pneumology, Klinikum Löwenstein; D. Krause, MD, Department of Medical Informatics, Biometry and Epidemiology, Ruhr University Bochum; C. Fiehn, MD, ACURA Centre for Rheumatic Diseases.
authors.forename: Hanns-Martin
authors.initials: HM
authors.lastname: Lorenz
authors.affiliation: From the ACURA Centre for Rheumatic Diseases, Baden-Baden; Department of Internal Medicine V, University of Heidelberg, Heidelberg; Department of Pneumology, Klinikum Löwenstein, Löwenstein; Department of Medical Informatics, Biometry and Epidemiology, Ruhr University Bochum, Bochum, Germany.J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Blank, MD, Department of Internal Medicine V, University of Heidelberg; R. Max, MD, Department of Internal Medicine V, University of Heidelberg; H.M. Lorenz, MD, ACURA Centre for Rheumatic Diseases, and Department of Internal Medicine V, University of Heidelberg; U. Wagner, MD, Department of Pneumology, Klinikum Löwenstein; D. Krause, MD, Department of Medical Informatics, Biometry and Epidemiology, Ruhr University Bochum; C. Fiehn, MD, ACURA Centre for Rheumatic Diseases.
authors.forename: Ulrich
authors.initials: U
authors.lastname: Wagner
authors.affiliation: From the ACURA Centre for Rheumatic Diseases, Baden-Baden; Department of Internal Medicine V, University of Heidelberg, Heidelberg; Department of Pneumology, Klinikum Löwenstein, Löwenstein; Department of Medical Informatics, Biometry and Epidemiology, Ruhr University Bochum, Bochum, Germany.J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Blank, MD, Department of Internal Medicine V, University of Heidelberg; R. Max, MD, Department of Internal Medicine V, University of Heidelberg; H.M. Lorenz, MD, ACURA Centre for Rheumatic Diseases, and Department of Internal Medicine V, University of Heidelberg; U. Wagner, MD, Department of Pneumology, Klinikum Löwenstein; D. Krause, MD, Department of Medical Informatics, Biometry and Epidemiology, Ruhr University Bochum; C. Fiehn, MD, ACURA Centre for Rheumatic Diseases.
authors.forename: Dietmar
authors.initials: D
authors.lastname: Krause
authors.affiliation: From the ACURA Centre for Rheumatic Diseases, Baden-Baden; Department of Internal Medicine V, University of Heidelberg, Heidelberg; Department of Pneumology, Klinikum Löwenstein, Löwenstein; Department of Medical Informatics, Biometry and Epidemiology, Ruhr University Bochum, Bochum, Germany.J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Blank, MD, Department of Internal Medicine V, University of Heidelberg; R. Max, MD, Department of Internal Medicine V, University of Heidelberg; H.M. Lorenz, MD, ACURA Centre for Rheumatic Diseases, and Department of Internal Medicine V, University of Heidelberg; U. Wagner, MD, Department of Pneumology, Klinikum Löwenstein; D. Krause, MD, Department of Medical Informatics, Biometry and Epidemiology, Ruhr University Bochum; C. Fiehn, MD, ACURA Centre for Rheumatic Diseases. c.fiehn@acura-kliniken.com.
authors.forename: Christoph
authors.initials: C
authors.lastname: Fiehn
mesh_terms: D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000032:analysis*; D003882:Dermatomyositis / Q000188:drug therapy* / Q000276:immunology; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D017563:Lung Diseases, Interstitial / Q000188:drug therapy* / Q000276:immunology; D008297:Male; D008875:Middle Aged; D009220:Myositis / Q000188:drug therapy* / Q000276:immunology; D012189:Retrospective Studies; D012261:Ribonucleoproteins / Q000276:immunology*; D000069283:Rituximab / Q000627:therapeutic use*; D012720:Severity of Illness Index; D016896:Treatment Outcome; D055815:Young Adult; D000099207:SS-A Antigen
chemical_list: D001323:Autoantibodies; D007166:Immunosuppressive Agents; D012261:Ribonucleoproteins; D000069283:Rituximab; D000099207:SS-A Antigen
keywords: ANTISYNTHETASE ANTIBODIES; INTERSTITIAL LUNG DISEASE; Jo1; POLYMYOSITIS; Ro52