Jutta Bauhammer1, Norbert Blank1, Regina Max1, Hanns-Martin Lorenz1, Ulrich Wagner1, Dietmar Krause1, Christoph Fiehn2. 1. 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. 2. 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.
Abstract
OBJECTIVE: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.
OBJECTIVE: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. METHODS: 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. RESULTS: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. CONCLUSION: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: Kun Huang; Ophir Vinik; Kam Shojania; James Yeung; Rachel Shupak; Michael Nimmo; J Antonio Avina-Zubieta Journal: Rheumatol Int Date: 2019-08-02 Impact factor: 2.631
Authors: Tracy J Doyle; Namrata Dhillon; Rachna Madan; Fernanda Cabral; Elaine A Fletcher; Diane C Koontz; Rohit Aggarwal; Juan C Osorio; Ivan O Rosas; Chester V Oddis; Paul F Dellaripa Journal: J Rheumatol Date: 2018-04-01 Impact factor: 4.666