F Romero-Bueno1, P Diaz Del Campo2, E Trallero-Araguás3, J C Ruiz-Rodríguez4, I Castellvi5, M J Rodriguez-Nieto6, M J Martínez-Becerra7, O Sanchez-Pernaute1, I Pinal-Fernandez8, X Solanich9, T Gono10, M A Gonzalez-Gay11, M N Plana12, A Selva-O'Callaghan13. 1. Rheumatology Dept. Fundación Jiménez Díaz University Hospital, Universidad Autónoma de Madrid, Madrid, Spain. 2. Research Unit, Spanish Society of Rheumatology, Madrid, Spain. 3. Rheumatology Unit. Vall d'Hebron University Hospital, GEAS group, Barcelona, Spain. 4. Intensive Care Department. Vall d'Hebron University Hospital, Shock, Organ Dysfunction, and Resuscitation Research Group, Vall d'Hebron Research Institute, Barcelona, Spain. 5. Rheumatology Department. Hospital de la Santa Creu i Sant Pau. Universitat Autònoma de Barcelona, Barcelona, Spain. 6. Pneumology Dept. Fundación Jiménez Díaz University Hospital, Centro de Investigación Biomédica en Red de Enfermedades Respiratorias (CIBERES), Instituto de Salud Carlos III, Madrid, Spain. 7. Immunology Dept. Fundación Jiménez Díaz University Hospital, Madrid, Spain. 8. National Institute of Arthritis and Musculoskeletal and Skin Diseases; NIH, Bethesda; Johns Hopkins University School of Medicine, Baltimore, MD, USA. 9. Department of Internal Medicine, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat, Barcelona, Spain. 10. Nippon Medical School Graduate School of Medicine, Department of Allergy and Rheumatology, Tokyo, Japan. 11. Epidemiology, Genetics and Atherosclerosis Research Group on Systemic Inflammatory Diseases, Rheumatology Div., Hosp. Universitario Marqués de Valdecilla, IDIVAL and University of Cantabria, Santander; and Univ. of the Witwatersrand, South Africa. 12. Research Unit, Spanish Society of Rheumatology, Madrid, Spain. CIBER Epidemiology and Public Health (CIBERESP), Madrid, Spain. 13. Systemic Autoimmune Diseases Unit. Medicine Dept. Vall d'Hebron University Hospital, GEAS group. Universitat Autònoma de Barcelona, Barcelona, Spain.. Electronic address: aselva@vhebron.net.
Abstract
OBJECTIVES: The study aimed to develop evidence-based recommendations for the treatment of rapidly progressive interstitial lung disease (RPILD) associated with the anti-Melanoma Differentiation-Associated Gene 5-positive dermatomyositis (DM) syndrome. METHODS: The task force comprised an expert panel of specialists in rheumatology, intensive care medicine, pulmonology, immunology, and internal medicine. The study was carried out in two phases: identifying key areas in the management of DM-RPILD syndrome and developing a set of recommendations based on a review of the available scientific evidence. Four specific questions focused on different treatment options were identified. Relevant publications in English, Spanish or French up to April 2018 were searched systematically for each topic using PubMed (MEDLINE), EMBASE, and Cochrane Library (Wiley Online). The experts used evidence obtained from these studies to develop recommendations. RESULTS: A total of 134 studies met eligibility criteria and formed the evidentiary basis for the recommendations regarding immunosuppressive therapy and complementary treatments. Overall, there was general agreement on the initial use of combined immunosuppressive therapy. Combination of high-dose glucocorticoids and calcineurin antagonists with or without cyclophosphamide is the first choice. In the case of calcineurin antagonist contraindication or treatment failure, switching or adding other immunosuppressants may be individualized. Plasmapheresis, polymyxin B hemoperfusion and/or intravenous immunoglobulins may be used as rescue options. ECMO should be considered in life-threatening situations while waiting for a clinical response or as a bridge to lung transplant. CONCLUSIONS: Thirteen recommendations regarding the treatment of the anti-MDA5 positive DM-RPILD were developed using research-based evidence and expert opinion.
OBJECTIVES: The study aimed to develop evidence-based recommendations for the treatment of rapidly progressive interstitial lung disease (RPILD) associated with the anti-Melanoma Differentiation-Associated Gene 5-positive dermatomyositis (DM) syndrome. METHODS: The task force comprised an expert panel of specialists in rheumatology, intensive care medicine, pulmonology, immunology, and internal medicine. The study was carried out in two phases: identifying key areas in the management of DM-RPILD syndrome and developing a set of recommendations based on a review of the available scientific evidence. Four specific questions focused on different treatment options were identified. Relevant publications in English, Spanish or French up to April 2018 were searched systematically for each topic using PubMed (MEDLINE), EMBASE, and Cochrane Library (Wiley Online). The experts used evidence obtained from these studies to develop recommendations. RESULTS: A total of 134 studies met eligibility criteria and formed the evidentiary basis for the recommendations regarding immunosuppressive therapy and complementary treatments. Overall, there was general agreement on the initial use of combined immunosuppressive therapy. Combination of high-dose glucocorticoids and calcineurin antagonists with or without cyclophosphamide is the first choice. In the case of calcineurin antagonist contraindication or treatment failure, switching or adding other immunosuppressants may be individualized. Plasmapheresis, polymyxin B hemoperfusion and/or intravenous immunoglobulins may be used as rescue options. ECMO should be considered in life-threatening situations while waiting for a clinical response or as a bridge to lung transplant. CONCLUSIONS: Thirteen recommendations regarding the treatment of the anti-MDA5 positive DM-RPILD were developed using research-based evidence and expert opinion.
Authors: Ingrid E Lundberg; Manabu Fujimoto; Jiri Vencovsky; Rohit Aggarwal; Marie Holmqvist; Lisa Christopher-Stine; Andrew L Mammen; Frederick W Miller Journal: Nat Rev Dis Primers Date: 2021-12-02 Impact factor: 52.329