TY - GEN AB - <p>Background: Systemic sclerosis (SSc) is a rare, complex, connective tissue disorder. Interstitial lung disease (ILD) is common in SSc, occurring in 35-52% of patients and accounting for 20-40% of mortality. Evolution of therapeutic options has resulted in a lack of consensus on how to manage this condition. This Delphi study was initiated to develop consensus recommendations based on expert physician insights regarding screening, progression, treatment criteria, monitoring of response, and the role of recent therapeutic advances with antifibrotics and immunosuppressants in patients with SSc-ILD.</p> <p>Methods: A modified Delphi process was completed by pulmonologists (n = 13) and rheumatologists (n = 12) with expertise in the management of patients with SSc-ILD. Panelists rated their agreement with each statement on a Likert scale from - 5 (complete disagreement) to + 5 (complete agreement). Consensus was predefined as a mean Likert scale score of ≤ - 2.5 or ≥ + 2.5 with a standard deviation not crossing zero.</p> <p>Results: Panelists recommended that all patients with SSc be screened for ILD by chest auscultation, spirometry with diffusing capacity of the lungs for carbon monoxide, high-resolution computed tomography (HRCT), and/or autoantibody testing. Treatment decisions were influenced by baseline and changes in pulmonary function tests, extent of ILD on HRCT, duration and degree of dyspnea, presence of pulmonary hypertension, and potential contribution of reflux. Treatment success was defined as stabilization or improvement of signs or symptoms of ILD and functional status. Mycophenolate mofetil was identified as the initial treatment of choice. Experts considered nintedanib a therapeutic option in patients with progressive fibrotic ILD despite immunosuppressive therapy or patients contraindicated/unable to tolerate immunotherapy. Concomitant use of nintedanib with MMF/cyclophosphamide can be considered in patients with advanced disease at initial presentation, aggressive ILD, or significant disease progression. Although limited consensus was achieved on the use of tocilizumab, the experts considered it a therapeutic option for patients with early SSc and ILD with elevated acute-phase reactants.</p> <p>Conclusions: This modified Delphi study generated consensus recommendations for management of patients with SSc-ILD in a real-world setting. Findings from this study provide a management algorithm that will be helpful for treating patients with SSc-ILD and addresses a significant unmet need.</p> AD - Cleveland Clinic Florida AD - Rutgers University AD - Weill Cornell Medicine AD - University of Texas AD - Brigham and Women’s Hospital AD - University of California, Los Angeles AD - Georgetown University AD - University of Chicago AD - Columbia University AD - Ohio State University AD - Harvard University AD - Stanford University AD - University of Pittsburgh AD - University of Michigan AD - University of Cincinnati AD - University of Toledo AD - Weill Cornell Medicine AD - Creighton University AD - Mayo Clinic AD - Boehringer Ingelheim Pharmaceuticals Inc AD - Inova Fairfax Hospital AD - Cleveland Clinic AD - University of California, Los Angeles AD - University of Michigan AU - Rahaghi, Franck F. AU - Hsu, Vivien M. AU - Kaner, Robert J. AU - Mayes, Maureen D. AU - Rosas, Ivan O. AU - Saggar, Rajan AU - Steen, Virginia D. AU - Strek, Mary E. AU - Bernstein, Elana J. AU - Bhatt, Nitin AU - Castelino, Flavia V. AU - Chung, Lorinda AU - Domsic, Robyn T. AU - Flaherty, Kevin R. AU - Gupta, Nishant AU - Kahaleh, Bashar AU - Martinez, Fernando J. AU - Morrow, Lee E. AU - Moua, Teng AU - Patel, Nina AU - Shlobin, Oksana A. AU - Southern, Brian D. AU - Volkmann, Elizabeth R. AU - Khanna, Dinesh DA - 2023-01-09 ID - 5395 JF - Respiratory Research KW - Algorithms KW - Autoimmune diseases KW - Connective tissue diseases KW - Drug therapy KW - Pulmonary fibrosis L1 - https://knowledge.uchicago.edu/record/5395/files/Expert-consensus-on-the-management-of-systemic-sclerosis-associated-interstitial-lung-disease.pdf L1 - https://knowledge.uchicago.edu/record/5395/files/12931_2022_2292_MOESM1_ESM.docx L2 - https://knowledge.uchicago.edu/record/5395/files/Expert-consensus-on-the-management-of-systemic-sclerosis-associated-interstitial-lung-disease.pdf L2 - https://knowledge.uchicago.edu/record/5395/files/12931_2022_2292_MOESM1_ESM.docx L4 - https://knowledge.uchicago.edu/record/5395/files/Expert-consensus-on-the-management-of-systemic-sclerosis-associated-interstitial-lung-disease.pdf L4 - https://knowledge.uchicago.edu/record/5395/files/12931_2022_2292_MOESM1_ESM.docx LA - eng LK - https://knowledge.uchicago.edu/record/5395/files/Expert-consensus-on-the-management-of-systemic-sclerosis-associated-interstitial-lung-disease.pdf LK - https://knowledge.uchicago.edu/record/5395/files/12931_2022_2292_MOESM1_ESM.docx N2 - <p>Background: Systemic sclerosis (SSc) is a rare, complex, connective tissue disorder. Interstitial lung disease (ILD) is common in SSc, occurring in 35-52% of patients and accounting for 20-40% of mortality. Evolution of therapeutic options has resulted in a lack of consensus on how to manage this condition. This Delphi study was initiated to develop consensus recommendations based on expert physician insights regarding screening, progression, treatment criteria, monitoring of response, and the role of recent therapeutic advances with antifibrotics and immunosuppressants in patients with SSc-ILD.</p> <p>Methods: A modified Delphi process was completed by pulmonologists (n = 13) and rheumatologists (n = 12) with expertise in the management of patients with SSc-ILD. Panelists rated their agreement with each statement on a Likert scale from - 5 (complete disagreement) to + 5 (complete agreement). Consensus was predefined as a mean Likert scale score of ≤ - 2.5 or ≥ + 2.5 with a standard deviation not crossing zero.</p> <p>Results: Panelists recommended that all patients with SSc be screened for ILD by chest auscultation, spirometry with diffusing capacity of the lungs for carbon monoxide, high-resolution computed tomography (HRCT), and/or autoantibody testing. Treatment decisions were influenced by baseline and changes in pulmonary function tests, extent of ILD on HRCT, duration and degree of dyspnea, presence of pulmonary hypertension, and potential contribution of reflux. Treatment success was defined as stabilization or improvement of signs or symptoms of ILD and functional status. Mycophenolate mofetil was identified as the initial treatment of choice. Experts considered nintedanib a therapeutic option in patients with progressive fibrotic ILD despite immunosuppressive therapy or patients contraindicated/unable to tolerate immunotherapy. Concomitant use of nintedanib with MMF/cyclophosphamide can be considered in patients with advanced disease at initial presentation, aggressive ILD, or significant disease progression. Although limited consensus was achieved on the use of tocilizumab, the experts considered it a therapeutic option for patients with early SSc and ILD with elevated acute-phase reactants.</p> <p>Conclusions: This modified Delphi study generated consensus recommendations for management of patients with SSc-ILD in a real-world setting. Findings from this study provide a management algorithm that will be helpful for treating patients with SSc-ILD and addresses a significant unmet need.</p> PY - 2023-01-09 T1 - Expert consensus on the management of systemic sclerosis-associated interstitial lung disease TI - Expert consensus on the management of systemic sclerosis-associated interstitial lung disease UR - https://knowledge.uchicago.edu/record/5395/files/Expert-consensus-on-the-management-of-systemic-sclerosis-associated-interstitial-lung-disease.pdf UR - https://knowledge.uchicago.edu/record/5395/files/12931_2022_2292_MOESM1_ESM.docx Y1 - 2023-01-09 ER -