Rahaghi et al Respiratory Research (2023)24:6Respiratory Researchhttps:./dol.org/10.1186s12931-022-02292-3RESEARCHOpen AccessExpert consensus on the managementof systemic sclerosis-associated interstitial lungdiseaseFranck F.Rahaghi,Vivien M.Hsu2,Robert J.Kaner3,Maureen D.Mayes4,Ivan O.Rosas5,Rajan Saggar5,Virginia D.Steen?,Mary E.Strek,Elana J.Bernstein,Nitin Bhatt0,Flavia V.Castelino1,Lorinda Chung2,Robyn T.Domsic3,Kevin R.Flaherty4,Nishant Gupta5,Bashar Kahaleh6,FemandoJ.Martinez,Lee E.Morrow 17,Teng Moua18,Nina Patel919,Oksana A.Shlobin20,Brian D.Southern21,Elizabeth R.Volkmann5 and Dinesh Khanna14AbstractBackground Systemic sclerosis(SSc)is a rare,complex,connective tissue disorder.Interstitial lung disease(ILD)iscommon in SSc,occurring in 35-52%of patients and accounting for 20-40%of mortality.Evolution of therapeu-tic options has resulted in a lack of consensus on how to manage this condition.This Delphi study was initiated todevelop consensus recommendations based on expert physician insights regarding screening,progression,treatmentcriteria,monitoring of response,and the role of recent therapeutic advances with antifibrotics and immunosuppres-sants in patients with SSc-ILD.Methods A modified Delphi process was completed by pulmonologists(n=13)and rheumatologists(n=12)withexpertise in the management of patients with SSc-ILD.Panelists rated their agreement with each statement on a Lik-ert scale from-5(complete disagreement)to+5(complete agreement).Consensus was predefined as a mean Likertscale score of <-2.5 or >+2.5 with a standard deviation not crossing zero.Results Panelists recommended that all patients with SSc be screened for ILD by chest auscultation,spirometry withdiffusing capacity of the lungs for carbon monoxide,high-resolution computed tomography (HRCT),and/or autoanti-body testing.Treatment decisions were influenced by baseline and changes in pulmonary function tests,extent of ILDon HRCT,duration and degree of dyspnea,presence of pulmonary hypertension,and potential contribution of refluxTreatment 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 therapeu-tic 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 consideredin patients with advanced disease at initial presentation,aggressive ILD,or significant disease progression.Althoughlimited consensus was achieved on the use of tocilizumab,the experts considered it a therapeutic option for patientswith early SSc and ILD with elevated acute-phase reactants."CorrespondenceFranck F.RahaghiRAHAGHF@ccforgDinesh Khannakhannad@umich.eduFull list of author infor mation is available at the end of the articleBMCThe Author(s)2023.Open AccessThis artidle is licensed undera Creative C
