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Thorax. 2014 May;69(5):428-36. doi: 10.1136/thoraxjnl-2013-204202. Epub 2013 Dec 24.

Connective tissue disease related interstitial lung diseases and idiopathic pulmonary fibrosis: provisional core sets of domains and instruments for use in clinical trials.

Collaborators (225)

Aggarwal R, Ainslie G, Alkassab F, Allanore Y, Anderson ME, Andonopoulos AP, Antin-Ozerkis D, Arrobas A, Ascherman DP, Assassi S, Baron M, Bathon JM, Behr J, Beretta L, Bingham CO 3rd, Binnie M, Birring SS, Boin F, Bongartz T, Bourdin A, Bouros D, Brasington R, Bresser P, Buch MH, Burge PS, Carmona L, Carreira PE, Carvalho CR, Catoggio LJ, Cela CJ, Carreira PE, Carvalho CR, Catoggio LJ, Chan KM, Chapman J, Chatterjee S, Chua F, Chung L, Conron M, Corte T, Costgrove G, Costabel U, Cox G, Crestani B, Crofford LJ, Csuka ME, Curbelo P, Czirják L, Daniil Z, D'Arsigny CL, Davis GS, de Andrade JA, De Vuyst P, Dempsey OJ, Derk CT, Distler J, Dixon WG, Downey G, Doyle MK, Drent M, Durairaj L, Emery P, Espinoza LR, Farge D, Fathi M, Fell CD, Fessler BJ, Fitzgerald JE, Foeldvari I, Fox GA, Frech TM, Freitas S, Furst DE, Gabrielli A, García-Vicuña R, Georgiev OB, Gerbino A, Gillisen A, Gladman DD, Glassberg M, Goduico BR, Gogali A, Goh NS, Goldberg A, Goldberg HJ, Gourley MF, Griffing L, Grutters JC, Gunnarsson R, Hachulla E, Huriez C, Hall FC, Harari S, Herrick AL, Herzog EL, Hesselstrand R, Hirani N, Hodgson U, Hollingsworth HM, Horner RJ, Hoyles RK, Hsu VM, Hubbard RB, Hunzelmann N, Isasi ME, Isasi ES, Jacobsen S, Jimenez SA, Johnson SR, Jones CH, Kahaleh B, Kairalla AA, Kalluri M, Kalra S, Kaner RJ, Kinder BW, Kiter G, Klingsberg RC, Kokosi M, Kolb MR, Kur-Zalewska J, Kuwana M, Lake FR, Lally EV, Lasky JA, Laurindo IM, Able L, Lee P, Leonard CT, Lien DC, Limper AH, Liossis SN, Lohr KM, Loyd JE, Lundberg IE, Mageto YN, Maher TM, Mahmud TH, Manganas H, Marie I, Marras TK, Baddini Martinez JA, Martinez FJ, Mathieu A, Matucci-Cerinic M, Mayes MD, McKown KM, Medsger T Jr, Meehan RT, Cristina MA, Meyer KC, Millar AB, Mogulkoç N, Molitor JA, Morais A, Müller V, Müller-Quernheim J, Nadashkevich O, Nador R, Nash P, Nathan SD, Navarro C, Neves S, Noth I, Nunes H, Olson AL, Opitz CF, Pappas D, Parfrey H, Pego-Reigosa JM, Porter JC, Renzoni EA, Riemekasten G, Riley DJ, Rischmueller M, Rodriguez-Reyna TS, Rojas-Serrano, Roman J, Rosen GD, Rossman M, Rothfield N, Sahn SA, Sanduzzi A, Scholand MB, Selman M, Senécal JL, Seo P, Silver RM, Solomon JJ, Steen V, Stevens W, Strange C, Sussman R, Sutton ED, Sweiss NJ, Tomling G, Tzelepis GE, Undurraga A, Vacca A, Vancheri C, Varga J, Veale DJ, Volkov S, Wencel M, Wilcox P, Wollheim FA, Wuyts WA, Yung G, Zanon P, Zappala CJ, Groshong SD, Leslie KO, Myers JL, Padera RF, Desai S, Goldin J, Kazerooni EA, Klein JS, Lynch DA, Cenac SL, Grewal HK, Christensen AM, Ferguson S, Tran M, Keen KJ.

Author information

Louisiana State University Health Sciences Center, , New Orleans, Louisiana, USA.

Erratum in

  • Thorax. 2014 Sep;69(9):834. CTD-ILD Special Interest Group [added]; multiple investigator names added.



Clinical trial design in interstitial lung diseases (ILDs) has been hampered by lack of consensus on appropriate outcome measures for reliably assessing treatment response. In the setting of connective tissue diseases (CTDs), some measures of ILD disease activity and severity may be confounded by non-pulmonary comorbidities.


The Connective Tissue Disease associated Interstitial Lung Disease (CTD-ILD) working group of Outcome Measures in Rheumatology-a non-profit international organisation dedicated to consensus methodology in identification of outcome measures-conducted a series of investigations which included a Delphi process including >248 ILD medical experts as well as patient focus groups culminating in a nominal group panel of ILD experts and patients. The goal was to define and develop a consensus on the status of outcome measure candidates for use in randomised controlled trials in CTD-ILD and idiopathic pulmonary fibrosis (IPF).


A core set comprising specific measures in the domains of lung physiology, lung imaging, survival, dyspnoea, cough and health-related quality of life is proposed as appropriate for consideration for use in a hypothetical 1-year multicentre clinical trial for either CTD-ILD or IPF. As many widely used instruments were found to lack full validation, an agenda for future research is proposed.


Identification of consensus preliminary domains and instruments to measure them was attained and is a major advance anticipated to facilitate multicentre RCTs in the field.


Connective tissue disease associated lung disease; Idiopathic pulmonary fibrosis; Interstitial Fibrosis; Rheumatoid lung disease; Systemic disease and lungs

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