Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.

Developing a Disease Activity and Therapeutic Response Index in Connective Tissue Disease Related Interstitial Lung Disease (CTD-ILD): Rationale, Aims, Design and Results from Tier 0, the Pre-Delphi Exercise.

Saketkoo4,  Lesley Ann, Huscher2,  Dörte, Khanna12,  Dinesh, Dellaripa1,  Paul F., Flaherty15,  Kevin, Matteson5,  Eric L., Oddis16,  Chester V.

Brigham & Womens Hospital, Boston, MA
Stanford University; Palo Alto, Portola Valley, CA
University Hospital Zurich, Switzerland
University of California Los Angeles
University of Connecticut Health Center, Farmington, CT
University of Michigan, Ann Arbor, MI
University of Michigan, Ann Arbor
University of Pittsburgh, PA
German Rheumatism Research Centre - Berlin, Germany
Institute of Clinical Pharmacology, Medical Faculty, University of Dresden, Germany
Louisiana State University Health Sciences Center - New Orleans, LA
Mayo Clinic, Rochester, MN
Medical University of Bialystok, Poland
National Jewish Health, Denver, CO
Royal Brompton Hospital and National Heart and Lung Institute, London, United Kingdom
Royal Free Hospital, London, United Kingdom


There are currently no licensed therapies for interstitial lung disease (ILD). There is no consensus on measures of disease activity or therapeutic responsiveness, thus hampering effective drug development and regulatory evaluation of candidate therapies. We present the first phase of a multi-tiered investigation to identify consensus on provisional criteria to measure disease activity and therapeutic response in CTD-ILD and Idiopathic Pulmonary Fibrosis (IPF).


A multi-disciplinary steering committee (authors) engaged in extensive literature review and agreed upon the following methodology: Pulmonary, rheumatology and cardiology specialists with ILD expertise were identified by literature review, task force membership in international societies and peer recommendation. Each was invited to participate in a 3-Tier Delphi project with a preceding 'Tier 0' exercise. The goal of 'Tier 0' was to engage the participants in a brainstorming exercise to identify qualities that are important to measure ('domains') in CTD-ILD and IPF and 'instruments' to measure these domains. The goal of contrasting CTD-ILD and IPF is to distinguish similarities and differences between these entities. Additionally, suggestions of positive and negative instruments were collected for specific CTD-ILD disease groups. Data was collected via a custom designed secure web-site. Participants typed their additions into a 'sparse' framework of 12 sample 'domains' with 12 sample 'instruments'. The steering committee initially participated in a series of individual exercises followed by a group effort to limit redundancy and identify the broadest spectrum of reasonable domains that would appropriately accommodate the diversity of 'instruments' or 'outcome measures' suggested by the participants.


98% of invited experts participated. 137 pulmonary, 102 rheumatology and 4 cardiology specialists participated from 32 countries representing 6 continents. 74% and 69% of participants considered 'ILD' and 'rheumatologic lung disease' respectively to be their primary fields of investigation. 133 domains were suggested and after review resulted in the 23 domains listed below. More than 6700 instruments were proposed and later reduced to 616.

Table 1. Final 23 Preliminary Domains From Tier 0 of the Delphi Process

SurvivalMental Health
ImagingGlobal Assessment
Lung Physiology/FunctionHealth Related Quality of Life
Lung ParenchymaFunctionality
Lung VascularParticipation
Cardiac FunctionEmployment
Composite ScoresExtra-Pulmonary/CTD Features
Gastroesophogeal RefluxMedication
DyspneaBarriers to Care


This is the first known multi-disciplinary effort to assess domains and instruments in the study of CTD ILD – which is essential in a field devoid of meaningful outcome measures and with many candidate therapies on the horizon. The high response rate of this comprehensive, multi-disciplinary, international effort reflects both the need for a systematic approach to ILD and the collective interest in establishing criteria for this group of diseases. The overall results are very promising and reflect the innate complexities of the spectrum of ILD, thus creating a robust platform for next phases of this consensus study.

To cite this abstract, please use the following information:
Saketkoo, Lesley Ann, Huscher, Dörte, Khanna, Dinesh, Dellaripa, Paul F., Flaherty, Kevin, Matteson, Eric L., et al; Developing a Disease Activity and Therapeutic Response Index in Connective Tissue Disease Related Interstitial Lung Disease (CTD-ILD): Rationale, Aims, Design and Results from Tier 0, the Pre-Delphi Exercise. [abstract]. Arthritis Rheum 2010;62 Suppl 10 :1212
DOI: 10.1002/art.28978

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