Lung Disease

Lung Disease Working Group

Summary of goals/project(s) of working group:

To define progression of SSc-ILD of disease
To determine what proportion of SSc patients have progression of their ILD after 7 years
Longer-term goal is to harmonize inclusion criteria for SSc-ILD clinical trials

Progress to date for working group:

Drs. David Roofeh and Andrew Lewandoski, rheumatologists at the University of Michigan, are leading the effort to define progressive SSc-ILD, as part of their larger effort to define progressive CTD-ILD. They performed a literature review and proposed definitions of progressive SSc-ILD. They then contacted 40 experts in CTD-ILD (18 pulmonologists, 15 rheumatologists, and 7 radiologists) via email, phone, and interactive teleconference. Experts were asked for their critical feedback of these proposed definitions, and invitations to propose their own definitions of progressive SSc-ILD via email. These changes were incorporated and subsequently revised after 3 teleconference calls, with a discussion focusing on further refining these definitions. Drs. Roofeh and Lewandoski subsequently provided ILD experts in pulmonology, rheumatology, and radiology with real-life examples of SSc-ILD patients with longitudinal data, and these experts categorized the patients as either progressed or not progressed, or unable to determine, and stated which components/domains influenced their decisions. Dr. Roofeh presented an abstract based on this work at the 2019 ACR Annual Meeting (abstract #2608).

Next steps:

Drs. Roofeh and Lewandoski are currently analyzing the data in order to operationalize the definition of progressive SSc-ILD and to develop a consensus definition.