Background/Purpose:  Limited information on the patient experience exists in CTD-ILD.  Herein supports that the patients’ perspective is essential to informing clinical practice and in developing optimal outcome measures for CTD-ILD.

Methods: Focus groups were dedicated to patients with each of the following ILD subtypes: rheumatoid arthritis, idiopathic inflammatory myositis, systemic sclerosis, and various CTD subtypes.  Focus groups were followed with patient questionnaires and/or patient interview.  Institutional review board approval was attained at all participating institutions.

Included were English speaking adults with a diagnosis of ILD by either histologic or computed tomography (CT) evidence with either a) symptoms of cough and/or dyspnea or b) restrictive pulmonary physiology or c) resting or exertion-related peripheral oxygen desaturation. CTD diagnoses were by rheumatologists based on accepted criteria.  Patients with pulmonary hypertension or hypersensitivity pneumonitis made were excluded.

The script included 2 questions (“How have you experienced your disease since the diagnosis of ILD?”, “How has the disease changed?”), with the WHO-100 domains as back-up to insure comprehensiveness.  Data were analyzed through inductive development.  The thematic structures of 5 independent analysts were triangulated, including 1 patient for each transcript.

Results:    Six focus groups of 6-9 (total 45)  participants per group across 5centres (University of Manitoba, University of Toronto, Louisiana State University, Johns Hopkins University, Massachusetts General/Brigham and Womens) and two countries (Canada and USA) were conducted; the following themes that emerged from preliminary findings from a subset analysis.

I. Biophysiologic Sphere:

A. Cough:

i. Universal and relevant patient experience

ii. Patients described types of coughs and its triggers

B. Dyspnea:

i. Reference to breath/breathing rarely used

ii. Often described within context of functional limitation or loss of pleasurable activity and/or connectedness (eg. reading to children)

II. Psychological Sphere:

A. Living with Uncertainty:

i. Perpetuated by inadequate physician communication

ii. Unknown and unpredictable disease course in the immediate and long-term (death)

iii. Conflicts in management between CTD and ILD

B. Struggle Over the New Self:

i. Maintaining an autonomy

ii. Parenting and grand-parenting roles were a central concern

C. Development Of Resilience Through Coping (Self-Efficacy) 

Conclusion: Patient experts have informed our understanding around communication and promising, relevant outcome measures in CTD-ILD.  Medical expert consensus identified 5 domains in CTD-ILD for outcome measures.  While dyspnea remained a core outcome measure for both patient and medical experts, cough was relevant to patients but not medical experts.  A discordant language related to dyspnea exists between clinicians and patients and may impact performance of current and potential metrics related to dyspnea.