Home Based Clinical Management of Interstitial Lung Disease in Systemic Rheumatic Diseases

Time from baseline to disease progression assessed as first forced vital capacity (FVC) ≥5% decline (assessed by hospital FVC measurements) or non-elective hospitalization due to respiratory cause.

Absolute change from baseline in FVC (mL) and absolute change from baseline in FVC (% predicted).

Proportion of patients who experience at least one FVC decline >= 10% event from baseline

Change in "Living with pulmonary fibrosis score" (LPF) total score, as well as the subdomains physical health, emotional well-being, social impact, functionality and daily activities as well as cognitive function where lower values indicate quality of life

Proportion of patients who experience a progressive pulmonary fibrosis event defined as fibrosing ILD fulfilling ≥2 (out of 3) criteria (worsening respiratory symptoms, radiological progression, and physiological progression (absolute decline in FVC ≥ 5% predicted within 1 year follow up OR absolute decline in diffusion capacity for carbon monoxide (DLCO) ≥10% predicted within 1 year of follow up) occurring within the past year with no alternative explanation in a patient with ILD.

Change in visual analogue scale (VAS) on RMD from 0 - 10 with higher values indicating more symptoms

Change in VAS on the patients global health from 0 - 10 with higher values indicating more symptoms

Change in the EuroQol Eq-5D-5L questionnaire with the following domains: Mobility, self-care, usual activities, pain/discomfort, anxiety/depression. The score typically ranges from 0 (representing a health state equivalent to death) to 1 (representing perfect health).

Change in Health Assessment Questionaire (HAQ) covering eight different domains: Dressing and grooming, arising, eating, walking, hygiene, reach, gripping and activities.

The HAQ has 20 questions. The score reaches from 0 (no incapacity) to 3 (full incapacity)

Change in EULAR Sjogren's Syndrome Patient Reported Index (ESSPRI) with a range of 0 - 10, with higher values indicating more symptoms.

Change in EULAR Systemic Sclerosis Impact of Disease (ScleroID) questionnaire whereby higher counts on a scale from 0-100 would indicate more impact

Change in VAS on global health from 0 - 10 with higher values indicating a higher burden

Change in Disease Activity Score-28 (DAS28)

Change in EULAR Sjögren's syndrome disease activity index (ESSDAI)

Change in MDAAT / MYOACT (range 0-60) where higher values would indicate more disease activity

Change in MDAAT / MITAX (range 0-63) where higher values would indicate more disease activity

Change in Revised European Scleroderma Trials and Research Group Activity Index (EUSTAR-AI) (range 0-10) where higher values would indicate more disease activity

Absolute change from baseline in DLCO (SI)

· Absolute change from baseline in DLCO (% predicted)

Change in extent of fibrosis as percentage of total lung parenchyma on HRCT

Change in "Modified Medical Research Council Dyspnoea Scale" (mMRC) with a range of 0-4 where higher values indicate more disability

Change on a visual analogue scale for dyspnea where higher values would indicate more symptoms

Change on a visual analogue scale for cough where higher values would indicate more symptoms

Change on a visual analogue scale for fatigue where higher values would indicate more symptoms

Number of verified afebrile episodes

Difference in "Client satisfaction questionnaire 8" (CSQ-8) scores (range 8-32 where higher values indicate a higher satisfaction) between the to arms

Difference on the "Hosital Anxiety and Depression Scale" (HADS) with a range of 0-21 where higher scores would indicate more depression and axiety

Total number and proportion of severe csRTIs compared between the arms

Days on antibiotic therapy for csRTIs compared between the arms

Days hospitalised for csRTIs compared between the arms

Time to first acute exacerbation event

Assess content and change in peripheral blood markers derived from peripheral blood (as evaluated by sequencing techniques, proteomics and cellular phenotyping)

Assess content and change in immune cells and soluble markers of inflammation in nasal swabs (as evaluated by sequencing techniques, proteomics and cellular phenotyping)

Proportion of participants subjected to increased immunosuppression

Proportion of participants in non-remission for non-ILD disease manifestations

Proportion of patients who experience ILD progression with reflux disease compared to no reflux disease