home based clinical management of interstitial lung disease in systemic rheumatic diseases

Conditions: Interstitial Lung Disease with Progressive Fibrotic Phenotype in Diseases Classified Elsewhere; Systemic Sclerosis Pulmonary; Dermatomyositis; Rheumatoid Arthritis; Sjogren Syndrome with Lung Involvement; Connective Tissue Diseases

Summary: the rmd-milder trial is a home monitoring strategy trial aiming to improve management of interstitial lung disease related to rheumatic diseases applying ehealth technology. it is planned as a 2 arm 54 week multi-centre randomised controlled trial to assess outcome of home monitoring with bi-weekly serial forced vital capacity- and patient reported outcome-measurements compared to standard of care with fixed-interval hospital visits in adult patients with rheumatic disease associated interstitial lung diseases.


Eligibility criteria:
inclusion criteria: * systemic rheumatic disease (systemic sclerosis (ssc), rheumatoid arthritis (ra), idiopathic inflammatory myopathies including antisynthetasis syndromes (iim), mixed connective tissue disease (mctd) or sjøgrens disease (sjd)) classifiable by disease-specific classification criteria * diagnosed interstitial lung disease (ild) on high resolution computed tomography (hrct) ≥ 1 year prior to randomization, not explained by other diseases or exposures * on stable standard of care treatment 6 months prior to randomization * participants must be able to understand and follow trial procedures including completion of questionnaires regarding patient reported outcome measures * participants must have access to the internet, and experience in using smartphones or other electronic devices with internet access * signed informed consent form exclusion criteria: * severe heart failure with ejection fraction (ef) \< 30% * chronic renal failure g4 or more (defined by kdigo) with glomerular filtration rate (egfr) \< 30 ml/min using cockroft-gault formula. * end stage lung disease with forced vital capacity (fvc) \< 50% and/or diffusion capacity for carbon monoxide (dlco) \< 40% or coexisting severe other lung diseases (e.g. chronic obstructive pulmonary disease, emphysema) * airway obstruction (pre-bronchodilator fev1/fvc \< 0.7) (fev1 is defined as forced expiratory volume in 1 sec) * in the opinion of the investigator, other clinically significant pulmonary abnormalities * significant pulmonary hypertension defined by the following: previous clinical or echocardiographic evidence of significant right heart failure or history of right heart catheterization showing a cardiac index \</= 2 l/min/m2 or pulmonary hypertension requiring therapy with epoprostenol/treprostinil * active treatment for cancer or non-curable cancer * relative contraindications to performing spirometry, as specified in ats/ers guidelines. * ongoing prednisolone ≥ 20 mg/day at inclusion * unable to speak, write and read norwegian, german or romanian in the respective country of inclusion. * unable to perform good quality measurements of fvc on the home-device comparable to results on an in-hospital device, after training. * pregnancy or planned pregnancy

Keywords: home monitoring remote monitoring interstitial lung disease rheumatic disease lung fibrosis systemic sclerosis dermatomyositis sjøgren