Disease Behaviour During the Peri-Diagnostic Period in Patients with Suspected Interstitial Lung Disease: The STARLINER Study

Abstract

Introduction Disease behaviour may guide diagnosis and treatment decisions in patients with interstitial lung disease (ILD). STARLINER aimed to characterise disease behaviour in patients with suspected ILD during the peri-diagnostic period using real-time home-based assessments. Methods STARLINER (NCT03261037) was an international, multicentre study. Patients ≥ 50 years old with suspected ILD were followed throughout the peri-diagnostic period, consisting of a pre-diagnostic period (from enrolment to diagnosis) and a post-diagnostic period (from diagnosis to treatment initiation). Study length was variable (≤ 18 months). The primary endpoint was time-adjusted semi-annual forced vital capacity (FVC) change measured during the peri-diagnostic period using daily home spirometry in patients with idiopathic pulmonary fibrosis (IPF). Secondary outcomes included changes in FVC (home spirometry) in patients with non-IPF ILD, changes in FVC (site spirometry), changes in physical functional capacity measured by daily home accelerometry and site 6-min walk distance (6MWD), and changes in patient-reported outcomes (PROs) in IPF or non-IPF ILD. Results Of the 178 patients enrolled in the study, 68 patients were diagnosed with IPF, 62 patients were diagnosed with non-IPF ILD, 9 patients received a non-ILD diagnosis and 39 patients did not receive a diagnosis. Technical and analytical issues led to problems in applying the prespecified linear regression model to analyse the home FVC data. Time-adjusted median (quartile [Q]1, Q3) semi-annual FVC change during the peri-diagnostic period measured using home and site spirometry, respectively, was – 147.7 (– 723.8, 376.2) ml and – 149.0 (– 314.6, 163.9) ml for IPF and 19.1 (– 194.9, 519.0) ml and – 23.4 (– 117.9, 133.5) ml in non-IPF ILD. A greater decline in steps per day was observed for IPF versus non-IPF ILD, whereas an increase in 6MWD was observed for patients with IPF versus a decline in 6MWD for patients with non-IPF ILD. No clear patterns of disease behaviour were observed for IPF versus non-IPF ILD for PROs. Conclusions Despite home spirometry being feasible for most patients and centres, technical and analytical challenges in the home-based assessments prevented firm conclusions regarding disease behaviour. This highlights that further optimisation of the technology and analysis methods is required before widespread implementation. Trial Registration NCT03261037. Supplementary Information The online version contains supplementary material available at 10.1007/s12325-021-01790-y.

Physical activity measurements in individuals with interstitial lung disease: a systematic review and meta-analysis

“…Table 1 shows the characteristics of the included studies. Of the 40 studies, 11 studies [ 9 , 21 , 34 , 35 , 37 , 46 , 49 – 52 , 58 ] were intervention studies, 13 studies [ 3 , 7 , 8 , 22 , 29 , 30 , 33 , 36 , 41 , 43 , 47 , 54 , 56 ] were cohort studies and 16 studies [ 4 , 5 , 10 , 31 , 32 , 38 – 40 , 42 , 44 , 45 , 48 , 53 , 55 , 57 , 59 ] were cross-sectional studies. Of the 16 cross-sectional studies, one was a validation study of a PA questionnaire [ 8 ].…”

“…15 studies included patients with ILD due to any causes [ 8 , 21 , 30 – 34 , 37 , 46 – 48 , 51 , 53 , 55 , 59 ], 15 studies included only idiopathic pulmonary fibrosis (IPF) [ 5 , 7 , 9 , 22 , 29 , 40 – 43 , 45 , 49 , 50 , 54 , 56 , 58 ], seven studies included only sarcoidosis [ 4 , 35 , 36 , 38 , 39 , 52 , 57 ] and three studies included another ILD subtype [ 3 , 10 , 44 ]. The total sample size of each study ranged from 13 [ 46 ] to 629 [ 57 ].…”

“…12 studies did not report the sensor location [ 21 , 31 , 32 , 35 , 37 , 38 , 40 , 43 , 50 , 52 , 57 , 58 ], nine studies located the monitors at the arm (upper arm: seven studies [ 3 , 22 , 36 , 39 , 44 , 55 , 56 ]; not specified: two studies [ 5 , 49 ]), three studies at the wrist [ 30 , 34 , 51 ], four studies at the waist [ 29 , 42 , 45 , 59 ], two studies at the hip [ 41 , 48 ], one study at the upper thigh [ 4 ], one study at the wrist and waist [ 8 ], and one study wrist or waist [ 10 ].…”

Holistic management of patients with progressive pulmonary fibrosis

“…Single centre studies have shown that data acquired from home is reliable when compared with hospital spirometry [82][83][84]. However, multicentre studies have reported problems with implausible outliers, long-term missing data, and weak correlations between longitudinal patterns in home and hospital spirometry [85,86]. Artificial intelligence may help in developing algorithms to standardise acceptability and usability of the spirometric manoeuvre and deliver real-time automated feedback to patients [87].…”

Role of the internet of medical things in care for patients with interstitial lung disease

“…The first studies, in patients with IPF, used paper diaries to collect home spirometry results, which hampers use in clinical practice [ 18 , 20 ]. Moreover, some studies encountered technical problems, such as connection issues with the spirometers, and large variability of the data [ 21 ▪▪ , 22 ]. Many of these problems can be overcome by using the IoMT.…”