Background Health-related quality of life (HRQL) in interstitial lung disease (ILD) patients is impaired. We aimed to identify baseline predictors for HRQL decline within a 12-month observation period. Methods We analyzed 194 ILD patients from two German ILD-centers in the observational HILDA study. We employed the disease-specific King’s Brief Interstitial Lung Disease questionnaire (K-BILD) with the subdomains ‘psychological impact’, ‘chest symptoms’ and ‘breathlessness and activities’, and the generic EQ-5D Visual Analog Scale (VAS). We evaluated how many patients experienced a clinically meaningful decline in HRQL. Subsequently, we investigated medical and sociodemographic factors as potential predictors of HRQL deterioration. Results Within the study population (34.0% male, Ø age 61.7) mean HRQL scores hardly changed between baseline and follow up (K-BILD: 52.8 vs. 52.5 | VAS: 60.0 vs. 57.3). On the intra-individual level, 30.4% (n = 59) experienced a clinically relevant deterioration in K-BILD total score and 35.4% (n = 68) in VAS. Lower baseline forced vital capacity (FVC) % predicted determined HRQL decline in K-BILD total score (ß-coefficient: − 0.02, p = 0.007), VAS (ß-coefficient: − 0.03, p < 0.0001), and in the subdomain ‘psychological impact’ (ß-coefficient: − 0.02, p = 0.014). Lower baseline diffusing capacity of carbon monoxide (DLCO) % predicted determined deterioration in ‘breathlessness and activities’ (ß-coefficient: − 0.04, p = 0.003) and ‘chest symptoms’ (ß-coefficient: − 0.04, p = 0.002). Additionally, increasing age predicted decline in ‘psychological impact’ (ß-coefficient: 0.06, p < 0.007). Conclusion Around a third of ILD patients experienced a clinically relevant HRQL deterioration in a 12-month period, which was associated with baseline lung function values in all K-BILD domains. As lung function values are time-dependent variables with possible improvements, in contrast to age and ILD subtype, it, thus, seems important to improve lung function and prevent its decline in order to maintain HRQL on the possibly highest level.

Introduction Treatments of interstitial lung diseases (ILDs) mainly focus on disease stabilization and relief of symptoms by managing inflammation or suppressing fibrosis by (in part costly) drugs. To highlight economic burden of drug treatment in different ILD-subtypes we assessed cost trends and therewith-associated drivers. Methods Using data from the German, observational HILDA study we estimated adjusted mean medication costs over 36-month intervals using one- and two-part Generalized Estimating Equation (GEE) regression models with a gamma distribution and log link. Next, we determined factors associated with costs. Results In Idiopathic pulmonary fibrosis (IPF) mean per capita medication costs increased from €1442 before to €11,000€ at the end of study. In non-IPF subtypes, the increase took place at much lower level. Mean per capita ILD-specific medication costs at the end of the study ranged between €487 (other ILD) and €9142 (IPF). At baseline, higher FVC %predicted values were associated with lower medication costs in IPF (−9%) and sarcoidosis (−1%). During follow up higher comorbidity burden escalated costs in progressive fibrosing ILD (PF-ILD) (+52%), sarcoidosis (+60%) and other ILDs (+24%). The effect of disease duration was not uniform, with cost savings in PF-ILD (−8%) and sarcoidosis (−6%), but increased spending in IPF (+11%). Conclusion Pharmacological management of ILD, in particular of IPF imposes a substantial economic burden on the healthcare system. Strategies to reduce comorbidity burden and early treatment may reduce the impact of ILDs on the healthcare system.

BackgroundNo data on healthcare utilisation and associated costs for the many rare entities of children’s interstitial lung diseases (chILD) exist. This paper portrays healthcare utilisation structures among individuals with chILD, provides a pan-European estimate of a 3-month interval per-capita costs and delineates crucial cost drivers.MethodsBased on longitudinal healthcare resource utilisation pattern of 445 children included in the Kids Lung Register diagnosed with chILD across 10 European countries, we delineated direct medical and non-medical costs of care per 3-month interval. Country-specific utilisation patterns were assessed with a children-tailored modification of the validated FIMA questionnaire and valued by German unit costs. Costs of care and their drivers were subsequently identified via gamma-distributed generalised linear regression models.ResultsDuring the 3 months prior to inclusion into the registry (baseline), the rate of hospital admissions and inpatient days was high. Unadjusted direct medical per capita costs (€19 818) exceeded indirect (€1 907) and direct non-medical costs (€1 125) by far. Country-specific total costs ranged from €8 713 in Italy to €28 788 in Poland. Highest expenses were caused by the disease categories ‘diffuse parenchymal lung disease (DPLD)-diffuse developmental disorders’ (€45 536) and ‘DPLD-unclear in the non-neonate’ (€47 011). During a follow-up time of up to 5 years, direct medical costs dropped, whereas indirect costs and non-medical costs remained stable.ConclusionsThis is the first prospective, longitudinal study analysing healthcare resource utilisation and costs for chILD across different European countries. Our results indicate that chILD is associated with high utilisation of healthcare services, placing a substantial economic burden on health systems.

Background: Health-related quality of life (HRQL) in interstitial lung disease (ILD) patients is impaired. We aimed to identify baseline predictors for HRQL decline within a 12-month observation period. Methods: We analyzed 194 ILD patients from two German ILD-centers in the observational HILDA study. We employed the disease-specific King’s Brief Interstitial Lung Disease questionnaire (K-BILD) with the subdomains ‘psychological impact’, ‘chest symptoms’ and ‘breathlessness and activities’, and the generic EQ-5D Visual Analog Scale (VAS). We evaluated how many patients experienced a clinically meaningful decline in HRQL. Subsequently, we investigated medical and sociodemographic factors as potential predictors of HRQL deterioration. Results: Within the study population (34.0% male, Ø age 61.7) mean HRQL scores hardly changed between baseline and follow up (K-BILD: 52.8 vs. 52.5 | VAS: 60.0 vs. 57.3). On the intra-individual level, 30.4% (n = 59) experienced a clinically relevant deterioration in K-BILD total score and 35.4% (n = 68) in VAS. Lower baseline forced vital capacity % predicted determined HRQL decline in K-BILD total score (ß– coefficient -0.02, p = 0.007), VAS (ß–coefficient -0.03, p < 0.0001), and in the subdomain ‘psychological impact’ (ß– coefficient -0.02, p = 0.014). Lower baseline diffusing capacity of carbon monoxide % predicted determined deterioration in ‘breathlessness and activities’ (ß– coefficient -0.04, p = 0.003) and ‘chest symptoms’ (ß– coefficient -0.04, p = 0.002). Additionally, increasing age predicted decline in ‘psychological impact’ (ß–coefficient 0.06, p < 0.007). Conclusion: A third of ILD patients experience a clinically relevant HRQL deterioration within 12 months, which is mainly predicted by lung function baseline value.

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and 95.5% respectively. Simplicity was high among all the parameters (96.8%) in the scale content validity index(S-CVI). The internal consistency of the questionnaire measured using Cronbach's alpha was found 0.9. A total of 46 patients completed, 5 were drop out. Asthma (21.95%) and COPD patients (48.75%) group majority were male patients. Majority of asthma patient (34.14%) were non alcoholic and non smoker, whereas majority of COPD patients were (39.02%) were alcoholic and smoker. The questionnaire analysis was able to measure the change in medication adherence. Conclusions: The prepared medication adherence questionnaire was capable to assess the change in medication adherence. COPD patient were having better adherence than asthma patients.