Severe asthma trajectories in adults: findings from the NORDSTAR cohort.

BACKGROUND: There is limited evidence on the pathways leading to severe asthma and we are presently unable to effectively predict the progression of the disease. We aimed to describe the longitudinal trajectories leading to severe asthma and to describe clinical events preceding disease progression in a nationwide population of patients with severe asthma. METHODS: We conducted an observational study based on Swedish data from the NORdic Dataset for aSThmA Research (NORDSTAR) research collaboration platform. We identified adult patients with severe asthma in 2018 according to the European Respiratory Society/American Thoracic Society definition and used latent class analysis to identify trajectories of asthma severity over a 10-year retrospective period from 2018. RESULTS: Among 169 128 asthma patients, we identified 4543 severe asthma patients. We identified four trajectories of severe asthma that were labelled as: trajectory 1 "consistently severe asthma" (n=389 (8.6%)), trajectory 2 "gradual onset severe asthma" (n=942 (20.7%)), trajectory 3 "intermittent severe asthma" (n=1685 (37.1%)) and trajectory 4 "sudden onset severe asthma" (n=1527 (33.6%)). "Consistently severe asthma" had a higher daily inhaled corticosteroid dose and more prevalent osteoporosis compared with the other trajectories. Patients with "gradual onset severe asthma" and "sudden onset severe asthma" developed type 2-related comorbidities concomitantly with development of severe asthma. In the latter group, this primarily occurred within 1-3 years preceding onset of severe asthma. CONCLUSIONS: Four distinct trajectories of severe asthma were identified illustrating different patterns of progression of asthma severity. This may eventually enable the development of better preventive management strategies in severe asthma.

Rapid implementation of home oxygen treatment and remote monitoring for COVID-19 patients at the verge of the Omicron wave in Turku, Finland.

BACKGROUND: In Turku, Finland, we introduced a home oxygen treatment and app-based monitoring program for hospitalized COVID-19 patients to facilitate an early discharge during the Omicron wave. In this case series we explore the clinical parameters of patients enrolled in the program and evaluate the cost-benefit and safety issues of the program. METHODS: Hospitalized COVID-19 patients with marked hypoxemia but otherwise in stable condition were screened from Turku City Hospital and Turku University Hospital by treating doctors for eligibility in the program. Peripheral oxygen saturation of > 92% and breathing frequency < 30/min in rest with oxygen supplementation were among the criteria. All patients actively participating in the program between 10th of January 2022 and 30th of September 2022 were included in this case series. Clinical data of hospitalization and monitoring were analysed, and cost-benefit evaluation was based on the number of saved hospitalization days. RESULTS: Nineteen COVID-19 patients were included in this case series and recruited from three different hospital departments in the Turku city region, South-West Finland. All patients were male, the median age was 59 years and the median duration of hospitalization before enrolment in the program was 6 days (range 3-20 days). The median duration of home oxygen treatment was 13 days (range 3-72 days) and the median duration of home monitoring was 18 days (range 7-41 days). A total of 210,5 hospital days were prevented, resulting in savings of €144,490 of healthcare expenditure (on average 9 days and €7,605 per patient). No major safety issues were reported during the program. CONCLUSIONS: In our case series, home oxygen treatment combined with home monitoring was safe and economically beneficial. Application based monitoring could be considered in other post-acute pulmonary conditions to reduce hospitalization and healthcare costs.

Clinical Characteristics and Disease Course of Fibrosing Interstitial Lung Disease Patients in a Real-World Setting.

Background and Objectives: This single-center retrospective study was conducted to describe clinical characteristics and the disease course of patients with interstitial lung diseases (ILD). Materials and Methods: The study included adult patients with fibrosing ILD (IPF, non-IPF fibrosing ILD (F-ILD), and non-IPF progressive pulmonary fibrosis (PPF)) treated between 2014 and 2017. Patients were followed annually from the first visit until the end of the study period in 2019. Data were collected from the Turku University Hospital data lake and analyzed using descriptive statistics. Results: 591 patients formed the patient cohort: 110 had IPF, 194 F-ILD, 142 PPF, and the remaining 145 patients were uncertain, F-ILD-U, whose disease progression nature could not be confirmed by FVC measurements. There were more males in each patient group and median age of the groups was similar, although there were younger patients in the PPF, F-ILD, and F-ILD-U groups. PPF patients had more UIP pattern than F-ILD patients. Exposure-related ILDs were clearly the most found ILD diagnoses for both PPF and F-ILD, followed by unclassifiable IIP. Baseline FVC % predicted reduction in every group was moderate. Half of the patients in each group had comorbidities, and the most common were cardiovascular diseases, diabetes, sleep apnea, and chronic lower respiratory diseases; F-ILD-U patients had malignant diseases as well. IPF patients had less medications than the other groups. Glucocorticoids were the most used medication in all patient groups. More PPF and F-ILD patients remained in the follow-up than IPF and F-ILD-U patients. Similarly, mortality of F-ILD-U was the highest, followed by IPF. Evolvement of lung function, oxygen use, and number of acute hospitalizations were similar for IPF and PPF patients whereas the corresponding results were always better for F-ILD patients. Conclusions: The disease course of IPF and PPF was similar, and PPF patient amount exceeded the amount of IPF patients.

Effectiveness of mepolizumab in patients with severe eosinophilic asthma: results from real-world clinical practice in Finland.

OBJECTIVES: Mepolizumab treatment provides clinical benefits for patients with severe eosinophilic asthma in randomized controlled trials. However, real-world data for patients in Finland are lacking. METHODS: This retrospective, non-interventional, chart review study included patients with severe eosinophilic asthma ≥18 years of age initiating mepolizumab between January 1, 2016 and January 31, 2019 at three investigational sites in Finland. Patient characteristics during the 12 months prior to mepolizumab initiation (baseline) were recorded and primary and secondary endpoints included changes from baseline in disease outcomes during follow-up (up to 24 months following mepolizumab initiation). Exploratory endpoints included association between patient characteristics and exacerbation frequency/annual cumulative oral corticosteroid (OCS) dose. RESULTS: Overall, 51 patients were included (mean 17.8 months follow-up). At baseline, patients had a mean (standard deviation) blood eosinophil count of 550 (410) cells/µL; impaired lung function and health-related quality of life; poor symptom control; frequent exacerbations (2.78/year); and 90% were using OCS (mean: 9.80 mg/day). At the last follow-up visit, reductions from baseline in blood eosinophil count (84%) and fractional exhaled nitric oxide (26%) were observed, as were improvements in Asthma Quality of Life Questionnaire score (36%) and Asthma Control Test score (34%). Reductions in the mean number of annual exacerbations (82%) and mean daily OCS dose (39%) were also seen; reductions were observed even after adjustment for several patient baseline characteristics. CONCLUSIONS: Results are consistent with previous randomized clinical trials, indicating that Finnish patients experience clinically relevant improvements when treated with mepolizumab in real-world clinical practice.

Development of self-assessed work ability among middle-aged asthma patients-a 10 year follow-up study.

OBJECTIVE: The prevalence of asthma has been growing among working age people over the last decades. In this study, we examine the development of Work Ability Score (WAS) among middle-aged asthmatics in a longitudinal setting, in order to find risk factors for poor development. METHODS: We followed the development of WAS trends during 10 years in a cohort of 529 middle-aged asthmatics, who were active in working life. Follow-up questionnaires were mailed in years 1, 2, 4, 6, 8, and 10. To study the development of WAS over time, we computed the discrete Frechet distance, which describes the similarity between the shapes of WAS curves. RESULTS: Sixty-eight percent of the patients' WAS remained good or excellent throughout the follow-up period, while 24% of the patients WAS trend remained moderate. However, in 8%, the WAS was poor already in baseline and decreased further throughout the study. Using logistic regression, the moderate/poor development was associated significantly with high body mass index (BMI), pack years, adult onset asthma, physically strenuous work, number of co-morbidities, especially in psychiatric conditions, hypertension, and gastroesophageal reflux disease(GERD). When the model was adjusted for age and gender, adulthood onset of asthma and pack years lost their significance. Based on medication (high dose of inhaled corticosteroids (ICS) and second controller in use), 8% of the patients had severe asthma. CONCLUSION: In the great majority of middle-aged asthma patients WAS remained stable throughout the follow-up period. However, 8% of the patients, who had more severe asthma and multiple co-morbidities, showed significantly poorer outcomes.

Individual FEV1 Trajectories Can Be Identified from a COPD Cohort.

OBJECTIVE: We aim to make use of clinical spirometry data in order to identify individual COPD-patients with divergent trajectories of lung function over time. STUDY DESIGN AND SETTING: Hospital-based COPD cohort (N = 607) was followed on average 4.6 years. Each patient had a mean of 8.4 spirometries available. We used a Hierarchical Bayesian Model (HBM) to identify the individuals presenting constant trends in lung function. RESULTS: At a probability level of 95%, one third of the patients (180/607) presented rapidly declining FEV1 (mean -78 ml/year, 95% CI -73 to -83 ml) compared to that in the rest of the patients (mean -26 ml/year, 95% CI -23 to -29 ml, p ≤ 2.2 × 10(-16)). Constant improvement of FEV1 was very rare. The rapid decliners more frequently suffered from exacerbations measured by various outcome markers. CONCLUSION: Clinical data of unique patients can be utilized to identify diverging trajectories of FEV1 with a high probability. Frequent exacerbations were more prevalent in FEV1-decliners than in the rest of the patients. The result confirmed previously reported association between FEV1 decline and exacerbation rate and further suggested that in clinical practice HBM could improve the identification of high-risk individuals at early stages of the disease.

CHRNA5/CHRNA3 Locus Associates with Increased Mortality among Smokers.

Polymorphisms in the nicotinic acetylcholine receptor gene (CHRNA5/CHRNA3 locus) have been associated with several smoking related traits such as nicotine dependence, cigarette consumption, smoking cessation, lung cancer, and COPD. The aim of this candidate gene study was to study the locus among the Finnish COPD patients and long-term smokers with regard to COPD risk, smoking behavior, cancer, and all-cause mortality. Genotyping of rs1051730, the locus tagging SNP was done in two longitudinal cohorts: Finnish COPD patients (N = 575, 74% men) and long-term smokers, all men (N = 1911). Finnish population sample (N = 1730) was used as controls. The analyses were done using logistic and Cox regression. The main findings were that the minor allele increased the risk of COPD when compared to the Finnish population at large (OR = 1.4, 95% CI 1.2-1.7, p = 3.2 × 10-5). Homozygosity for the risk allele was associated in both cohorts with all-cause mortality (crude HR 2.2, 95% CI 1.2-3.8 and 1.3, 95% CI 1.1-1.5, respectively), with any type of cancer (crude OR 2.3, 95% CI 1.0-5.1) among the COPD patients and with the number of pack-years (crude OR 1.4, 95% CI 1.1-1.9) among the male smokers. CHRNA5/CHRNA3 locus tagged by rs1051730, which has been previously associated with several smoking related diseases was now shown to be associated also with increased all-cause mortality among long-term smokers with or without clinical COPD further emphasizing the clinical importance of the finding.

Co-morbidities are the key nominators of the health related quality of life in mild and moderate COPD.

BACKGROUND: Co-morbidities are common in chronic obstructive pulmonary disease (COPD). We assessed the contribution of common co-morbidities on health related quality of life (HRQoL) among COPD patients. METHODS: Using both generic (15D) and respiratory-specific (AQ20) instruments, HRQoL was assessed in a hospital based COPD population (N = 739, 64% males, mean age 64 years, SD 7 years) in this observational study with inferential analysis. The prevalence of their co-morbidities was compared with those of 5000 population controls. The patients represented all severity stages of COPD and the patterns of common concomitant disorders differed between patients. RESULTS: Co-morbidities such as psychiatric conditions, alcohol abuse, cardiovascular diseases, and diabetes were more common among COPD patients than in age and gender matched controls. Psychiatric conditions and alcohol abuse were the strongest determinants of HRQoL in COPD and could be detected by both 15D (Odds Ratio 4.7 and 2.3 respectively) and AQ20 (OR 2.0 and 3.0) instruments. Compared to respiratory specific AQ20, generic 15D was more sensitive to the effects of comorbidities while AQ20 was slightly more sensitive for the low FEV1. FEV1 was a strong determinant of HRQoL only at more severe stages of disease (FEV1 < 40% of predicted). Poor HRQoL also predicted death during the next five years. CONCLUSIONS: The results suggest that co-morbidities may impair HRQoL at an early stage of the disease, while bronchial obstruction becomes a significant determinant of HRQoL only in severe COPD.

[Update on current care guideline: chronic obstructive pulmonary disease (COPD)]. / Keuhkoahtaumatauti.

Clinical serverity of COPD is based not only on the grade of obstruction in spirometry, but also on symptoms and risk of exacerbations. Symptoms can be defined by questionnaires, such as CAT-test (COPD assessment -test) or mMRC (modified Medical Research Council -test). Smoking cessation and physical activity are important treatment options. Pharmacological treatment is selected by symptoms, risk of exacerbations and co-occurrence of asthma and COPD. Non-invasive ventilation (NIV) is recommended in the treatment of severe hypercapnic exacerbations. Palliative treatment of end stage COPD is included in the guidelines.

[Work aggravated asthma]. / Tyon pahentama astma.

One out of five working persons with asthma has work-related respiratory symptoms. When exploring the symptoms of a working-age patient it is essential to survey the job description and working conditions. Early intervention in the factors aggravating the respiratory symptoms will decrease morbidity, maintain working capacity and improve the quality of life. Occupational health service and the employer play a central role in identifying and decreasing the exposure factors in the working environment as well as in patient guidance for asthma therapy and protecting from the stimuli. The working capacity of an asthmatic person can be improved by applying vocational rehabilitation.

Bayesian predictors of very poor health related quality of life and mortality in patients with COPD.

BACKGROUND: Chronic obstructive pulmonary disease (COPD) is associated with increased mortality and poor health-related quality of life (HRQoL) compared with the general population. The objective of this study was to identify clinical characteristics which predict mortality and very poor HRQoL among the COPD population and to develop a Bayesian prediction model. METHODS: The data consisted of 738 patients with COPD who had visited the Pulmonary Clinic of the Helsinki and Turku University Hospitals during 1995-2006. The data set contained 49 potential predictor variables and two outcome variables: survival (dead/alive) and HRQoL measured with a 15D instrument (very poor HRQoL < 0.70 vs. typical HRQoL ≥ 0.70).In the first phase of model validation we randomly divided the material into a training set (n = 538), and a test set (n = 200). This procedure was repeated ten times in random fashion to obtain independently created training sets and corresponding test sets. Modeling was performed by using the training set, and each model was tested by using the corresponding test set, repeated in each training set. In the second phase the final model was created by using the total material and eighteen most predictive variables. The performance of six logistic regressions approaches were shown for comparison purposes. RESULTS: In the final model, the following variables were associated with mortality or very poor HRQoL: age at onset, cerebrovascular disease, diabetes, alcohol abuse, cancer, psychiatric disease, body mass index, Forced Expiratory Volume (FEV1) % of predicted, atrial fibrillation, and prolonged QT time in ECG. The prediction accuracy of the model was 77%, sensitivity 0.30, specificity 0.95, positive predictive value 0.68, negative predictive value 0.78, and area under the ROC curve 0.69. While the sensitivity of the model reminded limited, good specificity, moderate accuracy, comparable or better performance in classification and better performance in variable selection and data usage in comparison to the logistic regression approaches, and positive and negative predictive values indicate that the model has potential in predicting mortality and very poor HRQoL in COPD patients. CONCLUSION: We developed a Bayesian prediction model which is potentially useful in predicting mortality and very poor HRQoL in patients with COPD.

Virtual communication is commonly used in Finnish interstitial lung disease multidisciplinary meetings.

Multidisciplinary meeting (MDM) is a core element in the diagnosis of interstitial lung diseases (ILD). The aim of the study was to investigate the implementation and key elements related to ILD MDMs in Finnish specialized care, which is characterized by long travel distances and a large number of small centers treating patients suffering from ILDs. An electronic questionnaire was sent to ILD experts working at five academic centers of Finland regarding the implementation of ILD MDMs with the focus on utilization of virtual communication. Responses were received from all academic centers of Finland (n = 5) whose catchment areas cover all of Finland. ILD MDMs were organized in each center approximately every two weeks and the core participants included a radiologist, respiratory physicians, junior staff, pathologist and a rheumatologist. All non-academic centers could refer their patients to be evaluated in ILD MDM of an academic center. Virtual communication was utilized by all academic centers in the implementation of ILD MDMs, being most common among small centers located in Eastern and Northern Finland. Virtual access to ILD MDM of an academic center was available in most parts of Finland, enabling small centers to benefit from the ILD expertise of academic centers.

Prevalence and management of severe asthma in the Nordic countries: findings from the NORDSTAR cohort.

Background: Real-life evidence on prevalence and management of severe asthma is limited. Nationwide population registries across the Nordic countries provide unique opportunities to describe prevalence and management patterns of severe asthma at population level. In nationwide register data from Sweden, Norway and Finland, we examined the prevalence of severe asthma and the proportion of severe asthma patients being managed in specialist care. Methods: This is a cross-sectional study based on the Nordic Dataset for Asthma Research (NORDSTAR) research collaboration platform. We identified patients with severe asthma in adults (aged ≥18 years) and in children (aged 6-17 years) in 2018 according to the European Respiratory Society/American Thoracic Society definition. Patients managed in specialist care were those with an asthma-related specialist outpatient contact (only available in Sweden and Finland). Results: Overall, we identified 598 242 patients with current asthma in Sweden, Norway and Finland in 2018. Among those, the prevalence of severe asthma was 3.5%, 5.4% and 5.2% in adults and 0.4%, 1.0%, and 0.3% in children in Sweden, Norway and Finland, respectively. In Sweden and Finland, 37% and 40% of adult patients with severe asthma and two or more exacerbations, respectively, were managed in specialist care; in children the numbers were 56% and 41%, respectively. Conclusion: In three Nordic countries, population-based nationwide data demonstrated similar prevalence of severe asthma. In children, severe asthma was a rare condition. Notably, a large proportion of patients with severe asthma were not managed by a respiratory specialist, suggesting the need for increased recognition of severe asthma in primary care.

Epidemiology of interstitial lung diseases and their progressive-fibrosing behaviour in six European countries.

The PERSEIDS study aimed to estimate incidence/prevalence of interstitial lung diseases (ILDs), fibrosing interstitial lung diseases (F-ILDs), idiopathic pulmonary fibrosis (IPF), systemic sclerosis-associated ILD (SSc-ILD), other non-IPF F-ILDs and their progressive-fibrosing (PF) forms in six European countries, as current data are scarce. This retrospective, two-phase study used aggregate data (2014-2018). In Phase 1, incident/prevalent cases of ILDs above were identified from clinical databases through an algorithm based on codes/keywords, and incidence/prevalence was estimated. For non-IPF F-ILDs, the relative percentage of subtypes was also determined. In Phase 2, a subset of non-IPF F-ILD cases was manually reviewed to determine the percentage of PF behaviour and usual interstitial pneumonia-like (UIP-like) pattern. A weighted mean percentage of progression was calculated for each country and used to extrapolate incidence/prevalence of progressive-fibrosing ILDs (PF-ILDs). In 2018, incidence/105 person-years ranged between 9.4 and 83.6 (ILDs), 7.7 and 76.2 (F-ILDs), 0.4 and 10.3 (IPF), 6.6 and 71.7 (non-IPF F-ILDs), and 0.3 and 1.5 (SSc-ILD); and prevalence/105 persons ranged between 33.6 and 247.4 (ILDs), 26.7 and 236.8 (F-ILDs), 2.8 and 31.0 (IPF), 22.3 and 205.8 (non-IPF F-ILDs), and 1.4 and 10.1 (SSc-ILD). Among non-IPF F-ILDs, sarcoidosis was the most frequent subtype. PF behaviour and UIP-like pattern were present in a third of non-IPF F-ILD cases each and hypersensitivity pneumonitis showed the highest percentage of progressive behaviour. Incidence of PF-ILDs ranged between 2.1 and 14.5/105 person-years, and prevalence between 6.9 and 78.0/105 persons. To our knowledge, PERSEIDS is the first study assessing incidence, prevalence and rate of progression of ILDs across several European countries. Still below the threshold for orphan diseases, the estimates obtained were higher and more variable than reported in previous studies, but differences in study design/population must be considered.

Comparison between the disease-specific Airways Questionnaire 20 and the generic 15D instruments in COPD.

BACKGROUND: Given that the assessment of health-related quality of life (HRQoL) is an essential outcome measure to optimize chronic obstructive pulmonary disease (COPD) patient management, there is a need for a short and fast, reliable and valid instrument for routine use in clinical practice. The objective of this study was to analyse the relationship between the disease-specific Airways questionnaire (AQ20) and the generic 15D health-related quality of life (HRQoL) instrument simultaneously in a large cohort of patients with COPD. We also compare the HRQoL of COPD patients with that of the general population. METHODS: The AQ20 and 15D were administered to 739 COPD patients representing an unselected hospital-based COPD population. The completion rates and validity of, and correlations among the questions and dimension scores were examined. A factor analysis with varimax rotation was performed in order to find subsets of highly correlating items of the questionnaires. RESULTS: The summary scores of AQ20 and 15D were highly correlated (r = - 0.71, p < 0.01). In AQ20 over 50% of patients reported frequent cough, breathlessness during domestic work, and chest problem limiting their full enjoyment of life. 15D results showed a noteworthy decrease of HRQoL in breathing, mobility, sleeping, usual activities, discomfort and symptoms, vitality, and sexual activity (scores ≤ 0.75). Compared to the age- and gender-standardized Finnish general population, the COPD patients were statistically significantly worse off on 13 of 15 dimensions. CONCLUSIONS: The AQ20 and 15D summary scores are comparable in terms of measuring HRQoL in COPD patients. The data support the validity of 15D to measure the quality of life in COPD. COPD compromises the HRQoL broadly, as reflected by the generic instrument. Both questionnaires are simple and short, and could easily be used in clinical practice with high completion rates.

Overlap syndrome of asthma and COPD predicts low quality of life.

BACKGROUND: In clinical practice, patients whose airway disease shares features of both asthma and chronic obstructive pulmonary disease (COPD) remain poorly recognized. MATERIAL AND METHODS: The study population consisted of 1546 patients with a diagnosis of asthma or COPD or both. Based on patient-reported outcomes and retrospective medical record data, the study population was divided into three groups: ( 1 ) asthma only, ( 2 ) COPD only, and ( 3 ) both asthma and COPD (overlap syndrome group). We evaluated patient characteristics associated with health-related quality of life (HRQoL). RESULTS: In many respects, the overlap group fell between the asthma and COPD groups. In the overlap group, however, HRQoL was the poorest of all. In the logistic regression model, with the asthma group as the reference, both the overlap and the COPD group showed higher risk for low HRQoL [odd ratio (OR): 1.9; 95% confidence interval (CI): 1.2-3.2; and OR: 1.8, 95% CI: 1.0-3.2; respectively]. In addition, female gender, obesity, duration of disease, disability pension, and coexisting cardiovascular disease were associated with low HRQoL across the study population. CONCLUSIONS: Patients with overlapping asthma and COPD differed from those patients with asthma or COPD only. Overlap syndrome was associated with low HRQoL.

[Peak expiratory flow monitoring--the basis for diagnosing occupational asthma--guidelines by the Finnish Expert Group on Occupational Lung Diseases]. / Ammattikeuhkosairauksien asiantuntijaryhmän (AKAR) suositus. PEF-työpaikkaseuranta--ammattiastma-diagnostiikan perusta.

Occupational exposures can cause adult-onset asthma. Early diagnosis and early avoidance of further exposure to causative agent improves the prognosis of occupational asthma. Occupational and primary care health services have an important role in the identification of new cases of occupational asthma. For the diagnosis of occupational asthma, serial peak expiratory flow (PEF) measurements should be performed in an early stage. Although it requires an effort from the patient, high quality recordings offer the best approach to assess the relationship between workplace exposure and respiratory symptoms. Good guidance and performance of serial PEF measurements in primary care is recommended and is worth the effort.

[Specific challenge tests in occupational asthma--guidelines by the Finnish Expert Group on Occupational Lung Diseases]. / Ammattikeuhkosairauksien asiantuntijaryhmän (AKAR) suositus. Hengitysteiden spesifiset altistuskokeet ammattiastman osoittamisessa.

In a specific inhalation challenge (SIC) test the patient inhales an occupational agent in controlled environment and the subsequent asthmatic reaction is monitored. SIC is considered as the reference standard when confirming the diagnosis of sensitizer-induced occupational asthma. However, SIC is not always needed for the diagnosis; in many cases the causal relationship between an occupational agent and asthma can be shown also with serial peak flow measurements and specific immunologic testing. SIC is invaluable in identifying new occupational airway sensitizers. This is essential for preventing occupational asthma in the future.

Economic Burden and Management of Systemic Sclerosis-Associated Interstitial Lung Disease in 8 European Countries: The BUILDup Delphi Consensus Study.

INTRODUCTION: Systemic sclerosis (SSc) is a rare chronic autoimmune disease characterised by microvascular damage, immune dysregulation and fibrosis, affecting the skin, joints and internal organs. Interstitial lung disease (ILD) is frequently associated with systemic sclerosis (SSc-ILD), leading to a poor prognosis and a high mortality rate. The aim of the BUILDup study (BUrden of Interstitial Lung Disease Consensus Panel) was to investigate the overall disease management and to estimate the social and economic burden of SSc-ILD across 8 European countries. METHODS: A modified Delphi method was used to obtain information on the management of SSc-ILD patients among 40 specialists (panellists) from 8 European countries. Average annual costs per patient and country were estimated by means of a direct cost-analysis study. RESULTS: The panellists had managed 805 SSc-ILD patients in the last year, 39.1% with limited (L-SSc-ILD) and 60.9% with extensive (E-SSc-ILD) disease. Of these, 32.8% of the panellists started treatment at diagnosis, 42.3% after signs of deterioration/progression and 24.7% when the disease had become extensive. The average annual cost of SSc-ILD per patient ranged from €6191 in Greece to €25,354 in Sweden. Main cost drivers were follow-up procedures, accounting for 80% of the total annual costs. Hospitalisations were the most important cost driver of follow-up costs. Healthcare resource use was more important for E-SSc-ILD compared to L-SSc-ILD. Early retirement was taken by 40.4% of the patients with an average of 11.9 years before the statutory retirement age. CONCLUSIONS: SSc-ILD entails not only a clinical but also a social and economic burden, and is higher for E-SSc-ILD.