Idiopathic pulmonary fibrosis (IPF) is characterized by progressive loss of lung function with high mortality within the first 5 years from diagnosis. In 2011–2014, two drugs, pirfenidone and nintedanib, have been approved worldwide for prevention of IPF progression. National IPF-registries have been established in both Finland and Sweden. Our study explored potential differences in the care of IPF in these two countries. Patients included consecutively in the Finnish and Swedish IPF-registries from January 1, 2014 through December 31, 2016 were included in the study. Data on demographics and lung function at the time of inclusion were collected. Access to antifibrotic drugs and data on disease outcomes, mortality and the proportion of patients who underwent lung transplantation, was collected during a 3-year follow up. One-hundred and fifty-two patients from the Finnish and 160 patients from the Swedish IPF-cohorts were included in the study. At inclusion, Finnish patients were significantly older than the Swedish patients (74.6 years vs 72.5 years, p = 0.017). The proportion of non-smokers was significantly higher in the Finnish cohort (41.7% vs 26.9%, p = 0.007). Forced vital capacity (FVC), % of predicted (78.2 vs 71.7 for Finnish and Swedish patients, respectively, p = 0.01) and diffusion capacity for carbon monoxide (DLCO), % of predicted (53.3 vs 48.2 for Finnish and Swedish patients, respectively, p = 0.002) were significantly higher in the Finnish cohort compared to the Swedish cohort at the time of inclusion. During the 3-year follow up period, 45 (29.6%) Finnish and 111 (69.4%) Swedish patients, respectively, were initiated on treatment with an antifibrotic drug (pirfenidone or nintedanib) (p < 0.001). When comparing possible determinants of treatment, patients with higher FVC % were less likely to start antifibrotic drugs (OR 0.96, 95%CI 0.93–1.00, p < 0.024). To be resident in Sweden was the main determinant for receiving antifibrotic drugs (OR 5.48, 95%CI 2.65–11.33, p < 0.0001). No significant difference in number of deaths and lung transplantation during the follow up period was found. This study highlights differences concerning how IPF patients are treated in Finland and Sweden. How these differences will influence the long-term outcome of these patients is unknown.

Background: Interstitial lung abnormalities (ILA) are incidental findings on chest computed tomography (CT). These patterns can present at an early stage of fibrotic lung disease. Our aim was to estimate the prevalence of ILA in the Swedish population, in particular in never-smokers, and find out its association with demographics, comorbidities and symptoms.Methods: Participants were recruited to the Swedish CArdioPulmonary BioImage Study (SCAPIS), a population-based survey including men and women aged 50–64 years performed at six university hospitals in Sweden. CT scan, spirometry and questionnaires were performed. ILA were defined as cysts, ground-glass opacities, reticular abnormality, bronchiectasis and honeycombing.Findings: Out of 29 521 participants, 14 487 were never-smokers and 14 380 were men. In the whole population, 2870 (9.7%) had ILA of which 134 (0.5%) were fibrotic. In never-smokers, the prevalence was 7.9% of which 0.3% were fibrotic. In the whole population, age, smoking history, chronic bronchitis, cancer, coronary artery calcium score and high-sensitive C-reactive protein were associated with ILA. Both ILA and fibrotic ILA were associated with restrictive spirometric pattern and impaired diffusing capacity of the lung for carbon monoxide. However, individuals with ILA did not report more symptoms compared with individuals without ILA.Interpretation: ILA are common in a middle-aged Swedish population including never-smokers. ILA may be at risk of being underdiagnosed among never-smokers since they are not a target for screening.

Objectives: There is a lack of knowledge about whether occupational exposures increase the risk of emphysema, especially in never-smokers. Our objective was to determine if occupational exposures are associated with emphysema and impaired diffusing capacity. Methods: In the Swedish CArdioPulmonary bioImage Study (SCAPIS), persons from the general population aged 50–64 answered a questionnaire and underwent CT of the lung as well as assessment of the diffusing capacity of their lungs for carbon monoxide (DL _CO ), presented as DL _CO <lower limit of normal (LLN). Emphysema was defined as emphysema in any part of the lungs. Occupational exposures were assessed by a job exposure matrix based on longest held job. ORs with 95% CIs were calculated using logistic multivariable models. Results: In this cross-sectional study (27 370 persons including 13 981 never-smokers), occupational exposure to inorganic dust was associated with emphysema (OR 1.25, 95% CI 1.07 to 1.47), also among never-smokers, (OR 1.46, 95% CI 1.00 to 2.11). There were associations with DL _CO <LLN for occupational exposure to inorganic dust and vapour and gases. With all exposures in the same model, inorganic dust was associated with emphysema (OR 1.30, 95% CI 1.08 to 1.57), and vapour and gases were associated with DL _CO <LLN (OR 1.17, 95% CI 1.00 to 1.38). In those with emphysema and impaired DL _CO , there was an association with inorganic dust (OR 1.65, 95% CI 1.20 to 2.28), also among never-smokers (OR 3.79, 95% CI 1.35 to 10.63). Conclusions: Occupational exposures to inorganic dust are associated with emphysema. The association is stronger in those with the combination of emphysema and impaired DL _CO indicating serious exposure effects in the alveoli.

Background: Idiopathic pulmonary fibrosis (IPF) is characterized by progressive loss of lung function with high mortality within the first 5 years from diagnosis. In 2011–2014, two drugs, pirfenidone and nintedanib, have been approved worldwide for prevention of IPF progression. National IPF-registries have been established in both Finland and Sweden. Our study explored potential differences in the care of IPF in these two countries. Methods: Patients included consecutively in the Finnish and Swedish IPF-registries from January 1, 2014 through December 31, 2016 were included in the study. Data on demographics and lung function at the time of inclusion were collected. Access to antifibrotic drugs and data on disease outcomes, mortality and the proportion of patients who underwent lung transplantation, was collected during a 3-year follow up. Results: One-hundred and fifty-two patients from the Finnish and 160 patients from the Swedish IPF-cohorts were included in the study. At inclusion, Finnish patients were significantly older than the Swedish patients (74.6 years vs 72.5 years, p = 0.017). The proportion of non-smokers was significantly higher in the Finnish cohort (41.7% vs 26.9%, p = 0.007). Forced vital capacity (FVC), % of predicted (78.2 vs 71.7 for Finnish and Swedish patients, respectively, p = 0.01) and diffusion capacity for carbon monoxide (DLCO), % of predicted (53.3 vs 48.2 for Finnish and Swedish patients, respectively, p = 0.002) were significantly higher in the Finnish cohort compared to the Swedish cohort at the time of inclusion. During the 3-year follow up period, 45 (29.6%) Finnish and 111 (69.4%) Swedish patients, respectively, were initiated on treatment with an antifibrotic drug (pirfenidone or nintedanib) (p < 0.001). When comparing possible determinants of treatment, patients with higher FVC % were less likely to start antifibrotic drugs (OR 0.96, 95%CI 0.93–1.00, p < 0.024). To be resident in Sweden was the main determinant for receiving antifibrotic drugs (OR 5.48, 95%CI 2.65–11.33, p < 0.0001). No significant difference in number of deaths and lung transplantation during the follow up period was found. Conclusions: This study highlights differences concerning how IPF patients are treated in Finland and Sweden. How these differences will influence the long-term outcome of these patients is unknown.

Introduction: Interstitial lung abnormalities (ILA) are incidental findings on computed tomography (CT). These patterns can present as an early stage of fibrotic lung diseases. Our aim was to estimate the prevalence of ILA in the Swedish population and its association with demographics, chronic diseases, symptoms and lung function. Methods: Participants in the population-based Swedish CArdioPulmonary BioImage Study (SCAPIS) who had undergone a chest CT, were included. ILA were divided in nonfibrotic (ground-glass opacities, cysts and reticular pattern without bronchiectasis) and fibrotic (honeycombing and reticular pattern with traction bronchiectasis). The significance level after Bonferroni correction was <0.001. Results: Out of 29 521 participants aged 50-64 years, 2 870 (9.7%) had ILA of which 2 736 (9.3%) were nonfibrotic and 134 (0.5%) fibrotic. Individuals with ILA were older (58.5±4.3 vs 57.4±4.3, p<0.001), had more pack-years (18.2±14.4 vs 15.4±12.9, p<0.001), chronic bronchitis (7.1% vs 4.9%, p<0.001), hypertension (25.9% vs 22.5%, p<0.001), previous myocardial infarction or angina pectoris (3.3% vs 2.2%, p<0.001), dyspnea (Modified Medical Research Council Dyspnea Scale ³2 2.6% vs 1.7%, p<0.001) and lower lung volumes (FVC% 101.5±13.6 vs 102.8±12.9, p<0.001) compared with no ILA. Persons with fibrotic ILA were older (60.0±4.1 vs 58.4±4.3, p<0.001) and higher proportion were males (59.7% vs 46.8%, p<0.004) compared with nonfibrotic ILA. Conclusions: ILA was a common finding in this middle-aged population. Individuals with ILA were older, smoked more, had more chronic diseases, dyspnea and lower lung volumes compared with no ILA. Fibrotic ILA resembled idiopathic pulmonary fibrosis.

A 39 years old African man presented with fatigue, loss of weight and night sweats; radiology showed a possible usual interstitial pneumonia pattern. The patient missed follow-up visits, and presented again after 3 years with productive cough and general illness. Pulmonary function tests showed a decline of FVC compared to a previous investigation. The CT scans showed progression of the interstitial lung disease, and a multidisciplinary conference recommended to proceed with a surgical lung biopsy. Histopathology showed an atypical pattern, with bronchiolar metaplasia. A new multidisciplinary conference made a diagnosis of IPF, and the patient was treated with antifibrotic drugs with a good effect, reaching stability of lung function. This case report highlights the need to improve knowledge and to better characterize rare pulmonary diseases, and especially IPF, among African patients.