In bronchiectasis, poor physical capacity correlates with poor quality of life

Purpose Patients with bronchiectasis (BE) who suffer frequent exacerbations are likely to experience negative effects on quality of life (QoL) and require more healthcare utilization. We aimed to discover, in a cohort of Finnish BE patients, those risk factors that influence QoL. Methods Non-cystic fibrosis BE patients of a Helsinki University Hospital cohort were examined with high-resolution computed tomography (HRCT) of the chest. They completed a disease-specific quality of life-bronchiectasis (QoL-B) questionnaire in Finnish translation. We considered scores in the lowest quarter (25%) of that QoL-B scale to indicate poor QoL. The bronchiectasis severity index (BSI), FACED score, and modified Medical Research Council (mMRC) dyspnoea scale were used. Results Overall, of 95 adult BE patients, mean age was 69 (SD +/- 13) and 79% were women. From the cohort, 82% presented with chronic sputum production and exacerbations, at a median rate of 1.7 (SD +/- 1.6). The number of exacerbations (OR 1.7), frequent exacerbations (>= 3 per year) (OR 4.9), high BSI score (OR 1.3), and extensive disease (>= 3 lobes) (OR 3.7) were all predictive of poor QoL. Frequent exacerbations were associated with bronchial bacterial colonisation, low forced expiratory volume in 1 s (FEV1), and radiological disease severity. Based on the BSI, 34.1% of our cohort had severe disease, with 11.6% classified as severe according to their FACED score. The mMRC dyspnoea score (r = -0.57) and BSI (r = -0.60) correlated, in the QoL-B questionnaire, negatively with physical domain. Conclusion The strongest determinants of poor QoL in the cohort of Finnish BE patients were frequent exacerbations, radiological disease severity, and high BSI score. Neither comorbidities nor BE aetiology appeared to affect QoL. Reduced physical capacity correlated with dyspnoea and severe disease. Study registration University of Helsinki, Faculty of Medicine, 148/16.08.2017.Peer reviewe

Asthma as aetiology of bronchiectasis in Finland

Background: By definition bronchiectasis (BE) means destructed structure of normal bronchus as a consequence of frequent bacterial infections and inflammation. In many senses, BE is a neglected orphan disease. A recent pan-European registry study, EMBARC, has been set up in order to better understand its pathophysiology, better phenotype patients, and to individualize their management. Aim: To examine the aetiology and co-morbidity of BE in the capital area in Finland. Methods: Two hundred five patients with BE diagnosis and follow up visits between 2016 and 2017 in Helsinki University Hospital were invited to participate in the study. Baseline demographics, lung functions, imaging, microbiological, and therapeutic data, together with co-morbidities were entered into EMBARC database. Clinical characteristics, aetiologic factors, co-morbidities, and risk factors for extensive BE were explored. Results: To the study included 95 adult patients and seventy nine percent of the BE patients were women. The mean age was 69 years (SD +/- 13). Asthma was a comorbid condition in 68% of the patients but in 26% it was estimated to be the cause of BE. Asthma was aetiological factor for BE if it had been diagnosed earlier than BE. As 41% BE were idiopathic, in 11% the disorder was postinfectious and others were associated to rheumatic disease, Alpha-1-antitrypsin deficiency, IgG deficiency and Kartagener syndrome. The most common co-morbidities in addition to asthma were cardiovascular disease (30%), gastroesophageal reflux disease (26%), overweight (22%), diabetes (16%), inactive neoplasia (15%), and immunodeficiency (12%). Extensive BE was found in 68% of BE patients in whom four or more lobes were affected. Risk factors for extensive BE were asthma (OR 2.7), asthma as aetiology for BE (OR 4.3), and rhinosinusitis (OR 3.1). Conclusions: Asthma was associated to BE in 68% and it was estimated as aetiology in every fourth patient. However, retrospectively, it is difficult to exclude asthma as a background cause in patients with asthma-like symptoms and respiratory infections. We propose asthma as an aetiology factor for BE if it is diagnosed earlier than BE. Asthma and rhinosinusitis were predictive for extensive BE.Peer reviewe

Astman rooli bronkiektasiataudin etiologiana ja liitännäissairautena : systemaattinen kirjallisuuskatsaus

Bronkiektasiatautia – keuhkoputkien laajentumia keuhkoputkien pitkäkestoisen tulehduksen ja bakteeri-infektioiden aiheuttaman kudostuhon seurauksena – on pitkään pidetty harvinaisena sairautena ja sen on ajateltu kehittyvän pääasiallisesti keuhkotuberkuloosin ja kystisen fibroosin seurauksena. Viimeaikaiset tutkimukset ovat kuitenkin osoittaneet, että taudin etiologisia tekijöitä on enemmän kuin aikaisemmin on ajateltu, ja että tautitapauksista merkittävä osa jää ilman selkeästi osoitettavaa selittävää tekijää. Keuhkoahtaumataudin rooli taudin etiologisena tekijänä tunnetaan, mutta tutkimusnäyttö astman roolista bronkiektasiataudin aiheuttajana ja liitännäissairautena on niukkaa. Tavoitteemme oli arvioida astman roolia bronkiektasiataudin etiologiana ja liitännäissairautena systemaattisen kirjallisuuskatsauksen keinoin. Teimme tietokantahaun yleisempiin sähköisiin tietokantoihin. Tutkimukseen päätyi 45 artikkelia, joista 8 määritteli astman bronkiektasiataudin etiologiaksi, 18 liitännäissairaudeksi, ja 20 tutkimusta raportoi astman esiintyvyyttä bronkiektasiatautia sairastavilla potilailla tai bronkiektasiataudin esiintyvyyttä astmaa sairastavilla potilailla ilman kannanottoa astman etiologia- tai liitännäissairaussuhteeseen. Astman osuus bronkiektasiataudin etiologiana vaihteli välillä 3,3–26,9 %. Astmaa esiintyi liitännäissairautena bronkiektasiatautia sairastavien potilaiden populaatioissa 11–68 %:lla, ja bronkiektasiatautia astmapotilaiden populaatiossa 0,8–67,5 %:lla tutkimuspotilaista. Katsauksemme perusteella astmalla on merkittävä rooli bronkiektasiataudin liitännäissairautena, ja astma näyttäisi olevan itsenäinen syytekijä bronkiektasiataudin kehittymisessä pienessä osassa tautitapauksia. Lisäksi, vaikeaan astmaan näyttäisi liittyvän suurempi riski bronkiektasiataudin kehittymiselle. Tutkimuksemme vahvistaa käsitystä siitä, että astma etiologisena tekijänä tulee ottaa huomioon bronkiektasiataudin diagnoosia tehdessä, ja että bronkiektasiataudin etiologialla ja liitännäissairauksilla on keskeinen rooli lääkehoidon valinnassa. Lisätutkimuksia tarvitaan astman eri vaikeusasteiden vaikutuksesta bronkiektasiatautiriskiin, astma-bronkiektasiatauti-fenotyypin ennusteesta ja siitä, miten astman hoidolla voidaan vaikuttaa bronkiektasiataudin kehittymisen riskiin. (218 sanaa

Sputum Vitamin D Binding Protein (VDBP) GC1S/1S Genotype Predicts Airway Obstruction : A Prospective Study in Smokers with COPD

Introduction: The vitamin D binding protein (VDBP, also known as GC-globulin) and vitamin D deficiency have been associated with chronic obstructive pulmonary disease (COPD). rs7041 and rs4588 are two single nucleotide polymorphisms of the VDBP gene, including three common allelic variants (GC1S, GC1F and GC2). Previous studies primarily assessed the serum levels of vitamin D and VDBP in COPD. However, less is known regarding the impact of the local release of VDBP on COPD lung function. Thus, we examined the association of sputum and plasma VDBP with lung function at baseline and at four years, and examined potential genetic polymorphism interactions. Methods: The baseline levels of sputum VDBP, plasma VDBP and plasma 25-OH vitamin D, as well as the GC rs4588 and rs7041 genotypes, were assessed in a 4-year Finnish follow-up cohort (n = 233) of non-smokers, and smokers with and without COPD. The associations between the VDBP levels and the longitudinal decline of lung function were further analysed. Results: High frequencies of the haplotypes in rs7041/rs4588 were homozygous GC1S/1S (42.5%). Higher sputum VDBP levels in stage I and stage II COPD were observed only in carriers with GC1S/1S genotype when compared with non-smokers (p = 0.034 and p = 0.002, respectively). Genotype multivariate regression analysis indicated that the baseline sputum VDBP and FEV1/FVC ratio at baseline independently predicted FEV1% at follow-up. Discussion and Conclusion: The baseline sputum VDBP expression was elevated in smokers with COPD among individuals with the GC1S/1S genotype, and predicted follow-up airway obstruction. Our results suggest that the GC polymorphism should be considered when exploring the potential of VDBP as a biomarker for COPD.Peer reviewe