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Additional file 2. ELVIS participating sites

Bronchiectasis (BE) might be frequently present in COPD but masked by COPD symptoms. We studied the relationship of clinical signs of bronchiectasis to the presence and extent of its radiological signs in patients of different COPD severity. Visit 4 data (GOLD grades 1-4) of the COSYCONET cohort was used. Chest CT scans were evaluated for bronchiectasis in 6 lobes using a 3-point scale (0: absence, 1: ≤50%, 2: >50% BE-involvement for each lobe). 1176 patients were included (61%male, age 67.3y), among them 38 (3.2%) with reported physicians' diagnosis of bronchiectasis and 76 (6.5%) with alpha1-antitrypsin deficiency (AA1D). CT scans were obtained in 429 patients. Within this group, any signs of bronchiectasis were found in 46.6% of patients, whereby ≤50% BE occurred in 18.6% in ≤2 lobes, in 10.0% in 3-4 lobes, in 15.9% in 5-6 lobes; >50% bronchiectasis in at least 1 lobe was observed in 2.1%. Scores ≥4 correlated with an elevated ratio FRC/RV. The clinical diagnosis of bronchiectasis correlated with phlegm and cough and with radiological scores of at least 3, optimally ≥5. In COPD patients, clinical diagnosis and radiological signs of BE showed only weak correlations. Correlations became significant with increasing BE-severity implying radiological alterations in several lobes. This indicates the importance of reporting both presence and extent of bronchiectasis on CT. Further research is warranted to refine the criteria for CT scoring of bronchiectasis and to determine the relevance of radiologically but not clinically detectible bronchiectasis and their possible implications for therapy in COPD patients.

The COPD Assessment Test (CAT) is in widespread use for the evaluation of patients with chronic obstructive pulmonary disease (COPD). We assessed whether the CAT items carry additional information beyond the sum score regarding COPD characteristics including emphysema. Patients of GOLD grades 1 to 4 from the COPD cohort COSYCONET (German COPD and Systemic Consequences - Comorbidities Network) with complete CAT data were included (n = 2270), of whom 493 had chest CT evaluated for the presence of emphysema. Comorbidities and lung function were assessed following standardised procedures. Cross-sectional data analysis was based on multiple regression analysis of the single CAT items against a panel of comorbidities, lung function, or CT characteristics (qualitative score, 15th percentile of mean lung density), with age, BMI and gender as covariates. This was supported by exploratory factor analysis. Regarding the relationship to comorbidities and emphysema, there were marked differences between CAT items, especially items 1 and 2 versus 3 to 8. This grouping was basically confirmed by factor analysis. Items 4 and 5, and to a lower degree 1, 2 and 6, appeared to be informative regarding the presence of emphysema, whereas the total score was not or less informative. Regarding comorbidities, similar findings as for the total CAT score were obtained for the modified Medical Research Council scale (mMRC) which was also informative regarding emphysema. Our findings suggest that the usefulness of the CAT can be increased if evaluated on the basis of single items which may be indicating the presence of comorbidities and emphysema.

Rationale Alterations of acid-base metabolism are an important outcome predictor in acute exacerbations of COPD, whereas sufficient metabolic compensation and adequate renal function are associated with decreased mortality. In stable COPD there is, however, only limited information on the combined role of acid-base balance, blood gases, renal and respiratory function on exacerbation risk grading. Methods We used baseline data of the COPD cohort COSYCONET, applying linear and logistic regression analyses, the results of which were implemented into a comprehensive structural equation model. As most informative parameters it comprised the estimated glomerular filtration rate (eGFR), lung function defined via forced expiratory volume in 1 s (FEV1), intrathoracic gas volume (ITGV) and (diffusing capacity for carbon monoxide (DLCO), moreover arterial oxygen content (CaO2), partial pressure of oxygen (PaCO2), base exess (BE) and exacerbation risk according to GOLD criteria. All measures were adjusted for age, gender, body-mass index, the current smoking status and pack years. Results 1506 patients with stable COPD (GOLD grade 1–4; mean age 64.5 ± 8.1 y; mean FEV1 54 ± 18 %predicted, mean eGFR 82.3 ± 16.9 mL/min/1.73 m2) were included. BE was linked to eGFR, lung function and PaCO2 and played a role as indirect predictor of exacerbation risk via these measures; moreover, eGFR was directly linked to exacerbation risk. These associations remained significant after taking into account medication (diuretics, oral and inhaled corticosteroids), whereby corticosteroids had effects on exacerbation risk and lung function, diuretics on eGFR, BE and lung function. Conclusion Even in stable COPD acid-base metabolism plays a key integrative role in COPD risk assessment despite rather small deviations from normality. It partially mediates the effects of impairments in kidney function, which are also directly linked to exacerbation risk.

Table S4. Sociodemographic and clinical parameters of the study sample comparing patients who were not included due to missing follow-up assessment of QoL in comparison to the cohort included into the analyses. (DOCX 24 kb)

Figure S1. Mean difference in QoL scores for patients with one, two, three and more than three hospitalizations compared to patients who were not hospitalized during the follow-up. (EQ-5D VAS, EuroQol five-dimensional questionnaire, recorded as a visual analog scale; QoL, quality of life; SGRQ, St. Georgeâ s Respiratory Questionnaire; USCD-SOBQ, University of California San Diego Shortness of Breath Questionnaire; WHO-5, World Health Organization-5 Well-Being Index). (DOCX 110 kb)

Table S1. Quality of life scores at each follow-up visit and change in quality of life over the 3-year follow-up. Table S2. Quality of life scores at last follow-up and change in quality of life between baseline and last follow-up by change in lung function parameters within 1 year after baseline. Table S3. Quality of life scores at visit prior to hospitalisation and subsequent visit after hospitalisation in follow-up. (DOCX 40 kb)

Comparison of baseline characteristics of patients with and without available QoL data (total enrolled patients n = 737). (DOCX 15 kb)

Background Guidelines on the diagnosis and management of idiopathic pulmonary fibrosis (IPF), a rare manifestation of chronic progressive fibrosing interstitial pneumonia, have been updated by ATS/ERS/JRS/ALAT in 2011. In Europe, data are limited on the characteristics and management of such patients. Methods/design Investigating significant health trends (INSIGHTS)-IPF is a prospective observational longitudinal registry designed to describe the characteristics and management of newly diagnosed (incident) and prevalent patients with IPF on the long term. The registry uses a non-probability sampling approach to collect data on characteristics, therapeutic interventions, health-related quality of life and health economic parameters. At least 500 patients in ambulatory care will be included consecutively in about 30 centres. The study has been initiated in November 2012, and currently (December 2013) follows 344 patients. ClinTrials.gov identifier is NCT01695408. Discussion INSIGHTS-IPF documents one of the largest IPF cohorts in Europe. The registry is expected to provide much-needed data on the characteristics and management situation of patients with IPF in Germany. It will allow comparisons with other countries. Gap analyses based on current guidelines for management of these patients will be possible.