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Background:  Accumulating evidence on the role of blood eosinophils as a biomarker prompted the Global Initiative for Chronic Obstructive Lung Disease (GOLD) committee to refine the existing treatment algorithm by incorporating eosinophil counts into treatment recommendations. However, there is a lack of data on when, why and how frequently such blood tests and other measures are being performed by German private respiratory specialists., Methods:  A questionnaire evaluating doctors' opinions on the use of diagnostic measures at initial diagnosis and during follow-up, including blood eosinophil count in patients with COPD, was completed by 27 respiratory specialists. Medical records from the past 12 months of 251 patients treated by the same physicians were reviewed retrospectively to investigate the use of these measures., Results:  Body plethysmography (100 % of doctors) and chest X-ray (96.3 %) were the most commonly used measures according to the doctor's questionnaire; other measures were COPD assessment test (CAT; 85.2 %) and blood eosinophil count (81.5 %). The evaluation of patients' medical records revealed that body plethysmography was performed in 72.7 %, the CAT in 61.8 % and chest X-ray in 40.6 % of patients. Blood eosinophil count was measured in 7.2 %., Conclusions:  In line with the GOLD recommendations, these results confirm that lung function, imaging and patient-reported outcome questionnaires play a crucial role in managing COPD. Our analyses reveal that measurement of the blood eosinophil count gained importance due to physicians' increased awareness of these cells as a useful biomarker. However, this test seems to be performed mainly for initial diagnosis and not on a regular basis., Competing Interests: Timm Greulich reports personal fees for advisory board meetings and lectures from AstraZeneca, Berlin-Chemie, Boehringer-Ingelheim, Chiesi, CSL-Behring, GlaxoSmithKline, Novartis and grants from Grifols and the German centre for Lung Research (DZL), Marburg, Germany, outside the submitted work.Volker Töpfer has no conflict of interest to declare.Michael Hennig, Christina C. Orehounig, Klaus Ams and Cordula D. Mohrlang are employed at GlaxoSmithKline GmbH & Co, Munich, the sponsor.Peter H. Killian was a full-time employee of GSK until 31st of March 2020, which included conduction of the study and the preparation of the manuscript.Peter Kardos reports personal fees for advisory board meetings and lectures from Astra Zeneca, Berlin-Chemie, Bionorica, Chiesi, Engelhardt, GlaxoSmithKline GmbH & Co, Klosterfrau Health Care Club, Novartis, MSD, Schwabe Pharma Italia, conference participation was reimbursed by AstraZeneca and he is an investigator in a phase 3 MSD study, outside the submitted work., (The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).)

The present addendum of the guideline for the diagnosis and treatment of asthma (2017) complements new insights into the diagnosis and management of asthma as well as for the newly approved drugs for the treatment of asthma. Current, evidence-based recommendations on diagnostic and therapeutic approaches are presented for children and adolescents as well as for adults with asthma., Competing Interests: Eine Übersicht der Interessenkonflikte findet sich im Internet unter http://www.awmf.org; AWMF-Registernummer 020-009., (Thieme. All rights reserved.)

Competing Interests: T. Greulich erhielt Kongressunterstützung durch AstraZenca, Boehringer-Ingelheim, Chiesi, CSL-Behring, Grifols, GSK, Mundopharma, Novartis sowie Forschungsunterstützung durch CSL-Behring und Grifols. C. Clarenbach erhielt Honorare für Beratertätigkeit von Roche, Novartis, Boehringer, GSK, Astra Zeneca, Sanofi, Vifor und Mundipharma. D. Skowasch erhielt Honorare für Beratertätigkeiten/Vorträge und Kongressteilnahmeunterstützung von Grifols. R. Bals berichtet über persönliche Zahlung (advisory boards) von AstraZeneca, Boehringer Ingelheim, GlaxoSmithKline, Grifols, Novartis und CSL Behring. Forschung von Prof. Bals wird unterstützt von: Bundesministerium für Bildung und Forschungskompetenznetzwerk (BMBF) Asthma und COPD (ASCONET), Sander Stiftung, Schwiete Stiftung, Boehringer Ingelheim, Novartis und Mukoviszidose e. V. A. R. Koczulla: Vorträge, Advisory Board für CSL und Grifols, Forschungsunterstützung von Grifols und CSL.S. Fähndrich, W. Gleiber, H. Hautmann, R. Heine, M. Idzko, K. Schmidt-Scherzer und R. Wiewrodt geben an, dass kein Interessenkonflikt besteht.

The present guideline is a new version and an update of the guideline for the diagnosis and treatment of asthma, which replaces the previous version for german speaking countries from the year 2006. The wealth of new data on the pathophysiology and the phenotypes of asthma, and the expanded spectrum of diagnostic and therapeutic options necessitated a new version and an update. This guideline presents the current, evidence-based recommendations for the diagnosis and treatment of asthma, for children and adolescents as well as for adults with asthma., Competing Interests: Interessenkonflikt: Eine Übersicht der Interessenkonflikte findet sich im Internet unter http://www.awmf.org; AWMF-Registernummer 020-009., (© Georg Thieme Verlag KG Stuttgart · New York.)

Competing Interests: Disclosure The authors report no conflicts of interest in this work.

Competing Interests: Disclosure The authors report no conflicts of interest in this work.

Background and objectives  Alpha-2 Macroglobulin (A2M) is a plasma protein with proteolytic effects on many proteases. In patients with an inborn alpha-1 antitrypsin deficiency (AATD) the homeostasis between proteases and antiproteases is disturbed. The aim of this study was to compare the levels of AAT and A2 M in patients and controls. We hypothesized that in patients with AATD A2 M levels are elevated. Methods  Patients with AATD (polymorphism Pi*ZZ, Pi*SZ, Pi*MZ and rare gene variants) as well as healthy volunteers (Pi*MM) were tested for A2 M and AAT levels. The concentration of the proteins was measured by nephelometry. The polymorphisms Pi*Z and Pi*S were detected by polymerase chain reaction (PCR), the rare genetic variants were identified by sequencing. Results  In our study, a total of 291 individuals were included. It could be shown that a significant increase in A2 M levels in the serum could be observed in the presence of a gene polymorphism (Pi*ZZ) and an alpha-1 antitrypsin serum level < 50 mg/dl compared to the healthy volunteers. Conclusions  In this study, an inverse correlation between the serum levels of AAT and A2 M was found in the presence of a gene polymorphism (Pi*ZZ). Further studies are necessary to elucidate the clinical significance of increased A2 M serum levels in patients with severe AAT deficiency Pi*ZZ and rare gene variants whose AAT serum level is < 50 mg/dl., Competing Interests: Interessenkonflikt: V. Kotke, S. Wiedmann, C. Nell, C. Vogelmeier, R. Bals, T. Greulich und A. Klemmer geben an, dass kein Interessenkonflikt besteht.Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Tieren., (© Georg Thieme Verlag KG Stuttgart · New York.)

Augmentation therapy in Alpha-1 Antitrypsin Deficiency aims to reduce the progression of lung emphysema, to reduce exacerbation frequency, and to improve quality of life. This expert statement briefly summarizes the most important treatment studies performed in patients with Alpha-1 Antitrypsin Deficiency and severe lung emphysema. Indications and contraindications for long-term intravenous augmentation therapy with human Alpha-1 Antitrypsin are derived from the available study results. Safety issues and the controversies of this topic are discussed in detail., (© Georg Thieme Verlag KG Stuttgart · New York.)

Background: Chronic diseases of the respiratory organs have, besides restrictions of lung function, also physical, mental and social consequences. The chronic disease impacts negatively not only the patient's own quality of life but also that of his/her partner and/or relative. As treatment modalities, besides drug therapy, above all outpatient rehabilitation measures have proved to be effective. In this pilot study we sought answers to three questions: (i) How is the quality of life of the patient's partner and/or relative influenced by the disease? How does the partner/relative assess the effect of the disease on the chronically ill patient? How does the partner/relative assess the effect of lung sports on the chronically ill patient?, Methods: A specially conceived questionnaire was given to the partners/relatives of 25 patients with chronic pulmonary diseases. The patients, 23 with chronic obstructive pulmonary disease and 2 with pulmonary fibrosis, have been participating in lung sports once a week for more than three years. The average age of the patients was 67 years., Results: The patients' illness also negatively influenced the quality of life of the partner to a considerable extent. The impact of the patients' pulmonary disease on the healthy partner was assessed as being rather moderate. Restrictions of social life were essentially influenced by the amount of assistance required by the pulmonary patient. The therapeutic effects of lung sports on the well-being of the patient were aways considered to be positive by the respective partner/relatives., Conclusion: A chronic pulmonary illness also has a negative influence on the partner/relative's quality of life. The necessity for extensive assistance in daily life is accompanied by a considerable negative impact on the partner's quality of life. According to the opinion of the partner/relative, lung sports have positive effects in all of the chronically ill patient's fields of life., (© Georg Thieme Verlag KG Stuttgart · New York.)

A 46-year-old patient was frequently seen with a medically treated Anti-Jo-1 syndrome. The patient had already been treated with azathioprine and oral corticosteroids on account of decreasing lung function, dyspnoea, fatigue, and beginning signs of myositis. Although high doses of steroids and azathioprine were administered, the muscleskeletal syndromes increased steadily. The patient used to be an active long-distance runner (20 km), but now was unable to perform that kind of physical exercise. It was decided to start a treatment with the GalileoTM training device for active muscle training of the lower extremities. Before and after three months of training the following assessment was performed: measurement of health-related quality of life (St. Georges respiratory questionnaire, SGRQ), ultrasound measurement of the cross-sectional area of the quadriceps muscle, 6 minute walk test (6 MWT), lung function testing, and assessment of serum markers of inflammation (TNF-alpha, interleukin-8, CRP, CK, myoglobin). After only two months, training with the GalileoTM five times a week has improved the patient's conditions dramatically. The training will be continued., (© Georg Thieme Verlag KG Stuttgart · New York.)

Background: alpha-1-Antitrypsin deficiency (AATD) is a rare condition with a prevalence of about 1/4000 individuals in Germany. The clinical manifestations are the early development of pulmonary emphysema or of liver cirrhosis. The low prevalence of AATD suggested the establishment of a registry with the aim to learn more details about the natural history and the quality of care for these patients., Methods, Patients, Results: Since 2004 the German registry for individuals with AATD is maintained in Marburg. Up to May 2008, 548 individuals were registered, 455 with the genotype PiZZ and 46 with PiSZ. The age range of the registered individuals extends from 2 to 82 years (mean 55, SD 20; men 54 [SD 19] years, women 55 [SD 21] years). Most patients are affected by chronic obstructive pulmonary disease (COPD, distribution to the GOLD stages: GOLD I = 16 %, GOLD II = 30 %, GOLD III = 33 %, GOLD IV = 21 %). Currently 109 subjects are under substitution therapy. The time period between the first symptom and the establishment of the correct diagnosis was on average 5.6 years., Conclusions: The data of the registry allow a detailed characterisation of the natural course of the disease and the levels of the patient care.