Treating connective tissue disease-associated interstitial lung disease - think outside the box: a perspective.

Connective tissue disease-associated interstitial lung disease is one of the most common subtypes of interstitial lung disease, which is a leading cause of morbidity and mortality in patients with these systemic autoimmune rheumatic diseases. A spectrum of disease trajectories exists within individual and across different connective tissue diseases. In individuals with connective tissue diseases who are at risk or at the early asymptomatic stage with interstitial lung changes, we have potential windows of opportunity for interventions to prevent the development of or progression to interstitial lung disease.

Sex-specific aspects in a population of patients undergoing evaluation for interstitial lung disease with transbronchial cryobiopsy.

There are well-documented differences in idiopathic pulmonary fibrosis (IPF) between sexes. The sex-specific prevalence of interstitial lung disease (ILD) subtypes in patients who require a full diagnostic work-up, including transbronchial cryobiopsy (TCB), after initial multidisciplinary discussion (MDD) is still unknown. Retrospective analysis of sex dispareties in patients with ILD who received an interdisciplinary indication for lung biopsy and underwent bronchoalveolar lavage, TCB and, if necessary, surgical lung biopsy at our ILD centre in Heidelberg between 11/17 and 12/21.

[Representation of respiratory medicine in medical curricula in Germany: an online survey among final-year medical students and chief physicians].

Students' experiences in medical school may have a significant influence on their later choice of medical specialty, but so far, there has been a lack of objective data on how the subject of pneumology is perceived at German medical schools and where there may be potential for improvement.From July to November 2022, we conducted an online survey among final-year students at German medical schools, as well as chief physicians in the field of pneumology. Students were invited to participate by e-mail via their faculties and the survey was further advertised by the Federal Representation of Medical Students in Germany (BVMD).

[Therapeutic Pathways in Sarcoidosis. A Position Paper of the German Society of Respiratory Medicine (DGP)].

The present recommendations on the therapy of sarcoidosis of the German Respiratory Society (DGP) was written in 2023 as a German-language supplement and update of the international guidelines of the European Respiratory Society (ERS) from 2021. It contains 5 PICO questions (Patients, Intervention, Comparison, Outcomes) agreed in the consensus process, which are explained in the background text of the four articles: Confirmation of diagnosis and monitoring of the disease under therapy, general therapy recommendations, therapy of cutaneous sarcoidosis, therapy of cardiac sarcoidosis.

Hospitalisation patterns in interstitial lung diseases: data from the EXCITING-ILD registry.

Background: Interstitial lung diseases (ILD) comprise a heterogeneous group of mainly chronic lung diseases with more than 200 entities and relevant differences in disease course and prognosis. Little data is available on hospitalisation patterns in ILD.

Methods: The EXCITING-ILD (Exploring Clinical and Epidemiological Characteristics of Interstitial Lung Diseases) registry was analysed for hospitalisations.

Quality of life in sarcoidosis.

Having sarcoidosis often has a major impact on quality of life of patients and their families. Improving quality of life is prioritized as most important treatment aim by many patients with sarcoidosis, but current evidence and treatment options are limited. In this narrative review, we describe the impact of sarcoidosis on various aspects of daily life, evaluate determinants of health-related quality of life (HRQoL), and provide an overview of the different patient-reported outcome measures to assess HRQoL in sarcoidosis.

Krebs von den Lungen-6 as a Potential Predictive Biomarker in Fibrosing Interstitial Lung Diseases.

Background: As fibrosing interstitial lung diseases (fILDs) are associated with high mortality, monitoring of disease activity under treatment is highly relevant. Krebs von den Lungen-6 (KL-6) is associated with the presence and severity of different fILDs, mainly in Asian patient populations.

Objectives: Our aim was to evaluate KL-6 as a predictive biomarker in fILDs in Caucasian patients.

Is the internet a sufficient source of information on sarcoidosis?

Introduction: Many patients use the internet as a source of health information. Sarcoidosis is a complex disease, and internet resources have not yet been analyzed for reliability and content on sarcoidosis.

Aims: Our study aimed to investigate the content and the quality of information on sarcoidosis provided by internet resources.

Severe tracheal tear - an alternative extracorporeal membrane oxygenation indication.

Introduction: Conservative management is usually preferred for iatrogenic tracheal injuries. Venovenous extracorporeal membrane oxygenation (V-V ECMO) is mostly used in acute refractory hypoxemia, airway lesions are an alternative indication.

Case Report: A 51-year-old female was transferred with a large tracheal tear after plastic tracheotomy.

How Informed Are German Patients with Pulmonary Sarcoidosis about Their Disease?

Background: Sarcoidosis is a multisystemic disease with a heterogenous course of disease. Comprehensive information about the complexity and treatment indications is essential for improving patient knowledge and adhering to therapy.

Objectives: The aim of our study was to investigate the level and resources of information in patients with sarcoidosis and to analyze differences in patient subgroups including age and gender.

Characterization of three new variants PiQ0, PiQ0 and PiQ0 in patients with severe alpha-1 antitrypsin deficiency.

Background: The clinical and molecular characteristics of three patients with previously unreported mutations associated with severe alpha-1 antitrypsin deficiency (AATD) are described. The pathophysiology of the chronic obstructive pulmonary disease (COPD) present in these patients was characterized through clinical, biochemical, and genetic examinations.

Case Presentations: Case 1: A 73-year-old male with bilateral centri-to panlobular emphysema and multiple increasing ventrobasal bullae and incomplete fissures, COPD (Global Initiative for Chronic Obstructive Lung Disease (GOLD) grade III B), progressive dyspnea on exertion (DOE), AAT level of 0.

[Interstitial lung diseases : Classification, differential diagnosis and treatment approaches in a heterogeneous group of chronic lung disorders].

Interstitial lung diseases (ILD) comprise a heterogeneous group of chronic lung disorders of different etiologies that can not only affect the interstitium but also the alveolar space and the bronchial system. According to the "Global Burden of Disease Study" there has been an increase in incidence over the last decades and it is expected that the number of ILD-associated deaths will double over the next 20 years. ILD are grouped into those of unknown cause, e.

The world of rare interstitial lung diseases.

The world of rare interstitial lung diseases (ILDs) is diverse and complex. Diagnosis and therapy usually pose challenges. This review describes a selection of rare and ultrarare ILDs including pulmonary alveolar proteinosis, pulmonary alveolar microlithiasis and pleuroparenchymal fibroelastosis.

[Treatment of lung fibrosis in systemic rheumatic diseases (new treatment)].

An interstitial lung disease represents a relevant organ manifestation in many systemic rheumatic diseases (connective tissue disease-interstitial lung disease, CTD-ILD). In 10% of the cases pulmonary fibrosis even results in an underlying systemic disease. The CTD-ILDs are frequently associated with a poor prognosis.