Pharmacological Treatment of Idiopathic Pulmonary Fibrosis (Update) and Progressive Pulmonary Fibroses S2k Guideline of the German Respiratory Society.

Interstitial lung diseases (ILD) are characterized by a variable degree of inflammatory and fibrotic changes within the alveolar space and distal airways (bronchioles). An inverse correlation exists between the extent of fibrosis and the possibility that an ILD is reversible. While the acute (inflammatory) type of extrinsic allergic alveolitis may resolve without sequelae (restitutio ad integrum), IPF is the prototypic fibrotic ILD with a progressive course, leading to an irreversible and progressive fibrosis of the lung parenchyma. This guideline provides guidance on differnential pharmacological treatment approaches to different types of fibrotic interstitial lung diseases.

[Therapeutic Pathways in Sarcoidosis. A Position Paper of the German Society of Respiratory Medicine (DGP)]. / Therapie der Sarkoidose. Ein Positionspapier der Deutschen Gesellschaft für Pneumologie und Beatmungsmedizin (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.

[Diagnosis and Treatment of Hypersensitivity Pneumonitis - S2k Guideline of the German Respiratory Society and the German Society for Allergology and Clinical Immunology]. / Diagnostik und Therapie der exogen-allergischen Alveolitis.

Hypersensitivity pneumonitis (HP) is an immune-mediated interstitial lung disease (ILD) in sensitized individuals caused by a large variety of inhaled antigens. The clinical form of acute HP is often misdiagnosed, while the chronic form, especially the chronic fibrotic HP, is difficult to differentiate from other fibrotic ILDs. The present guideline for the diagnosis and treatment of HP replaces the former German recommendations for the diagnosis of HP from 2007 and is amended explicitly by the issue of the chronic fibrotic form, as well as by treatment recommendations for the first time. The evidence was discussed by a multidisciplinary committee of experts. Then, recommendations were formulated for twelve questions on important issues of diagnosis and treatment strategies. Recently published national and international guidelines for ILDs and HP were considered. Detailed background information on HP is useful for a deeper insight into HP and the handling of the guideline.

[CF Lung Disease - a German S3 Guideline: Pseudomonas aeruginosa]. / S3-Leitlinie: Lungenerkrankung bei Mukoviszidose ­ Pseudomonas aeruginosa.

Cystic Fibrosis (CF) is the most common autosomal recessive genetic multisystemic disease. In Germany, it affects at least 8000 people. The disease is caused by mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene leading to dysfunction of CFTR, a transmembrane chloride channel. This defect causes insufficient hydration of the airway epithelial lining fluid which leads to reduction of the mucociliary clearance.Even if highly effective, CFTR modulator therapy has been available for some years and people with CF are getting much older than before, recurrent and chronic infections of the airways as well as pulmonary exacerbations still occur. In adult CF life, Pseudomonas aeruginosa (PA) is the most relevant pathogen in colonisation and chronic infection of the lung, leading to further loss of lung function. There are many possibilities to treat PA-infection.This is a S3-clinical guideline which implements a definition for chronic PA-infection and demonstrates evidence-based diagnostic methods and medical treatment in order to give guidance for individual treatment options.

S2K Guideline for Diagnosis of Idiopathic Pulmonary Fibrosis.

Idiopathic pulmonary fibrosis (IPF) is a severe and often fatal disease. Diagnosis of IPF requires considerable expertise and experience. Since the publication of the international IPF guideline in the year 2011 and the update 2018 several studies and technical advances have occurred, which made a new assessment of the diagnostic process mandatory. The goal of this guideline is to foster early, confident, and effective diagnosis of IPF. The guideline focusses on the typical clinical context of an IPF patient and provides tools to exclude known causes of interstitial lung disease including standardized questionnaires, serologic testing, and cellular analysis of bronchoalveolar lavage. High-resolution computed tomography remains crucial in the diagnostic workup. If it is necessary to obtain specimens for histology, transbronchial lung cryobiopsy is the primary approach, while surgical lung biopsy is reserved for patients who are fit for it and in whom a bronchoscopic diagnosis did not provide the information needed. After all, IPF is a diagnosis of exclusion and multidisciplinary discussion remains the golden standard of diagnosis.

German Guideline for Idiopathic Pulmonary Fibrosis - Update on Pharmacological Therapies 2017.

Idiopathic pulmonary fibrosis (IPF) is a severe and often fatal disease with a median survival of 2 - 4 years after diagnosis. Since the publication of the German IPF guideline in 2013 new treatment trials have been published, necessitating an update of the pharmacological therapy of IPF. Different from the previous guideline, the GRADE system was discarded and replaced by the Oxford evidence classification system which allows a more differentiated judgement. The following pharmacological therapies were rated not suitable for the treatment of IPF patients (recommendation A; evidence 1-b): triple therapy with prednisolone, azathioprine and acetyl-cysteine; imatinib; ambrisentan; bosentan; macitentan. A less clear but still negative recommendation (B, 1-b) was attributed to the treatment of IPF with the phosphodiesterase-5-inhibitor sildenafil and acetyl-cysteine monotherapy. In contrast to the international guideline antacid therapy as a general treatment for IPF was rated negative, based on conflicting results of recent analyses (recommendation C; evidence 4). An unanimous positive recommendation was granted for the antifibrotic drugs nintedanib and pirfenidone for the treatment of IPF (A, 1-a). For some open questions in the management of IPF patients for which firm evidence is lacking the guideline also offers recommendations based on expert consensus.

Sure, or unsure? Measuring students' confidence and the potential impact on patient safety in multiple-choice questions.

BACKGROUND: Multiple-choice questions (MCQs) provide useful information about correct and incorrect answers, but they do not offer information about students' confidence. METHODS: Ninety and another 81 medical students participated each in a curricular neurology multiple-choice exam and indicated their confidence for every single MCQ. Each MCQ had a defined level of potential clinical impact on patient safety (uncritical, risky, harmful). Our first objective was to detect informed (IF), guessed (GU), misinformed (MI), and uninformed (UI) answers. Further, we evaluated whether there were significant differences for confidence at correct and incorrect answers. Then, we explored if clinical impact had a significant influence on students' confidence. RESULTS: There were 1818 IF, 635 GU, 71 MI, and 176 UI answers in exam I and 1453 IF, 613 GU, 92 MI, and 191 UI answers in exam II. Students' confidence was significantly higher for correct than for incorrect answers at both exams (p < 0.001). For exam I, students' confidence was significantly higher for incorrect harmful than for incorrect risky classified MCQs (p = 0.01). At exam II, students' confidence was significantly higher for incorrect harmful than for incorrect benign (p < 0.01) and significantly higher for correct benign than for correct harmful categorized MCQs (p = 0.01). CONCLUSIONS: We were pleased to see that there were more informed than guessed, more uninformed than misinformed answers and higher students' confidence for correct than for incorrect answers. Our expectation that students state higher confidence in correct and harmful and lower confidence in incorrect and harmful MCQs could not be confirmed.