'Diagnosis and Treatment of Hypersensitivity Pneumonitis' S2k Guideline of the German Respiratory Society and the German Society for Allergology and Clinical Immunology.

German recommendations for the diagnosis of hypersensitivity pneumonitis (HP), also known as extrinsic allergic alveolitis (EAA), were last published in 2007 [1]. The current S2k Guideline for the Diagnosis and Treatment of Hypersensitivity Pneumonitis (HP) replaces these diagnostic recommendations. They were supplemented by the aspect of chronic, and in particular of the chronic fibrotic phenotype of HP, and also, as first HP guideline, include treatment recommendations.

Continued Treatment with Nintedanib in Patients with Progressive Pulmonary Fibrosis: Data from INBUILD-ON.

Purpose: In the INBUILD trial in patients with progressive pulmonary fibrosis (PPF), nintedanib slowed the decline in forced vital capacity (FVC) versus placebo, with a safety profile characterised mainly by gastrointestinal events. INBUILD-ON, the open-label extension of INBUILD, assessed the safety of nintedanib during longer-term treatment. Data on FVC were collected.

Online survey on existing sarcoidosis registries and biobanks: an ERN lung initiative.

The present survey, promoted by the European Reference Network on rare respiratory diseases (ERN-Lung, Core Networks Sarcoidosis and ILD), aims to assess the existing sarcoidosis registries and biobanks across Europe and to compare the various types of biospecimen collected, the different procedures performed, and the sample storage conditions applied. This survey was initiated by the European Reference Network on rare respiratory diseases (ERN-Lung) Core Network "Sarcoidosis" in April 2023. The survey was launched by ERN-Lung Core Network "Sarcoidosis" in August 2023 and remained active until end of February 2024.

Childhood interstitial lung disease survivors in adulthood: a European collaborative study.

Background: Interstitial lung disease (ILD) is rarer in children (chILD) than adults, but with increasing diagnostic awareness, more cases are being discovered. chILD prognosis is often poor, but increasing numbers are now surviving into adulthood.

Aim: To characterize chILD-survivors and identify their impact on adult-ILD centers.

Fully automatic quantification of pulmonary fat attenuation volume by CT: an exploratory pilot study.

Background: Non-malignant chronic diseases remain a major public health concern. Given the alterations in lipid metabolism and deposition in the lung and its association with fibrotic interstitial lung disease (fILD) and chronic obstructive pulmonary disease (COPD), this study aimed to detect those alterations using computed tomography (CT)-based analysis of pulmonary fat attenuation volume (CTpfav).

Methods: This observational retrospective single-center study involved 716 chest CT scans from three subcohorts: control (n = 279), COPD (n = 283), and fILD (n = 154).

Serum soluble isoform of receptor for advanced glycation end product is a predictive biomarker for acute exacerbation of idiopathic pulmonary fibrosis: a German and Japanese cohort study.

Background: The receptor for advanced glycation end product (RAGE) is a transmembrane receptor accelerating a pro-inflammatory signal. RAGE signalling is promoted by decreased soluble isoform of RAGE (sRAGE), which is a decoy receptor for RAGE ligands, and RAGE SNP rs2070600 minor allele. In Caucasian and Japanese cohorts, low circulatory sRAGE levels and presence of the minor allele are associated with poor survival of idiopathic pulmonary fibrosis (IPF) and increased disease susceptibility to interstitial lung disease, respectively.

CT-based body composition analysis and pulmonary fat attenuation volume as biomarkers to predict overall survival in patients with non-specific interstitial pneumonia.

Background: Non-specific interstitial pneumonia (NSIP) is an interstitial lung disease that can result in end-stage fibrosis. We investigated the influence of body composition and pulmonary fat attenuation volume (CTpfav) on overall survival (OS) in NSIP patients.

Methods: In this retrospective single-center study, 71 NSIP patients with a median age of 65 years (interquartile range 21.

[Diagnosis and Treatment of Hypersensitivity Pneumonitis - S2k Guideline of the German Respiratory Society and the German Society for Allergology and Clinical Immunology].

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.

Automated 3D-Body Composition Analysis as a Predictor of Survival in Patients With Idiopathic Pulmonary Fibrosis.

Purpose: Idiopathic pulmonary fibrosis (IPF) is the most common interstitial lung disease, with a median survival time of 2 to 5 years. The focus of this study is to establish a novel imaging biomarker.

Materials And Methods: In this study, 79 patients (19% female) with a median age of 70 years were studied retrospectively.

Kidney manifestations of sarcoidosis.

Renal involvement is a clinically relevant organ manifestation of sarcoidosis, leading to increased morbidity and complications. Although the exact incidence remains unknown, renal disease is likely to occur in up to one third of all sarcoidosis patients. Every patient with newly diagnosed sarcoidosis should receive a renal work-up and screening for disrupted calcium metabolism.

Phase 2, Double-Blind, Placebo-controlled Trial of a c-Jun N-Terminal Kinase Inhibitor in Idiopathic Pulmonary Fibrosis.

Idiopathic pulmonary fibrosis is a fatal and progressive disease with limited treatment options. We sought to assess the efficacy and safety of CC-90001, an oral inhibitor of c-Jun N-terminal kinase 1, in patients with idiopathic pulmonary fibrosis. In a Phase 2, randomized (1:1:1), double-blind, placebo-controlled study (ClinicalTrials.

[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.

Serum KL-6 as a Candidate Predictor of Outcome in Patients with SARS-CoV-2 Pneumonia.

Severe acute respiratory syndrome coronavirus type 2 (SARS-CoV-2)-infection is associated with an extremely variable disease course. When interstitial pneumonia (IP) occurs, it can lead to acute respiratory distress syndrome and death. Serum Krebs von den Lungen-6 (KL-6) is an established marker of IP, but its role as a marker of SARS-CoV-2 pneumonia is debated.

Current and Future Treatment Landscape for Idiopathic Pulmonary Fibrosis.

Idiopathic pulmonary fibrosis (IPF) remains a disease with poor survival. The pathogenesis is complex and encompasses multiple molecular pathways. The first-generation antifibrotics pirfenidone and nintedanib, approved more than 10 years ago, have been shown to reduce the rate of progression, increase the length of life for patients with IPF, and work for other fibrotic lung diseases.

Gastrointestinal pirfenidone adverse events in idiopathic pulmonary fibrosis depending on diet: the MADIET clinical trial.

https://bit.ly/3LuzAUJ

Krebs von den Lungen 6 (KL-6) is a novel diagnostic and prognostic biomarker in pleural mesothelioma.

Objectives: Pleural mesothelioma (PM) is a rare disease with dismal outcome. Systemic treatment options include chemotherapy and immunotherapy, but biomarkers for treatment personalization are missing. The only FDA-approved diagnostic biomarker is the soluble mesothelin-related protein (SMRP).

Impact of Systemic Sclerosis-Associated Interstitial Lung Disease With and Without Pulmonary Hypertension on Survival: A Large Cohort Study of the German Network for Systemic Sclerosis.

Background: Pulmonary involvement is the leading cause of death in systemic sclerosis (SSc) and may manifest as interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), or in combination of both (ILD with pulmonary hypertension [ILD-PH]). The aim of this analysis was to determine prevalence, clinical characteristics, and survival of these different forms within the registry of the German Network for Systemic Sclerosis.

Research Question: Does SSc-associated ILD-PH or ILD without PH affect survival differently, and are there any risk factors that have an additional impact?

Study Design And Methods: Clinical data of 5,831 patients with SSc were collected in the German Network for Systemic Sclerosis registry.

Pulmonary alveolar proteinosis - current and future therapeutical strategies.

Purpose Of Review: We discuss the most recent advances in the treatment of pulmonary alveolar proteinosis (PAP), an ultra-rare syndrome.

Recent Findings: Whole lung lavage (WLL) remains the gold standard of treatment for PAP syndrome. For the autoimmune form, recent trials with recombinant human granulocyte-macrophage colony-stimulating factor (GM-CSF) confirmed the efficacy in up to 70% of cases, especially under continuous administration.