[Diagnostics of Interstitial Lung Diseases - Practical Instructions with a Focus on Rheumatologic Systemic Diseases].

Interstitial lung diseases (ILD) are etiologically heterogeneous with unknown and known causes like rheumatologic systemic diseases differing in their therapeutic and prognostic consequences. In consensus between pulmonologists, rheumatologists, radiologists, and pathologists, we developed practical instructions for ILD diagnosis in rheumatologic systemic diseases, in particular because ILD can present in early stages of rheumatic systemic diseases. ILD diagnosis is based on clinical assessment results including a detailed medical history, physical examination, focused laboratory tests, radiology with a high-resolution computed tomography, lung function, and histopathology also to differentiate it from cardiac and infection associated lung diseases.

Digital Tracking of Physical Activity, Heart Rate, and Inhalation Behavior in Patients With Pulmonary Arterial Hypertension Treated With Inhaled Iloprost: Observational Study (VENTASTEP).

Background: Pulmonary arterial hypertension restricts the ability of patients to perform routine physical activities. As part of pulmonary arterial hypertension treatment, inhaled iloprost can be administered via a nebulizer that tracks inhalation behavior. Pulmonary arterial hypertension treatment is guided by intermittent clinical measurements, such as 6-minute walk distance, assessed during regular physician visits.

Alemtuzumab-associated diffuse alveolar damage - a case report.

Background: Identifying causes of alemtuzumab induced respiratory symptoms in Multiple Sclerosis (MS) patients is crucial.

Case Presentation: We report a case of diffuse alveolar damage (DAD) in a patient with MS after the first course of alemtuzumab treatment. A 42-year-old female developed progressive non-productive cough and exertional dyspnea 2 months after alemtuzumab treatment.

Survival and quality of life after early discharge in low-risk pulmonary embolism.

Introduction: Early discharge of patients with acute low-risk pulmonary embolism requires validation by prospective trials with clinical and quality-of-life outcomes.

Methods: The multinational Home Treatment of Patients with Low-Risk Pulmonary Embolism with the Oral Factor Xa Inhibitor Rivaroxaban (HoT-PE) single-arm management trial investigated early discharge followed by ambulatory treatment with rivaroxaban. The study was stopped for efficacy after the positive results of the predefined interim analysis at 50% of the planned population.

Exhaled Volatile Organic Compounds during Inflammation Induced by TNF-α in Ventilated Rats.

Systemic inflammation alters the composition of exhaled breath, possibly helping clinicians diagnose conditions such as sepsis. We therefore evaluated changes in exhaled breath of rats given tumor necrosis factor-alpha (TNF-α). Thirty male Sprague-Dawley rats were randomly assigned to three groups (n = 10 each) with intravenous injections of normal saline (control), 200 µg·kg bodyweight TNF-α (TNF-α-200), or 600 µg·kg bodyweight TNF-α (TNF-α-600), and were observed for 24 h or until death.

Survival and course of lung function in the presence or absence of antifibrotic treatment in patients with idiopathic pulmonary fibrosis: long-term results of the INSIGHTS-IPF registry.

Objective: There is a paucity of observational data on antifibrotic therapy for idiopathic pulmonary fibrosis (IPF). We aimed to assess the course of disease of IPF patients with and without antifibrotic therapy under real-life conditions.

Methods: We analysed data from a non-interventional, prospective cohort study of consecutively enrolled IPF patients from 20 interstitial lung disease expert centres in Germany.

Leptomeningeal dissemination of low-grade neuroepithelial CNS tumors in adults: a 15-year experience.

Background: Leptomeningeal dissemination (LD) in adults is an exceedingly rare complication of low-grade neuroepithelial CNS tumors (LGNs). We aimed to determine relative incidence, clinical presentation, and predictors of outcome.

Methods: We searched the quality control database of the Section of Neuro-Oncology, Yale Cancer Center, for patients with LGN (WHO grade I/II) seen between 2002 and 2017.

Early discharge and home treatment of patients with low-risk pulmonary embolism with the oral factor Xa inhibitor rivaroxaban: an international multicentre single-arm clinical trial.

Aims: To investigate the efficacy and safety of early transition from hospital to ambulatory treatment in low-risk acute PE, using the oral factor Xa inhibitor rivaroxaban.

Methods And Results: We conducted a prospective multicentre single-arm investigator initiated and academically sponsored management trial in patients with acute low-risk PE (EudraCT Identifier 2013-001657-28). Eligibility criteria included absence of (i) haemodynamic instability, (ii) right ventricular dysfunction or intracardiac thrombi, and (iii) serious comorbidities.

Obstructive sleep apnea is associated with pulmonary artery thrombus load, disease severity, and survival in acute pulmonary embolism.

Introduction: Obstructive sleep apnea (OSA) may have prothrombotic effects.

Objective: To investigate the effect of OSA on disease severity, pulmonary artery thrombus load, and prognosis in patients with acute pulmonary embolism (PE).

Methods: In 101 PE patients, disease severity was determined by the simplified PE severity index (sPESI) score, pulmonary artery thrombus load was quantified by the pulmonary artery obstruction index (PAOI), and sleep-disordered breathing was evaluated by nocturnal polygraphy.

The clinical course of idiopathic pulmonary fibrosis and its association to quality of life over time: longitudinal data from the INSIGHTS-IPF registry.

Background: Quality of life (QoL) is profoundly impaired in patients with idiopathic pulmonary fibrosis (IPF). However, data is limited regarding the course of QoL. We therefore analysed longitudinal data from the German INSIGHTS-IPF registry.

Clinical presentation, management, and biomarkers of neurotoxicity after adoptive immunotherapy with CAR T cells.

Chimeric antigen receptor (CAR) T cells have emerged as a promising class of cell-based immunotherapy in refractory malignancies. Neurotoxicity represents a common and potentially life-threatening adverse effect of CAR T cells, and clinical experience is limited. Here, we describe the clinical presentation and management of 25 adult patients who presented with neurotoxic syndromes after CAR T-cell therapy at the Massachusetts General Hospital.

Risk stratification strategy and assessment of disease progression in patients with pulmonary arterial hypertension: Updated Recommendations from the Cologne Consensus Conference 2018.

In the summer of 2016, delegates from the German Respiratory Society, the German Society of Cardiology and the German Society of Pediatric Cardiology met in Cologne, Germany, to define consensus-based practice recommendations for the management of patients with pulmonary arterial hypertension (PAH). These recommendations were built on the 2015 European Pulmonary Hypertension guidelines and included new evidence, where available, and were last updated in the spring of 2018. This article focusses on the proposed risk stratification and assessment of disease progression in patients with pulmonary arterial hypertension (PAH), covering 3 parts: In part 1, methods and markers that are recommended to assess severity and progression of PAH are discussed and commented.

Successful long-term treatment of persistent pulmonary air leak in pneumocystis jirovecii pneumonia by unidirectional endobronchial valves.

Spontaneous pneumothorax is a rare complication of pneumocystis jirovecii pneumonia. We report a patient with pneumocystis jirovecii pneumonia and therapy-refractory, right-sided pneumothorax due to persistent air leak (PAL) despite prolonged chest tube placement and multiple pleurodesis attempts. Due to the patient's morbidity, we evaluated if the PAL can be sealed by unidirectional endobronchial valves (EBVs).

Is acute pulmonary embolism more severe in the presence of obstructive sleep apnea? Results from an observational cohort study.

Obstructive sleep apnea (OSA) might influence disease severity in acute pulmonary embolism (PE). 253 survivors of acute PE were evaluated for sleep-disordered breathing by portable monitoring and nocturnal polysomnography. PE patients with an apnea-hypopnoea index (AHI) ≥ 15/h were significantly older (p < 0.

The association of the N-terminal pro-brain-type natriuretic peptide response to exercise with disease severity in therapy-naive pulmonary arterial hypertension: a cohort study.

Background: While the N-terminal pro-brain-type natriuretic peptide (NT-proBNP) at rest is known to be associated with prognosis in pulmonary arterial hypertension (PAH), it is unclear if the NT-proBNP response to exercise (ΔNT-proBNP) can contribute to a better assessment of disease severity.

Methods: We investigated the association of NT-proBNP values at rest and during peak exercise with hemodynamics and cardiopulmonary exercise testing parameters in 63 therapy-naive PAH patients.

Results: The median NT-proBNP increases from 1414 at rest to 1500 pg/ml at peak exercise.

The association of N-terminal pro-brain-type natriuretic peptide with hemodynamics and functional capacity in therapy-naive precapillary pulmonary hypertension: results from a cohort study.

Background: N-terminal pro-brain-type natriuretic peptide (NT-proBNP) is currently used as a surrogate marker for disease severity in pulmonary hypertension (PH). However, NT-proBNP tends to have a high variability and may insufficiently correlate with hemodynamics and exercise capacity.

Methods: To investigate the association of NT-proBNP with hemodynamics and cardio-pulmonary exercise testing (CPET) in 84 therapy-naive patients with precapillary PH.

Health related quality of life in patients with idiopathic pulmonary fibrosis in clinical practice: insights-IPF registry.

Background: The INSIGHTS-IPF registry provides one of the largest data sets of clinical data and self-reported patient related outcomes including health related quality of life (QoL) on patients with idiopathic pulmonary fibrosis (IPF). We aimed to describe associations of various QoL instruments between each other and with patient characteristics at baseline.

Methods: Six hundred twenty-three IPF patients with available QoL data (St George's Respiratory Questionnaire SGRQ, UCSD Shortness-of-Breath Questionnaire SoB, EuroQol visual analogue scale and index EQ-5D, Well-being Index WHO-5) were analysed.

Obstructive sleep apnea might trigger acute pulmonary embolism: results from a cohort study.

Background: Obstructive sleep apnea (OSA) might be an independent risk factor for acute pulmonary embolism (APE).

Aim Of The Study: A prospective cohort study was conducted to investigate if APE is sleep-related in untreated OSA syndrome or not.

Methods: 206 APE patients were evaluated by portable monitoring and polysomnography.

[Risk stratification and follow-up assessment of patients with pulmonary arterial hypertension: Recommendations of the Cologne Consensus Conference 2016].

The 2015 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension are also valid for Germany. The guidelines contain detailed information about the diagnosis of pulmonary hypertension, and furthermore provide novel recommendations for risk stratification and follow-up assessments. However, the practical implementation of the European Guidelines in Germany requires the consideration of several country-specific issues and already existing novel data.

The prevalence of sleep-disordered breathing among survivors of acute pulmonary embolism.

Background: Sleep-disordered breathing (SDB) is associated with prothrombotic effects that could lead to venous thromboembolic diseases.

Objective: The objective of this study is to clarify the prevalence of SDB among survivors of pulmonary embolism (PE).

Methods: One hundred six consecutive PE patients were prospectively evaluated by portable monitoring (PM).

Management of patients with idiopathic pulmonary fibrosis in clinical practice: the INSIGHTS-IPF registry.

After introduction of the new international guidelines on idiopathic pulmonary fibrosis (IPF) in 2011, we investigated clinical management practices for patients with IPF according to physicians' diagnoses. A prospective, multicenter, noninterventional study with comprehensive quality measures including on-site source data verification was performed in Germany. 502 consecutive patients (171 newly diagnosed, 331 prevalent; mean±SD age 68.

Drug induced interstitial lung disease.

With an increasing number of therapeutic drugs, the list of drugs that is responsible for severe pulmonary disease also grows. Many drugs have been associated with pulmonary complications of various types, including interstitial inflammation and fibrosis, bronchospasm, pulmonary edema, and pleural effusions. Drug-induced interstitial lung disease (DILD) can be caused by chemotherapeutic agents, antibiotics, antiarrhythmic drugs, and immunosuppressive agents.