A New Tool to Assess Quality of Life in Patients with Idiopathic Pulmonary Fibrosis or Non-specific Interstitial Pneumonia.

Background:  Quality of life (QoL) is significantly impaired in patients with pulmonary fibrosis, however reliable tools to assess QoL issues specific for this group of patients are still missing. We thus aimed to develop a new questionnaire called "Quality of life in patients with idiopathic pulmonary fibrosis" (QPF) to measure QoL in patients with fibrotic idiopathic interstitial pneumonias (IIP).

Methods:  An item pool was created on the basis of a German expert group with support of patients suffering from pulmonary fibrosis.

[Linguistic Validation of the "German Lung Fibrosis Health Related Quality of Life Questionnaire"].

Unlabelled: Health status and quality of life are impaired in patients with idiopathic pulmonary fibrosis (IPF) and idiopathic non-specific interstitial fibrosis (iNSIP). In Germany exists only the K-BILD questionnaire for patients with ILD 1 in a professional translation by Kreuter et al. 2 This questionnaire focuses on the main problems in patients with progressive lung fibrosis in a limited manner.

A Comparison of Existing Questionnaires for Identifying the Causes of Interstitial and Rare Lung Diseases.

Background: A thorough diagnostic process is essential with regard to prognosis and treatment of the more than 200 different types of interstitial lung diseases (ILD). Key to this complex process is a comprehensive medical history. For this, a template is recommended and questionnaires are increasingly used.

[DGP Interstitial Lung Disease Patient Questionnaire].

Background:  Interstitial lung diseases (ILD) encompass different heterogeneous, mainly chronic diseases of the pulmonary interstitium and/or alveoli with known and unknown reasons. The diagnostic of ILD is challenging and should be performed interdisciplinary. The medical history is of major importance and therefore, in German-speaking countries the Frankfurter Bogen (published in 1985) was utilised to scrutinise the medical history of the patient.

Physical Activity and Fatigue in Patients with Sarcoidosis.

Background: Little is known about physical activity in daily life among patients with sarcoidosis. Fatigue is a frequent and disabling symptom that might negatively affect physical activity levels.

Methods: In patients with sarcoidosis, we measured physical activity (steps per day) by accelerometry (SenseWear Armband) for 1 week.

Prognosis and longitudinal changes of physical activity in idiopathic pulmonary fibrosis.

Background: Physical activity (PA) is associated with disease severity in idiopathic pulmonary fibrosis (IPF), but longitudinal studies evaluating its prognostic value and changes over time are lacking.

Methods: We measured PA (steps per day, SPD) in a cohort of 46 IPF-patients (mean age, 67 years; mean FVC, 76.1%pred.

[Marcello Malpighi (1628-1694) and the Terms Miliary and Tubercle].

Today Miliary Tuberculosis in Central Europe is a rare disease, quite often with resulting diagnostic uncertainty. The terms "miliary" and "tubercle" are outlined with their up to now accepted historical roots. An analysis of Marcello Malpighi's quite unknown post-mortem reports by the Italian author L.

Clinical Correlates of Reduced Physical Activity in Idiopathic Pulmonary Fibrosis.

Background: Little is known about the consequences of idiopathic pulmonary fibrosis (IPF) for physical activity (PA).

Objectives: We aimed to investigate levels of PA in IPF and to study associations of PA with lung function, exercise capacity, symptoms, and quality of life.

Methods: In stable patients with IPF we measured PA (steps per day, SPD; physical activity level, PAL; minutes of moderate activity, MMA) by accelerometry (SenseWear Armband) for 1 week.

Diagnostic Gain from Surgical Biopsy for Interstitial Lung Disease - When is it Worth the Risk?

Background: History, clinical presentation, lung function testing, radiographs including HRCT and nonsurgical biopsy techniques in most cases provide sufficient information for classification of interstitial lung disease (ILD). However, in a small percentage it is not possible to establish the diagnosis so that lung biopsy may be required. We analyzed under which circumstances a reduction of invasive procedures is reasonable.

Reduced physical activity in lymphangioleiomyomatosis compared with COPD and healthy controls: disease-specific impact and clinical correlates.

Rationale: Sporadic lymphangioleiomyomatosis (LAM) is an orphan lung disease for which daily physical activity has not been studied so far and it is unclear whether a disease-specific impact beyond airflow limitation exists. Clinical correlates indicating reduced physical activity in addition to established parameters like airflow limitation and hypoxaemia are largely undetermined.

Method: We measured physical activity (steps per day, SPD; physical activity level, PAL; minutes of moderate activity, MMA) in 34 women with LAM, 32 FEV1-matched female patients with COPD and 15 age-matched healthy women for 1 week using an accelerometer.

Screening for Helicobacter pylori in Idiopathic Pulmonary Fibrosis Lung Biopsies.

Background: Increasing evidence suggests a role of gastro-oesophageal reflux (GER) in idiopathic pulmonary fibrosis (IPF) pathogenesis. Recently, an association between serum Helicobacter pylori (HP) antibody positivity and more severe disease was described, but HP has not been directly analysed in lung tissue so far.

Objective: To investigate the presence of HP in the lung tissue of IPF patients.

[Position Paper: Significance of the Forced Vital Capacity in Idiopathic Pulmonary Fibrosis].

Spirometry is a highly standardized method which allows to measure the forced vital capacity (FVC) with high precision and reproducibility. In patients with IPF FVC is directly linked to the disease process which is characterized by scaring of alveoli and shrinkage of the lungs. Consequently, there is ample evidence form clinical studies that the decline of FVC over time is consistently associated with mortality in IPF.

Sarcoidosis with involvement of the paranasal sinuses - a retrospective analysis of 12 biopsy-proven cases.

Background: Extrapulmonary involvement by sarcoidosis is observed in about 30-40% of patients with sarcoidosis. Little is known about the frequency and clinical characteristics of sinonasal sarcoidosis.

Methods: We retrospectively analyzed 12 cases of biopsy-proven sinonasal sarcoidosis.

[Pulmonary sarcoidosis: current diagnosis and treatment].

Sarcoidosis is a multisystemic granulomatous disorder of unknown origin, which can involve multiple organs. However the lymph node and lung manifestations dominate. Most frequent symptoms are cough, fatigue, dyspnea and exercise limitations.

[Marcello Malpighi (1628 - 1694) and the terms miliary and tubercle. A completion of hitherto existing historical terminology].

Today Miliary Tuberculosis in Central Europe is a rare disease, quite often with resulting diagnostic uncertainty. The terms "miliary" and "tubercle" are outlined with their up to now accepted historical roots. An analysis of Marcello Malpighi's quite unknown post-mortem reports by the Italian author L.

Susceptibility to tuberculosis is associated with TLR1 polymorphisms resulting in a lack of TLR1 cell surface expression.

Human TLR1 plays an important role in host defense against Mycobacterium tuberculosis. Our aim was to analyze the association of the loss of TLR1 surface expression and TLR1 SNPs with susceptibility to TB. TLR1neg and TLR1pos cells from healthy individuals were identified by flow cytometry and compared by sequencing.

[Non-specific interstitial pneumonia (NSIP)].

Non-specific interstitial pneumonia (NSIP) belongs to the group of idiopathic interstitial pneumonias (IIP). However, NSIP can also be found in several other diseases. For example, the NSIP pattern is most commonly found in interstitial lung disease due to connective tissue disease.

Antimycobacterial immune responses in patients with pulmonary sarcoidosis.

Introduction: Sarcoidosis is a multisystem granulomatous disease of unknown origin. Pathogenetic involvement of Mycobacterium tuberculosis has frequently been discussed in the aetiology of sarcoidosis; however, studies still remain contradictory.

Objective: We addressed the question of mycobacterial involvement in the pathogenesis of sarcoidosis by analysing cellular immune responses to mycobacterial antigens.