Interstitial pneumonia with autoimmune features (IPAF) was defined in 2015 by the Working Group of the European Respiratory Society (ERS) and the American Thoracic Society (ATS) as interstitial pneumonia with some clinical and/or serological features suggesting presence of an underlying autoimmune disorder. However, ofiicial criteria for diagnosis of an autoimmune disease are not met. Aims of the study: 1. Determine the incindence of IPAF in comparison with interstitial lung diseases (ILDs) and classic autoimmune diseases (ADs) in polish pulmonological centers. 2. Clinical, serological, functional and radiological and...
Born out of the European Union 7th Framework Programme funded project European IPF Network (eurIPFnet), the European IPF Registry (eurIPFreg) has become Europe's leading database of longitudinal data from IPF patients, including control groups of patients with other lung diseases. The registry was initiated with the intention of creating a permanent and continuously growing record of well defined data on IPF in Europe, in order to increase the chances of finding better treatment options for this devastating disease. Clinical colleagues who would like to actively participate (both in terms of patient recruitment and data analysis) are...
Idiopathic pulmonary fibrosis (IPF) is a chronic and ultimately fatal disease characterized by a progressive damage of lung structure and decline in lung function.This study intends to carry out an open, single-center, non-randomized, self control phase I clinical trial. During the treatment, lung stem cells will be isolated from patients' own bronchi and expanded in vitro. Cultured cells will be injected directly into the lesion by fiberoptic bronchoscopy after lavage. After twelve-month observation, the investigators will evaluate the safety and efficacy of the treatment by measuring the key clinical indicators.
This study will evaluate the safety and efficacy of Jianfei Kangfu Cao in the treatment of primary Sjogren's syndrome associated interstitial lung disease.
Multi-center observational study to evaluate the histopathology and transcriptome of cutaneous lesions in patients with several different types of vasculitis.
This open label clinical study will be conducted in School of Chinese Medicine Clinics at The University of Hong Kong (HKU) to preliminarily determine whether treatment with the herbal formula PROLUNG could improve Idiopathic Pulmonary Fibrosis (IPF) symptoms, respiratory function and the quality of life of patients with IPF compared with pretreatment baseline. We propose to recruit 30 participants. The diagnosis and screening will be conducted by respiratory physicians. Those who meet the inclusion criteria will be referred by the expert to the PI. The PI will prescribe the formula in granule form to the participants in Traditional Chinese...
This is a research study of a drug called cobimetinib in children and adults diagnosed with Langerhans cell histiocytosis (LCH), and other histiocytic disorders that has returned or does not respond to treatment. Cobimetinib blocks activation of a protein called Mitogen-activated protein kinase (MEK) that is part of incorrect growth signals in histiocytosis cells. Four different groups of patients will be enrolled.
Extension of the PFBIO cohort which includes patients with newly diagnosed idiopathic pulmonary fibrosis (IPF) for longitudinal follow-up for up to 5 years. In the PFBIO-EXA extension, patients are included if they experience an exacerbation, or other increase in respiratory symptoms requiring hospital admission, for further collection of clinical and biological data.
The purpose of this study is to look at whether bortezomib, mycophenolate or the combination of both is better to treat scarring of the lung caused by Systemic Sclerosis.
Idiopathic pulmonary fibrosis (IPF) is a chronic and potentially fatal lung disease. As IPF progresses, patients become increasingly breathless with reduced exercise capacity and quality of life. A cardiopulmonary exercise test (CPET) is a gold standard way of assessing patients with IPF. An incremental shuttle walk test is simpler, cheaper, more widely available and anecdotally preferable to CPET. The investigators will compare the measurements made in an ISWT and a CPET in patients with IPF . We aim to determine whether sufficient information can be gathered in an ISWT to negate the need to undertake CPET.