View clinical trials related to Lung Diseases, Interstitial.
Filter by:Interstitial lung disease (ILD) is a common complication of dermatomyositis (DM) with prevalence up to 65%, and is considered to be one of the determining factors of prognosis. Clinical amyopathic dermatomyositis (CADM), which is a special phenotype of DM, with characteristic cutaneous manifestations but no or only subclinical myopathy. Many studies, mainly from Asia, including ours, have demonstrated that these patients with CADM tend to develop a rapidly progressive ILD (RPILD) and have a poor response to conventional therapy, such as high-dose corticosteroids and immunosuppressants, leading to lethal outcome with a 6-month survival rate of less than 50%. Pirfenidone, a new oral antifibrotic agent, has been approved for the treatment of idiopathic pulmonary fibrosis (IPF). Randomized controlled trials of pirfenidone in patients with IPF suggested that it could ameliorate pulmonary function decline and improve the progression-free survival. Its utility in connective tissue disease (CTD) related ILD has been implicated, but no evidence has yet demonstrated its efficacy. Therefore, the investigators conduct this study to evaluate the possible therapeutic effects of pirfenidone on RPILD associated with CADM.
To characterize stem cell compartments in their niches in different clinical situations (non-diseased compared to emphysematous and fibrotic pulmonary tissue) and to assess their proliferative and developmental properties in vitro. To further implement lung organoid culture system in the drug screening and development of patient personalized medicine.
Evaluating the diagnostic value of transbronchial lung cryobiopsy (TBLC) as well as its procedural feasibility and safety in a prospective series of 20 patients with diffuse interstitial lung diseases (DILD) who are referred for invasive histopathological diagnostics
1. To evaluate the health status of patients with Interstitial Lung Disease (ILD), and how this varies between subgroups of ILD. 2. To investigate the prevalence of symptoms, anxiety, depression, and sleep-disordered breathing within ILD patient population. 3. To assess the economic impact of ILD. 4. To understand how self-aware ILD patients are regarding their treatment/management. 5. To compare the demographic details, including smoking status and occupational history, of patients with interstitial lung disease
There is a rare condition causing scarring of the lungs termed interstitial lung disease. This comprises a group of conditions which can be divided into separate diseases. The aim of this study is compare a group of patients (the cases) with 2 types of this disease (fibrotic non-specific interstitial pneumonia (NSIP) and idiopathic pulmonary fibrosis (IPF)) with patients without the disease. The study will look at the 2 groups and their exposures to factors such as medications, smoking, previous jobs, previous medical problems and specifically heart disease and any operations or procedures involving the heart. There have been previous studies showing a link between heart disease and scarring of the lungs. This study will look at whether this association is stronger in one or the other type of lung scarring diseases.
Over time, patients with fibrosing or interstitial lung disease (ILD) can develop high lung blood pressures (pulmonary hypertension), and this is associated with poorer prognosis and survival. It is thought that development of PH contributes to the deterioration and death of patients with ILD. Endothelin-1 (ET1) is a substance contributing to the development of both PH and ILD. Bosentan is a drug blocking the action of ET-1 by binding to its receptors. Bosentan clearly benefits patients with PH of unknown cause, or related to other diseases (such as heart conditions, or HIV) both alone and in combination with other treatments. In patients with fibrosing lung disease and PH, there have been no controlled treatment studies. Clearly it is important to evaluate the effectiveness of bosentan in these patients. This study aims to determine the ability of bosentan to reduce high blood pressure in the lungs (pulmonary hypertension) in patients with scarring (fibrosing) lung disease. It is a placebo-controlled double blinded study for 16 weeks (and it is proposed to follow patients in a 16 week open-label phase with bosentan therapy).