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Acute exacerbations (AE) are a dreaded manifestation of idiopathic pulmonary fibrosis (IPF) that presents with rapidly worsening respiratory function over days to weeks. AE account for about 1/2 the deaths in IPF patients, and are refractory to all medical therapies attempted to date. Considerable preliminary data shows pathological B-cell abnormalities and autoantibodies are present in AE-IPF and associated with disease severity. The experimental therapy here (therapeutic plasma exchange plus rituximab plus intravenous immunoglobulin) is mechanistically targeted to ameliorate autoantibody-mediated pulmonary injury. Anecdotal pilot...
Chronic respiratory diseases including chronic obstructive pulmonary disease (COPD), bronchiectasis (BE) or pulmonary fibrosis (PF) are usually not curable with damaged pulmonary structure and function. Bronchial basal cells are proved to regenerate bronchus and alveoli to repair the pulmonary injuries. In this study, we intend to perform an open, single-armed phase I clinical trial by transplantation of autologous bronchial basal cells on patients suffered from COPD, BE or PF. During the treatment, autologous bronchial basal cells, which were isolated from fiberoptic bronchoscopy and expanded in vitro, will be injected directly into...
Interstitial lung diseases (ILD) are a group of diseases affecting the lung interstitium. The lung scarring that occurs in ILD is often irreversible with only mitigating therapy available so far. This study intends to carry out an open, single-armed, phase I/II clinical trial to investigate whether lung stem cells can regenerate damaged lung tissue. During the treatment, lung stem cells will be isolated from patients' own bronchi and expanded in vitro. After careful characterization, cultured cells will be injected directly into the lesion by fiberoptic bronchoscopy. The safety and efficacy of the treatment will be monitored by measuring...
The purpose of this study is to determine whether a regimen of high-dose immunoablative therapy will demonstrate safety that is consistent or improved with other published regimens in SSc patients, while maintaining a treatment effect. We also hypothesize that our mechanistic studies will yield biomarkers that may herald disease recurrence or progression following alterations in the recovery of immune cells in the skin and/or bronchial lavage or blood.
Patients with sarcoidosis need treatment options that effectively control their disease without causing undesirable side effects. An appealing strategy is to repurpose existing drugs which possess beneficial immune modulating activity and are safe for long-term use. Recently, increased activity of the mTOR intracellular signalling pathway in inflammatory cells has emerged as a key driver of granulomatous inflammation in mouse models and patients with sarcoidosis. The macrolide antibiotic azithromycin directly inhibits mTOR activity in inflammatory cells, making it a prime target for drug repurposing in sarcoidosis. Azithromycin has an...
This is a 52-week, randomized, open and routine treatment controlled study. This study will assess the safety and efficacy of basiliximab as an add-on treatment for interstitial pneumonia in clinical amyopathic dermatomyositis (CADM) patients. 100 CADM patients are planned to be enrolled in a single center.
This prospective cohort study will investigate whether progression of the interstitial lung diseases is related to specific clinical endpoints and their changes over time. Longitudinal data of patients will be compared to an age-matched control group during a follow-up of at least two years.
The investigators aim to examine the genetic determinants of interstitial lung disease in a cohort of subjects with regular exposure to pigeons, a known cause of one form of interstitial lung disease known as hypersensitivity pneumonitis. The investigators hope the investigator's work will provide insights of use to clinicians and patients with hypersensitivity pneumonitis and other interstitial lung diseases.
Objectives: 1.- To compare the level of oxygenation achieved during muscular training with conventional oxygen systems (nasal cannulas) versus nasal High-flow oxygen therapy. 2.-To compare benefits achieved with both systems, in terms of: level of exercise during training; effort tolerance in the 6 minutes walking test (6MWT); improvement of dyspnoea and Health-related quality of life (HRQoL). And analyse the effects of nasal High-flow oxygen therapy on the acute exercise in a subgroup of patients. Method: Multicentric randomized clinical trial. Patients with ILD in fibrotic phase who present oxygen desaturation during 6MWT (SpO2 mean ≤...
Fibroproliferative diseases, including pulmonary, cardiac and vascular fibrosis share common pathogenetic mechanisms. Furthermore, cardiovascular comorbidities are frequently found in patients with IPF. However, the prevalence of cardiac and vascular fibrosis in patients with IPF have yet to be determined. Main Purpose of this study is to evaluate, with non-invasive methods (echocardiogram, endothelial function and pulse wave velocity) and blood biomarkers (galectins-3, osteopontin, periostin and pro-BNP), the presence of vascular fibrosis (vascular rigidity and endothelial function) and cardiac fibrosis (prevalence of HFpEF - Heart...
The Pulmonary Fibrosis Foundation rates among top charities in the U.S. The PFF has a three-star rating from Charity Navigator and is a Better Business Bureau accredited charity.
