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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 -...
Sarcoidosis is a disease of unknown cause which affects adults of all ethnic backgrounds. Clumps of tissue called granulomas develop primarily in the lungs, but can damage other organs, especially the heart. Anecdotal evidence from autopsy studies suggests the heart is affected in up to 68% of patients, but there is much uncertainty about this figure. If undetected and untreated, it can lead to serious complications or even sudden death. The current recommendation is to perform heart tracings (ECG s) on all patients, but this detects fewer than half of those with heart involvement. Blood markers traditionally used to diagnose...
Idiopathic pulmonary fibrosis (IPF) is a condition where scar tissue (called fibrosis) builds up in the lungs. It usually gets worse over time. Fibrosis causes the lungs to become stiff, and reduces the amount of oxygen that the lungs can take up. People with IPF complain of worsening breathlessness, which limits their day to day activities. Lung function tests are breathing tests that measure how well your lungs are working, and are used by doctors to decide whether to start or stop medicines in people with IPF. However, people with IPF tell us that lung function tests require a lot of effort, can make them cough and feel very...
Background: - Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease that becomes worse over time. There is currently no effective treatment for it. Researchers want to study the disease and learn new ways to treat it. Objectives: - To discover new pathways that are involved in pulmonary fibrosis. To develop new drugs that may be used to treat pulmonary fibrosis. Eligibility: - People at least 18 years old with IPF. - Healthy volunteers at least 18 years old. Design: - Participants will be screened with medical history, questionnaire, and physical exam. They will...
Very recent studies indicate that a high percentage of HCL, about 50%, have mutations in the B-RAF oncogene. The development of ultrasensitive methodologies capable of identifying these mutations in bronchoalveolar lavage will represent a significant advance in the diagnosis and treatment of these patients. An undetermined percentage of HCL does not present mutations in B-RAF. Consequently, the deep genetic analysis, through the use of techniques of massive sequencing, can favor the identification of new alterations that contribute to the development of the disease. We hypothesized that patients with HCL may present a different...
Hermansky-Pudlak Syndrome (HPS) is an inherited disease which results in decreased pigmentation (oculocutaneous albinism), bleeding problems due to a platelet abnormality (platelet storage pool defect), and storage of an abnormal fat-protein compound (lysosomal accumulation of ceroid lipofuscin). The disease can cause poor functioning of the lungs, intestine, kidneys, or heart. The major complication of the disease is pulmonary fibrosis and typically causes death in patients ages 40 - 50 years old. The disorder is common in Puerto Rico, where many of the clinical research studies on the disease have been conducted. Neither the full...
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)...
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.
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.
