Active clinical trials for "Pulmonary Fibrosis"

Idiopathic pulmonary fibrosis is the most severe form of interstitial lung disease. It is known that the prognosis is poor due to extensive inflammation and fibrosis of the lung parenchyma. In case of acute exacerbation, the prognosis becomes worse. In early studies, the 3-month mortality rate reached 50-80%, and in a recent study, the 1-month survival rate was 66%, and the 3-month survival rate was 41%. It is known that 20% of patients with IPF will experience acute exacerbations in their lifetime. The most commonly used treatment for such acute exacerbations is antibiotics and high-dose steroids, or steroid pulse therapy. However, its effectiveness is unclear, and the survival rate is still low. However, as there is no evident therapeutic agent other than steroids, it is included in the treatment guidelines, so conservative treatment is administered while steroids are administered to patients with acute exacerbation of idiopathic pulmonary fibrosis in most upper institutions. There is no precise treatment other than steroids for patients with idiopathic pulmonary fibrosis-acute exacerbation, but the side effects of steroid administration cannot be overlooked. Therefore, a study is needed to confirm whether steroid pulse therapy is necessary or not. Inclusion criteria Among patients with clinically or histologically confirmed idiopathic pulmonary fibrosis, patients who visited the emergency room with dyspnea symptoms Patients within 1 month of exacerbation of respiratory symptoms Patients with increased GGO or worsening of IPF on chest CT within the last 2 weeks Patients who understand the purpose of the clinical study and voluntarily agree to participate in this clinical study When it is determined that steroid administration is necessary under the judgment of the medical staff during the treatment process Exclusion criteria Patients who complain of dyspnea symptoms due to causes other than the respiratory system, such as fluid overload, congestive heart failure, pulmonary embolism, etc. Patients whose respiratory symptoms have worsened for more than 1 month Persons who cannot read consent forms (eg. illiterate, foreigners, etc.) Study design Using an open-label RCT randomization method, the administration will be divided into Group 1 (high-dose followed by low-dose steroid administration) and Group 2 (high-dose/low-dose steroid administration after steroid pulse therapy). Test group: Group 1 (high dose followed by low dose steroid administration) Control group: Group 2 (high-dose/low-dose steroid administration after steroid pulse therapy) ▶ Steroid administration Protocol Group 1: Methylprednisolone 1 mg/kg 7 days → 0.5 mg/kg 7 days → 0.25 mg kg 7 days Group 2: Methylprednisolone 10 mg/Kg (500 mg ~ 1g) pulse 3 days -> Methylprednisolone 1 mg/kg 7 days → 0.5 mg/kg 7 days → 0.25 mg/kg 7 days Response evaluation The level of inflammatory markers Imaging improvement: chest x-ray or CT Pulmonary function test: performed at the outpatient clinic before discharge or 12 weeks after the first visit for acute exacerbation

Despite intense research efforts and clinical trials, there is still no effective treatment that can prolong the survival of patients with IPF. Conventional therapeutic approach includes combination of corticosteroids, anti-oxidants, immunodepressants and immune modulatory anti-fibrotic agents to be discontinued 20 days before screening. The only, so far, therapeutic approach that has been proven effective in terms of prolonging patient's survival is lung transplantation. Nonetheless, not all the patients with IPF are eligible for lung transplantation; there is a significant proportion of these patients that finally succumb while waiting in a lung transplantation list. Therefore, there is critical need for more effective and reliable therapeutic modalities5. Adult Stem Cells (ASCs) seem to represent one of these. Therefore, it is conceivable to assume that adult-stem cells can be easily and safely be applied as a novel therapeutic agent in chronic and fatal lung diseases, including chronic obstructive pulmonary disease (COPD) and IPF. Therefore, there is an urgent need to provide a safe, effective and affordable treatment option for IPF patients. New diagnostic, prognostic and therapeutic strategies need to be developed to reduce the burden of IPF. Given the present lack of appropriate treatment adjunctive in the therapy of IPF, adipose derived stromal vascular fraction provides new opportunities for development of the same. MSCs are having anti-fibrotic activity and hence may be excellent source to tackle pulmonary fibrosis and hence could be explored for their therapeutic potential for treating Idiopathic pulmonary fibrosis. MSC's also display membrane-bound and insoluble secreted molecules involved with cell attachment to neighbouring cells and to the extra cellular matrix.18 This cell surface configuration may enable mesenchymal stem cells to home from bloodstream to mesenchymal tissue.14 As limited clinical information is available about use of SVF and MSC in the IPF patients hence this Open Label, Prospective, Randomized multi center comparative study has been undertaken to explore the tolerability & effectiveness of SVF in one treatment arm and MSC in second treatment arm in IPF patients. Adipose derived stromal vascular fraction and Mesenchymal Stem Cells has been found in preclinical studies to be safe and effective

Idiopathic pulmonary fibrosis (IPF) is a fibroproliferative, irreversible disease of unknown cause, occurring mainly in patients older than 50. IPF is a rare but fatal lung disease, with an estimated prevalence of 14 to 28/100000 and a median survival time of 3 years. Acute exacerbation of IPF (AE-IPF) is a major event of IPF, as it is responsible for the death of 30-50 % of IPF patients; its annual incidence varies between 5 and 10%. The current literature indicates that IPF is associated with the development of an auto-immunity process targeting epithelial and endothelial lung cells. Autoantibodies have been associated with a poorer prognosis. A study by DONAHOE et al. (Plos One, 2015) indicates that the combination of corticosteroids, plasma exchanges, rituximab and immunoglobulins may improve the prognosis of the most severe forms of AE-IPF. In that study, the observed survival rate in patients receiving this combination of treatment was 70% as compared with 20% in historical controls. This therapeutic combination approach is designed both to eliminate and inhibit the production of circulating antibodies targeting the lungs. Considering the high mortality rate of an AE-IPF episode and the potential benefit of such an original approach, a well-conducted randomized controlled trial is critical.

Single-arm, open-label observational study in idiopathic pulmonary fibrosis (IPF) patients receiving usual care at an interstitial lung disease specialist center. The objectives are [1] to characterise the longitudinal trends of patient-measured Forced Vital Capacity (FVC) and impact of IPF on daily life Patient Reported Outcome Measures (PROM) in a cohort of patients with IPF [2] to determine the correlation (if any) between patient-measured FVC and PROMs with clinic-observed measurements and [3] to assess if longitudinal trends in patient-measured FVC are predictive of clinical health outcomes in IPF. An additional purpose is to assess the acceptability and utility of the patientMpower app in helping IPF patients and their healthcare professional caregivers manage their condition. Patients will record FVC, symptoms (e.g. dyspnea) and activity (step count) daily and PROM once a week on the patientMpower app. The planned observation period is sixteen weeks. No additional clinic visits are required (versus usual care). In-clinic assessments of lung function, dyspnea and PROM will be done at baseline and study end. Patients and healthcare professionals will provide their opinion on utility and acceptability of patientMpower app at study end.

"Determination of the effectiveness of nebulized morphine in the treatment of dyspnea in patients with advanced idiopathic pulmonary fibrosis"

SARS-CoV-2 infection induces a hyperinflammatory syndrome, causing the acute respiratory distress syndrome, massive lung cell destruction and, as a plausible sequelae, pulmonary fibrosis in COVID-19 patients. Current focus has been on the development of novel immunosuppressant therapies, in order to control the cytokine storm in COVID-19 patients. Thus, the effect of steroids, intravenous immunoglobulin, non-steroidal immunosuppressants, selective cytokine blockade, JAK/STAT pathway inbhibition, and mesenchymal precursor cells have been evaluated. Based on the above information, we propose COLLAGEN-POLYVINYLPYRROLIDONE (Distinctive name: FibroquelMR, active substance: Collagen-polyvinylpyrrolidone, pharmaceutical form: intramuscular injectable solution, with sanitary registration No. 201M95 SSA IV and SSA code: 010 000 3999) as a potential drug for the downregulation of the cytokine storm. Polymerized type I collagen reduces the expression of IL-1β, IL-8, TNF-alpha, TGF-β1, IL-17, Cox-1, leukocyte adhesion molecules (ELAM-1, VCAM- 1 and ICAM-1), some other mediators of inflammation and increases the levels of IL-10 and the number of regulatory T cells. In addition, it promotes the mechanisms of inhibition of tissue fibrosis, without adverse effects in rheumatoid arthritis and osteoarthritis.

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).

Idiopathic pulmonary fibrosis (IPF) is a diffuse lung disease, associated with the histological appearance of usual interstitial pneumonia (UIP), with an inexorably deteriorating clinical course. Prognosis is poor, reported median survival is less than 3 years. The prevalence is estimated as being 3 to 10 per 100.000 in different Western populations. To date, no pharmacological therapy has been proven to alter or reverse the pathogenic process of IPF. Most treatments trials have been observational case series of small patient populations and very few have been randomized, prospective and placebo-controlled. Two recent Cochrane reviews investigated the role of corticosteroids and other immunomodulatory agents and concluded that there is no evidence for their use in IPF. Most current therapies are targeted to suppress the inflammatory component of the disease, based on the theory that it would be chronic alveolar inflammation which leads to parenchymal remodeling and fibrosis. Recently, a hypothesis that has gained acceptance suggests that fibrosis may result directly from alveolar injury, promoting an abnormal fibrogenic repair mediated by fibroblasts and myofibroblasts. One of the cytotoxic agents most widely used and better tolerated in the management of IPF is azathioprine. Based upon limited data available and from a single small high quality randomized controlled trial (RCT), this drug appears to confer, given in conjunction with prednisone, a marginal long term survival advantage. Since this combination therapy is associated serious adverse effect, we planned to design a trial of low dose corticosteroid and azathioprine versus placebo in management of IPF, evaluating progression-free survival. Our study hypothesis is: Combined therapy with azathioprine and corticosteroids improves progression-free survival in patients with the diagnosis of IPF.

The Chronic Hypersensitivity Pneumonitis (HP), is an inflammatory disease who has an evolution to develop progressive interstitial fibrosis, who cause the death of the patient. Actually HP has been treated with Prednisone and occasionally with Azathioprine, but unfortunately the treatment with these drugs have not an effective result to treat the interstitial fibrosis. Pirfenidone has been studied over the world for the treatment of Fibrotic diseases, with positive results, and due to the Pirfenidone mechanism of action has anti-inflammatory and anti-fibrotic properties, the investigators propose to evaluate the addition of Pirfenidone to the actual treatment with Prednisone and Azathioprine in the treatment of patients with Pulmonary Fibrosis secondary to a Chronic Hypersensitivity Pneumonitis.

The first-in-man study are designed as below to assess safety, tolerability, and preliminary pharmacokinetics of ZL-2102. Double-blind randomized, placebo-controlled ascending single oral doses (Part 1, ZL-2102-SAD); Open-label, randomized, 2-sequence, 2-period, 2-treatment crossover (Part 2, ZL-2102-FED); Double-blind randomized, placebo-controlled, ascending repeated oral doses for 14 days (Part 3, ZL-2102-MAD). A total of 104 subjects will be enrolled.