FDA OKs Nintedanib (Ofev) for Rare Lung Disease FDA OKs Nintedanib (Ofev) for Rare Lung Disease
Nintedanib capsules are approved for slowing the rate of decline in pulmonary function in adults with systemic sclerosis-associated interstitial lung disease.News Alerts
CONCLUSION: Three-dimensional computed tomography reconstruction, as well as the hollow appearance of a tubular structure after removal of a central catheter may help differentiate a fibroblastic sheath from a retained catheter fragment. Accurate surgical notes mentioning the length of the catheter at implant and explant are also of paramount importance. PMID: 31526092 [PubMed - as supplied by publisher]
Condition: Glabellar Lines Interventions: Drug: AGN-151586; Drug: Placebo Sponsor: Allergan Not yet recruiting
Conclusions Our data demonstrate that hedgehog pathways and TGF-β signalling both converge to GLI2 and that GLI2 integrates those signalling to promote tissue fibrosis. These findings may have translational implications as non-selective inhibitors of GLI2 are in clinical use and selective molecules are currently in development.
Conclusion These results suggest that nucleosomes and its signalling in B and T cells contribute to disease development in SSc via TLR9.
Conclusions We demonstrate that nintedanib effectively inhibits the endogenous as well as cytokine-induced activation of SSc fibroblasts and exerts potent antifibrotic effects in different complementary mouse models of SSc. These data have direct translational implications for clinical trials with nintedanib in SSc.
Conclusions Typical histopathological features of SSc-related cardiomyopathy are mimicked by uPAR-deficient mice. The downregulation of uPAR in the myocardium of patients with SSc may suggest similar underlying pathogenetic mechanisms. uPAR-deficient mice could be used as a preclinical model to study the mechanisms and therapeutic approaches of myocardial involvement in SSc.
MONTREAL – The immunosuppressant mycophenolate mofetil worked as effectively as cyclophosphamide for treating scleroderma-related interstitial lung disease while being better tolerated and causing fewer adverse effects in a multicenter, head-to-head comparison with 142 randomized patients. “The...
Conclusions The comparison of RTX treated versus untreated matched-control SSc patients from the EUSTAR cohort demonstrated improvement of skin fibrosis and prevention of worsening lung fibrosis, supporting the therapeutic concept of B cell inhibition in SSc.
Patients with newly diagnosed systemic sclerosis should have a high-resolution lung CT and pulmonary function tests, because when assessed together, their findings identify patients at high risk of interstitial lung disease, researchers say. In a study of 305 patients with systemic sclerosis...
Background Systemic sclerosis (SSc)-overlap syndromes are a very heterogeneous and remarkable subgroup of SSc-patients, who present at least two connective tissue diseases (CTD) at the same time, usually with a specific autoantibody status. Objectives To determine whether patients, classified as overlap syndromes, show a disease course different from patients with limited SSc (lcSSc) or diffuse cutaneous SSc (dcSSc). Methods The data of 3240 prospectively included patients, registered in the database of the German Network for Systemic Scleroderma and followed between 2003 and 2013, were analysed. Results Among 3240 regi...