338 research outputs found
POS0834 Long-Term Outcome of SSC Associated ILD: Improved Survival in PPI Treated Patients
No full textBackground: Gastroesophageal reflux disease (GERD) occurs frequently in patients with systemic sclerosis (SSc) and SSc-associated interstitial lung disease (SSc-ILD). PPI use has to been shown to improve survival in patients with idiopathic pulmonary fibrosis, whereas to date there are no data on the use of PPI in SSc-ILD. Objectives: This study was aimed to assess whether use of PPI is associated with progression of SSc-ILD and survival. Methods: We retrospectively analysed 1931 patients with SSc and SSc-ILD from the German Network for Systemic Sclerosis (DNSS) database (2003 onwards). Kaplan–Meier analysis compared overall survival (OS) and progression-free survival (PFS) in patients with vs. without GERD (SSc and SSc-ILD), and PPI vs. no PPI use (SSc-ILD only). Progression was defined as a decrease in either % predicted forced vital capacity ≥10% or single-breath diffusing capacity for carbon monoxide ≥15%, or death. Results: GERD was not associated with decreased OS or PFS in patients with either SSc or SSc-ILD. In patients with SSc-ILD, PPI use was associated with improved OS vs. no PPI use after 1 year (98.4% [95% confidence interval: 97.6–99.3]; n=760 vs. 90.8% [87.9–93.8]; n=290) and after 5 years (91.4% [89.2–93.8]; n=357 vs. 70.9% [65.2–77.1]; n=106; p<0.0001). PPI use was also associated with improved PFS vs. no PPI use after 1 year (95.9% [94.6–97.3]; n=745 vs. 86.4% [82.9–90.1]; n=278) and after 5 years (66.8% [63.0–70.8]; n=286 vs. 45.9% [39.6–53.2]; n=69; p<0.0001). Conclusion: GERD had no effect on survival in SSc or SSc-ILD. PPIs improved survival in patients with SSc-ILD; however, controlled, prospective trials are needed to confirm this finding. Disclosure of Interests: Michael Kreuter Speakers bureau: Boehringer, Consultant of: Boehringer, Grant/research support from: Boehringer, Francesco Bonella Speakers bureau: Boehringer, Roche, GSK, Consultant of: Boehringer, Roche, GSK, Grant/research support from: Boehringer, Kuhr Kathrin: None declared, Jörg Henes Speakers bureau: Abbvie, Boehringer, Chugai, Roche, Janssen, Novartis, SOBI, Pfizer and UCB, Consultant of: Boehringer, Celgene, Chugai, Roche, Janssen, Novartis, SOBI, Grant/research support from: Chugai, Roche, Janssen, Novartis, SOBI, Pfizer, Elise Siegert: None declared, Gabriela Riemekasten Speakers bureau: Novartis, Janssen, Roche, GSK, Boehringer, Consultant of: Janssen, Actelion, Boehringer, Norbert Blank Consultant of: Sobi, Novartis, Roche, UCB, MSD, Pfizer, Actelion, Abbvie, Boehringer, Grant/research support from: Novartis, Sobi, Christiane Pfeiffer: None declared, Ulf Müller-Ladner: None declared, Alexander Kreuter Speakers bureau: MSD, Boehringer, InfectoPharm, Paid instructor for: MSD, PETER KORSTEN Consultant of: Glaxo, Abbvie, Pfizer, BMS, Chugai, Sanofi, Lilly, Boehringer, Novartis, Grant/research support from: Glaxo, Aaron Juche: None declared, Marc Schmalzing Speakers bureau: Chugai Roche, Boehringer, Celgene, Medac, UCB, Paid instructor for: Novartis, Abbvie, Astra Zeneca, Chugai Roche, Janssen, Consultant of: Chugai Roche, Hexal Sandoz, Gilead, Abbvie, Janssen, Boehringer, Margitta Worm Speakers bureau: Boehringer, Ilona Jandova Speakers bureau: Boehringer, Novartis, Abbvie, Laura Susok Speakers bureau: MSD, Novartis, BMS, Sunpharma, Consultant of: MSD, Tim Schmeiser Consultant of: Abbvie, Boehringer, Novartis, UCB, Claudia Guenther Paid instructor for: Advisory Board Boehringer January 2020, Employee of: Novartis 2002-2005, Gernot Keyszer Consultant of: Boehringer, Jan Ehrchen Speakers bureau: Boehringer, Janssen, Chugai, Sobi, Employee of: Pfizer, Actelion (now Janssen), Andreas Ramming Speakers bureau: Boehringer, Gilead, Janssen, Pfizer, Roche, Consultant of: Boehringer, Pfizer, Grant/research support from: Novartis, Pfizer, Ina Kötter Speakers bureau: several companies, Consultant of: several companies, Grant/research support from: several companies, Hanns-Martin Lorenz Speakers bureau: Abbvie, Astra Zeneca, Actelion, Alexion Amgen, Bayer Vital, Baxter, Biogen, Boehringer, BMS, Celgene, Fresenius, Genzyme, GSK, Gilead, Hexal, Janssen, Lilly, Medac, MSD, Mundipharm, Mylan, Novartis, Octapharm, Pfizer, Roche Chugai, Sandoz, Sanofi, Shire SOBI, Thermo Fischer, UCB, Grant/research support from: basic research studies: Pfizer, Novartis, Abbvie, Gilead, Lilly, MSD, Roche Chugai, Pia Moinzadeh Speakers bureau: Boehringer, Actelion, Grant/research support from: Actelion, Nicolas Hunzelmann Speakers bureau: Boehringer Janssen, Roche, Sanofi, Consultant of: Boehringe
European Dermatology Forum: skin diseases in Europe. Skin diseases with a high public health impact: scleroderma
No full textEarly accrual of organ damage in systemic sclerosis: rationale for forming a multinational inception cohort of patients with scleroderma (The Insync Study)
No full textScientific abstract SAT0202M. Nikpour, M. Baron, M. Hudson, P. Carreira, N. Hunzelmann, T. Frech, J. Sahhar, P. Nash, J. Roddy, L. Schrieber, W. Stevens, S. Proudma
Early mortality in systemic sclerosis: rationale for forming a multinational inception cohort of patients with scleroderma (the INSYNC Study)
No full textScientific abstract FRI0372M. Nikpour, M. Baron, M. Hudson, P. Carreira, N. Hunzelmann, T. Frech, J. Sahhar, P. Nash, G. Major, P. Youssef, J. Roddy, J. Zochling, S. Proudman, W. Steven
Enhanced fibrillin-2 expression is a general feature of wound healing and sclerosis: potential alteration of cell attachment and storage of TGF-beta
No full textWound healing and sclerosis are characterized by an increase of extracellular matrix proteins, which are characteristically expressed in the embryo–fetal period. We analyzed the expression of fibrillin-2, which is typically found in embryonic tissues, but only scarcely in adult skin. In wound healing and sclerotic skin diseases such as lipodermatosclerosis and scleroderma, a marked increase of fibrillin-2 expression was found by immunohistology. Double labelling of fibrillin-2 and tenascin-C, which is also expressed in wound healing and sclerosis, showed co-localization of both proteins. Solid-phase and slot blot-overlay assays showed a dose-dependent binding of the recombinant N-terminal half of fibrillin-2 (rFBN2-N) to tenascin-C. Real-time PCR showed an increase of the fibrillin-2 gene expression in cell culture triggered by typical mediators for fibroblast activation such as serum, IL-4, and TGF-β. By contrast, prolonged hypoxia is not associated with changes in fibrillin-2 expression. Tenascin-C is an anti-adhesive substrate for fibroblasts, whereas fibrillin-2 stimulates cell attachment. Attachment assays using mixed substrates showed decreased cell attachment when tenascin-C and rFBN2-N were coated together, compared with the attachment to rFBN2-N alone. Fibrillins are involved in storage and activation of TGF-β. Immunohistology with an antibody against the latency-associated peptide (LAP (TGF-β1)) showed a marked increase of inactive LAP-bound TGF-β1 in wound healing and sclerotic skin whereas normal skin showed only a weak expression. Double immunofluorescence confirmed a partial colocalization of both proteins. In conclusion, we show that a stimulation of the fibrillin-2 expression is a characteristic feature of fibroblasts present in wound healing and sclerosis, which may be involved in the alteration of cell attachment and storage of inactive TGF-β in the matrix.Jürgen Brinckmann, Nico Hunzelmann, Birgit Kahle, Jürgen Rohwedel, Jan Kramer, Mark A Gibson, Dirk Hubmacher and Dieter P Reinhard
Vaskuläre Therapie der Gefäßbeteiligung bei Patienten des Deutschen Netzwerks für Systemische Sklerodermie: aktueller Stand
No full text
Localized and systemic forms of scleroderma in adults and children
No full textSystemic sclerosis (SSc) presents a great deal of variability in the extent and severity of skin and internal organ involvement. The diagnostic and prognostic significance of autoantibodies in SSc is undisputed and the patient's autoantibody profile represents a fundamental tool for clinicians. Scleroderma is a rare condition in children. Unlike adults, localized scleroderma is more frequent than the systemic sclerosis, nevertheless it represents a disabling condition. In both conditions, no validated outcome measures and proven effective treatment is available to date.Raynaud's phenomenon (RP) is one the most common and significant clinical symptoms of SSc and therefore in patients with RP a capillaroscopic analysis should be carried out as soon as possible. The actual and select advantage of the early nailfold videocapillaroscopic (NVC) analysis is to distinguish between the primary RP and the secondary RP and to allow the early detection of SSc
- …
