More Than 3000 Patients Vasoactive Therapy in Systemic Sclerosis : Real-life Therapeutic Practice in Hunzelmann and The German Network for Systemic Scleroderma

Objective. Vasculopathy is a key factor in the pathophysiology of systemic sclerosis (SSc) and the main cause for Raynaud phenomenon (RP), digital ulcers (DU), and/or pulmonary arterial hypertension (PAH). It is so far unknown how patients with SSc are treated with vasoactive agents in daily practice. To determine to which extent patients with SSc were treated with different vasoactive agents, we used data from the German Network for Systemic Scleroderma registry. Methods. The data of 3248 patients with SSc were analyzed. Results. Patients were treated with vasoactive drugs in 61.1% of cases (1984/3248). Of these, 47.6% received calcium channel inhibitors, followed by 34.2% treated with angiotensin-converting enzyme (ACE) inhibitors, 21.1% treated with intravenous (IV) prostanoids, 10.1% with pentoxifylline, 8.8% with angiotensin 1 receptor antagonists (AT1RA), 8.7% with endothelin 1 receptor antagonists (ET1RA), 4.1% with phosphodiesterase type 5 (PDE5) inhibitors, and 5.3% with others. Patients with RP received vasoactive therapy in 63.3% of cases, with DU in 70.1%, and with PAH in 78.2% of cases. Logistic regression analysis revealed that patients with PAH were significantly more often treated with PDE5 inhibitors and ET1RA, and those with DU with ET1RA and IV prostanoids. In addition, 41.8% of patients were treated with ACE inhibitors and/or AT1RA. Patients registered after 2009 received significantly more often ET1RA, AT1RA, and IV prostanoids compared with patients registered prior to 2005. Conclusion. These data clearly indicate that many patients with SSc do not yet receive sufficient vasoactive therapy. Further, in recent years, a marked change of treatment regimens can be observed. (First Release November 15 2015; J Rheumatol 2016;43:66–74; doi:10.3899/jrheum.150382) Key Indexing Terms: SYSTEMIC SCLEROSIS VASOACTIVE THERAPY REAL LIFE GERMAN NETWORK FOR SYSTEMIC SCLERODERMA From the Department of Dermatology, University Hospital of Cologne; Department of Internal Medicine, Hospital of Cologne Merheim; Institute of Medical Statistics, Informatics and Epidemiology, University of Cologne, Cologne; Department of Rheumatology, University Medical Center Schleswig-Holstein, Lübeck; Department of Rheumatology and Clinical Immunology, and Department of Dermatology, University of Berlin, Charité, Berlin; Department of Rheumatology, Immanuel Hospital, Berlin-Buch; Department of Dermatology, and Department of Rheumatology, University Hospital Tübingen, Tübingen; Department of Internal Medicine, Division of Rheumatology, Heidelberg University Hospital; Division of Immunogenetics, German Cancer Research Center, Heidelberg; Department of Rheumatology and Clinical Immunology, University Medical Center, Freiburg; Department of Rheumatology and Clinical Immunology, University of Giessen, Kerckhoff Clinic, Bad Nauheim; Department of Dermatology, HELIOS St. Elisabeth Clinic, Oberhausen; Department of Dermatology, St. Joseph Hospital, RuhrUniversity of Bochum, Bochum; Department of Dermatology, University Hospital Carl Gustav Carus, Dresden; Department of Rheumatology, Johanniter-Hospital, Treuenbrietzen; Department of Dermatology, Ulm University Hospital, Ulm; Department of Dermatology, University Hospital Regensburg, Regensburg; Department of Rheumatology, Clinic of Rheumatology of Aachen, Aachen; Department of Dermatology, University Hospital Leipzig, Leipzig; Department of Rheumatology, and Department of Dermatology, University Hospital Erlangen, Erlangen; Department of Dermatology, TUM University Hospital; Department of Dermatology, Ludwig Maximilian University, Munich; Department of Rheumatology and Nephrology, University Hospital Duesseldorf, Duesseldorf; Department of Dermatology, University Hospital Göttingen, Göttingen; Department of Dermatology, University Medical Center, Johannes Gutenberg University, Mainz; Clinic for Dermatology, Hamburg Journal of Rheumatology The on November 6, 2017 Published by www.jrheum.org Downloaded from Alstertal; Department of Dermatology, Helios Clinic, Wuppertal; Center for Rheumatology Bad Doberan, Bad Doberan; Center for Rheumatology Acura, Baden-Baden; Department of Dermatology, University Hospital of Würzburg, Würzburg; Department of Rheumatology, University Hospital Frankfurt, Frankfurt; Center for Rheumatology (Rehabilitation), Rheinfelden; Clinic for Internal Medicine, Klinikum Stephansplatz; Department of Rheumatology, Asklepios Clinic Altona, Hamburg; Department of Rheumatology, GRP-Hospital Ruesselheim, Darmstadt; Department of Dermatology and Venereology, University Hospital Muenster, Muenster, Germany; Department of Dermatology, Medical University of Graz, Graz, Austria. Supported by a grant of the German Federal Ministry of Education and Research (BMBF; 01GM0310 NH, TK; 01GM0631 CS) and the Edith Busch Foundation. The work of Pia Moinzadeh was supported by “Koeln Fortune” (155/2014) and “Deutsche Stiftung Sklerodermie” (3649/0096/31) grants. P. Moinzadeh, MD, Department of Dermatology, University Hospital of Cologne; G. Riemekasten, MD, Department of Rheumatology, University Medical Center Schleswig-Holstein; E. Siegert, Department of Rheumatology and Clinical Immunology, University of Berlin, Charité; G. Fierlbeck, MD, Department of Dermatology, University Hospital Tübingen; J. Henes, MD, Department of Rheumatology, University Hospital Tübingen; N. Blank, MD, Department of Internal Medicine, Division of Rheumatology, Heidelberg University Hospital; I. Melchers, MD, Department of Rheumatology and Clinical Immunology, University Medical Center; U. Mueller-Ladner, MD, Department of Rheumatology and Clinical Immunology, University of Giessen, Kerckhoff Clinic; M. Frerix, MD, Department of Rheumatology and Clinical Immunology, University of Giessen, Kerckhoff Clinic; A. Kreuter, MD, Department of Dermatology, HELIOS St. Elisabeth Clinic; C. Tigges, MD, Department of Dermatology, HELIOS St. Elisabeth Clinic; N. Lahner, MD, Department of Dermatology, St. Joseph Hospital, Ruhr-University of Bochum; L. Susok, MD, Department of Dermatology, St. Joseph Hospital, Ruhr-University of Bochum; C. Guenther, MD, Department of Dermatology, University Hospital Carl Gustav Carus; G. Zeidler, MD, Department of Rheumatology, Johanniter-Hospital; C. Pfeiffer, MD, Department of Dermatology, Ulm University Hospital; M. Worm, MD, Department of Dermatology, University of Berlin, Charité; S. Karrer, MD, Department of Dermatology, University Hospital Regensburg; E. Aberer, MD, Department of Dermatology, Medical University of Graz; A. Bretterklieber, MD, Department of Dermatology, Medical University of Graz; E. Genth, MD, Department of Rheumatology, Clinic of Rheumatology of Aachen; J.C. Simon, MD, Department of Dermatology, University Hospital Leipzig; J.H. Distler, MD, Department of Rheumatology, University Hospital Erlangen; R. Hein, MD, Department of Dermatology, TUM University Hospital; M. Schneider, MD, Department of Rheumatology and Nephrology, University Hospital Duesseldorf; C.S. Seitz, MD, Department of Dermatology, University Hospital Göttingen; C. Herink, MD, Department of Dermatology, University Hospital Göttingen; K. Steinbrink, MD, Department of Dermatology, University Medical Center, Johannes Gutenberg University; M. Sárdy, MD, Department of Dermatology, Ludwig Maximilian University; R. Varga, MD, Department of Dermatology, Ludwig Maximilian University; H. Mensing, MD, Clinic for Dermatology; C. Mensing, MD, Clinic for Dermatology; P. Lehmann, MD, Department of Dermatology, Helios Clinic; G. Neeck, MD, Center for Rheumatology Bad Doberan; C. Fiehn, MD, Center for Rheumatology Acura; M. Weber, MD, Department of Internal Medicine, Hospital of Cologne Merheim; M. Goebeler, MD, Department of Dermatology, University Hospital of Würzburg; H. Burkhardt, MD, Department of Rheumatology, University Hospital Frankfurt; M. Buslau, MD, Center for Rheumatology (Rehabilitation); K. Ahmadi-Simab, MD, Clinic for Internal Medicine, Klinikum Stephansplatz; A. Himsel, MD, Department of Rheumatology, GRP-Hospital Ruesselheim; A. Juche, MD, Department of Rheumatology, Immanuel Hospital; I. Koetter, MD, Department of Rheumatology, Asklepios Clinic Altona; A. Kuhn, MD, Division of Immunogenetics, German Cancer Research Center; M. Sticherling, MD, Department of Dermatology, University Hospital Erlangen; M. Hellmich, MD, Institute of Medical Statistics, Informatics and Epidemiology, University of Cologne; K. Kuhr, MD, Institute of Medical Statistics, Informatics and Epidemiology, University of Cologne; T. Krieg, MD, Department of Dermatology, University Hospital of Cologne; J. Ehrchen, MD, Department of Dermatology and Venereology, University Hospital Muenster; C. Sunderkoetter, MD, Department of Dermatology and Venereology, University Hospital Muenster; N. Hunzelmann, MD, Department of Dermatology, University Hospital of Cologne. Address correspondence to Dr. P. Moinzadeh, Department of Dermatology and Venereology, University of Cologne, Kerpener Str. 62, 50937 Cologne, Germany. E-mail: pia.moinzadeh@uk-koeln.de Full Release Article. For details see Reprints and Permissions at jrheum.org Accepted for publication August 28, 2015. Vasculopathy presents as a major clinical problem in patients with systemic sclerosis (SSc) and is the main cause for Raynaud phenomenon (RP), digital ulcerations (DU), pulmonary hypertension, telangiectasia and/or renovascular complications with proteinuria, and severe renal crisis1,2. The pathophysiology of vasculopathy in SSc is complex and only partially understood. It is a result of an imbalance between vasoconstrictors and vasodilators. Endothelial cell damage is one of the early events in SSc, although it is still not definite whether this is also the initiating factor in this multisystem disease3. Unfortunately, no curative treatment strategies are available yet; however, depending on organ manifestations, several vasodilator agents are available to reverse vasoconstrictio