Jan-Gerd Rademacher1, Chris Wincup2,3, Björn Tampe1, Peter Korsten1. 1. Department of Nephrology and Rheumatology, University Medical Center Göttingen, Göttingen, Germany. 2. Department of Rheumatology, University College London, London, UK. 3. Arthritis Research UK Centre for Adolescent Rheumatology, University College London, London, UK.
Abstract
Background: Systemic sclerosis is a rare autoimmune disease characterized by skin and organ fibrosis, and vasculopathy. Raynaud's phenomenon is almost universally present in systemic sclerosis and can be the most debilitating symptom. Raynaud's phenomenon may lead to the development of digital ulcers, potentially complicated by infection, tissue necrosis, and auto-amputation. Recommended treatments have variable efficacy. Methods: We report the case of a 30-year-old woman with diffuse systemic sclerosis suffering from severe Raynaud's phenomenon and digital ulcers with digital tissue necrosis who was treated with combination therapy of an endothelin receptor antagonist and phosphodiesterase 5 inhibitor. In addition, we reviewed the literature on the topic. Results: Previous therapy with calcium-channel blockers, intravenous iloprost, and bosentan had all failed to control symptoms. We added sildenafil in combination with bosentan and observed a rapid and sustained treatment effect. Raynaud's phenomenon severity, number of attacks, and attack duration decreased within 2 weeks of initiating treatment. Furthermore, this resulted in the healing of established digital ulcers. Conclusion: Our case report suggests that combination therapy may be a feasible treatment for the most severely affected and refractory patients. In our literature review, we found one retrospective study and three additional cases with similarly encouraging results.
Background: Systemic sclerosis is a rare autoimmune disease characterized by skin and organ fibrosis, and vasculopathy. Raynaud's phenomenon is almost universally present in systemic sclerosis and can be the most debilitating symptom. Raynaud's phenomenon may lead to the development of digital ulcers, potentially complicated by infection, tissue necrosis, and auto-amputation. Recommended treatments have variable efficacy. Methods: We report the case of a 30-year-old woman with diffuse systemic sclerosis suffering from severe Raynaud's phenomenon and digital ulcers with digital tissue necrosis who was treated with combination therapy of an endothelin receptor antagonist and phosphodiesterase 5 inhibitor. In addition, we reviewed the literature on the topic. Results: Previous therapy with calcium-channel blockers, intravenous iloprost, and bosentan had all failed to control symptoms. We added sildenafil in combination with bosentan and observed a rapid and sustained treatment effect. Raynaud's phenomenon severity, number of attacks, and attack duration decreased within 2 weeks of initiating treatment. Furthermore, this resulted in the healing of established digital ulcers. Conclusion: Our case report suggests that combination therapy may be a feasible treatment for the most severely affected and refractory patients. In our literature review, we found one retrospective study and three additional cases with similarly encouraging results.
Authors: Gideon A Paul; J Simon R Gibbs; Alan R Boobis; Allifia Abbas; Martin R Wilkins Journal: Br J Clin Pharmacol Date: 2005-07 Impact factor: 4.335
Authors: J H Korn; M Mayes; M Matucci Cerinic; M Rainisio; J Pope; E Hachulla; E Rich; P Carpentier; J Molitor; J R Seibold; V Hsu; L Guillevin; S Chatterjee; H H Peter; J Coppock; A Herrick; P A Merkel; R Simms; C P Denton; D Furst; N Nguyen; M Gaitonde; Carol Black Journal: Arthritis Rheum Date: 2004-12
Authors: Anthony J Tyndall; Bettina Bannert; Madelon Vonk; Paolo Airò; Franco Cozzi; Patricia E Carreira; Dominique Farge Bancel; Yannick Allanore; Ulf Müller-Ladner; Oliver Distler; Florenzo Iannone; Raffaele Pellerito; Margarita Pileckyte; Irene Miniati; Lidia Ananieva; Alexandra Balbir Gurman; Nemanja Damjanov; Adelheid Mueller; Gabriele Valentini; Gabriela Riemekasten; Mohammed Tikly; Laura Hummers; Maria J S Henriques; Paola Caramaschi; Agneta Scheja; Blaz Rozman; Evelien Ton; Gábor Kumánovics; Bernard Coleiro; Eva Feierl; Gabriella Szucs; Carlos Alberto Von Mühlen; Valeria Riccieri; Srdan Novak; Carlo Chizzolini; Anna Kotulska; Christopher Denton; Paulo C Coelho; Ina Kötter; Ismail Simsek; Paloma García de la Pena Lefebvre; Eric Hachulla; James R Seibold; Simona Rednic; Jirí Stork; Jadranka Morovic-Vergles; Ulrich A Walker Journal: Ann Rheum Dis Date: 2010-06-15 Impact factor: 19.103
Authors: Florian M P Meier; Klaus W Frommer; Robert Dinser; Ulrich A Walker; Laszlo Czirjak; Christopher P Denton; Yannick Allanore; Oliver Distler; Gabriela Riemekasten; Gabriele Valentini; Ulf Müller-Ladner Journal: Ann Rheum Dis Date: 2012-05-21 Impact factor: 19.103
Authors: Christopher P Denton; Thomas Krieg; Loic Guillevin; Barbara Schwierin; Daniel Rosenberg; Mariabeth Silkey; Maurice Zultak; Marco Matucci-Cerinic Journal: Ann Rheum Dis Date: 2012-01-12 Impact factor: 19.103
Authors: Claudia S Brueckner; Mike O Becker; Thomas Kroencke; Doerte Huscher; Hans Ulrich Scherer; Margitta Worm; Gerd Burmester; Gabriela Riemekasten Journal: Ann Rheum Dis Date: 2009-11-08 Impact factor: 19.103