International Journal of Rheumatology Vascular Disease in Systemic Sclerosis Guest Editors: Lorinda Chung, Oliver Distler, Laura Hummers, Eswar Krishnan, and Virginia Steen Vascular Disease in Systemic Sclerosis International Journal of Rheumatology Vascular Disease in Systemic Sclerosis Guest Editors: Lorinda Chung, Oliver Distler, Laura Hummers, Eswar Krishnan, and Virginia Steen Copyright © 2010 Hindawi Publishing Corporation. All rights reserved. This is a special issue published in volume 2010 of “International Journal of Rheumatology.” All articles are open access articles dis- tributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. International Journal of Rheumatology Editorial Board Salvatore M. F. Albani, USA Shinichi Kawai, Japan Lisa G. Rider, USA Ernest Brahn, USA Charles J. Malemud, USA Vicente Rodriguez-Valverde, Spain Ruben Burgos-Vargas, Mexico Johanne Martel-Pelletier, Canada Bruce M. Rothschild, USA Dirk Elewaut, Belgium Terr y L. Moore, USA J. R. Seibold, USA Luis R. Espinoza, USA Ewa Paleolog, UK Leonard H. Sigal, USA Barri J. Fessler, USA Thomas Pap, Germany Malcolm Smith, Australia Piet Geusens, Belgium Karel Pavelka, Czech Republic G. C. Tsokos, USA Hiroshi Hashimoto, Japan Jean-Pierre Pelletier, Canada Ronald van Vollenhoven, Sweden Sergio Jimenez, USA Proton Rahman, Canada Muhammad B. Yunus, USA Kenneth C. Kalunian, USA Morris Reichlin, USA Contents Vascular Disease in Systemic Sclerosis, Lorinda Chung, Oliver Distler, Laura Hummers, Eswar Krishnan, and Virginia Steen Volume 2010, Article ID 714172, 2 pages The Vascular Microenvironment and Systemic Sclerosis, Tracy Frech, Nathan Hatton, Boaz Markewitz, Mary Beth Scholand, Richard Cawthon, Amit Patel, and Allen Sawitzke Volume 2010, Article ID 362868, 6 pages Digital Ischemia in Scleroderma Spectrum of Diseases, Elena Schiopu, Ann J. Impens, and Kristine Phillips Volume 2010, Article ID 923743, 8 pages Digital Ischemic Loss in Systemic Sclerosis, Umaima Marvi and Lorinda Chung Volume 2010, Article ID 130717, 7 pages Lower Extremity Ulcers in Systemic Sclerosis: Features and Response to Therapy, Victoria K. Shanmugam, Patricia Price, Christopher E. Attinger, and Virginia D. Steen Volume 2010, Article ID 747946, 8 pages Registry Evaluation of Digital Ulcers in Systemic Sclerosis, Felice Galluccio and Marco Matucci-Cerinic Volume 2010, Article ID 363679, 5 pages Capillaroscopy as an Outcome Measure for Clinical Trials on the Peripheral Vasculopathy in SScIs It Useful?, Maurizio Cutolo, Alberto Sulli, Carmen Pizzorni, and Vanessa Smith Volume 2010, Article ID 784947, 6 pages Interleukin-6 as a Potential Therapeutic Target for Pulmonary Arterial Hypertension, Yoshiaki Furuya, Toru Satoh, and Masataka Kuwana Volume 2010, Article ID 720305, 8 pages Serum Endoglin Levels in Patients Suffering from Systemic Sclerosis and Elevated Systolic Pulmonary Arterial Pressure, Paola Ximena Coral-Alvarado, Maria Fernanda Garces, Jorge Eduardo Caminos, Antonio Iglesias-Gamarra, JoseF´ elix´ Restrepo, and Gerardo Quintana Volume 2010, Article ID 969383, 6 pages Characteristics of Interstitial Fibrosis and Inflammatory Cell Infiltration in Right Ventricles of Systemic Sclerosis-Associated Pulmonary Arterial Hypertension,MariaJ.Overbeek, Koen T. B. Mouchaers, Hans M. Niessen, Awal M. Hadi, Koba Kupreishvili, Anco Boonstra, Alexandre E. Voskuyl, Jeroen A. M. Belien, Egbert F. Smit, Ben C. Dijkmans, Anton Vonk-Noordegraaf, and Katrien Grunberg¨ Volume 2010, Article ID 604615, 10 pages Identification of Myocardial Damage in Systemic Sclerosis: A Nuclear Cardiology Approach, Kenichi Nakajima, Shinro Matsuo, Minoru Hasegawa, Seigo Kinuya, and Kazuhiko Takehara Volume 2010, Article ID 496509, 9 pages Renal Manifestations in Scleroderma: Evidence for Subclinical Renal Disease as a Marker of Vasculopathy, Victoria K. Shanmugam and Virginia D. Steen Volume 2010, Article ID 538589, 8 pages Scleroderma Renal Crisis: A Pathology Perspective, Ibrahim Batal, Robyn T. Domsic, Thomas A. Medsger Jr., and Sheldon Bastacky Volume 2010, Article ID 543704, 7 pages An International, Web-Based, Prospective Cohort Study to Determine Whether the Use of ACE Inhibitors prior to the Onset of Scleroderma Renal Crisis Is Associated with Worse Outcomes–Methodology and Preliminary Results, Marie Hudson, Murray Baron, Ernest Lo, Joanna Weinfeld, Daniel E. Furst, and Dinesh Khanna Volume 2010, Article ID 347402, 7 pages Vascular Complications of Systemic Sclerosis during Pregnancy, Eliza F. Chakravarty Volume 2010, Article ID 287248, 5 pages Vascular Alterations and Sexual Function in Systemic Sclerosis, Ann Julie Impens and James R. Seibold Volume 2010, Article ID 139020, 5 pages Penile involvement in Systemic Sclerosis: New Diagnostic and Therapeutic Aspects, Antonio Aversa, Roberto Bruzziches, Davide Francomano, Edoardo Rosato, Felice Salsano, and Giovanni Spera Volume 2010, Article ID 708067, 5 pages Microscopic Polyangiitis in Systemic Sclerosis, Hiroshi Hashimoto Volume 2010, Article ID 148528, 6 pages Vasculitis in Systemic Sclerosis, Lily Kao and Cornelia Weyand Volume 2010, Article ID 385938, 9 pages Hindawi Publishing Corporation International Journal of Rheumatology Volume 2010, Article ID 714172, 2 pages doi:10.1155/2010/714172 Editorial Vascular Disease in Systemic Sclerosis Lorinda Chung,1 Oliver Distler,2 Laura Hummers,3 Eswar Krishnan,4 and Virginia Steen5 1 Division of Immunology and Rheumatology, Stanford University School of Medicine, Palo Alto VA Health Care System, 3801 Miranda Avenue, Palo Alto, CA 94305, USA 2 Department of Rheumatology, University Hospital Zurich, Gloriastr. 25, 8091 Zurich,¨ Switzerland 3 Division of Rheumatology, Johns Hopkins University, 5501 Hopkins Bayview Circle, Room 1B.7, Baltimore, MD 21224, USA 4 Division of Immunology and Rheumatology, Stanford University School of Medicine, 1000 Welch Road, Suite 203, Palo Alto, CA 94305, USA 5 Division of Rheumatology, Georgetown University, 3800 Reservoir Road NW, Pasquerilla Healthcare Center, Suite 3004, Washington, DC 20007, USA Correspondence should be addressed to Lorinda Chung, [email protected] Received 4 October 2010; Accepted 10 October 2010 Copyright © 2010 Lorinda Chung et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Systemic sclerosis (SSc) is an autoimmune disease character- utility of registries in understanding the natural history ized by widespread fibrosis affecting the skin, internal organs, of digital ulcers and discusses the need for classification and vasculature. Vascular disease with intimal proliferation criteria for the assessment of digital ulcers. This is followed and obliterative vasculopathy is extremely prevalent in SSc, by a review article discussing the potential use of nailfold most commonly manifesting as Raynaud’s phenomenon and videocapillaroscopy as an outcome measure in clinical trials digital ulcerations. Other manifestations of vascular disease for patients with SSc and vascular complications, particularly occur less frequently in patients with SSc, including ischemic digital ulcers and PAH. digital loss, pulmonary arterial hypertension (PAH), and The next section of the special issue relates to cardiopul- renal crisis. Vascular disease in SSc may also commonly affect monary vascular complications in SSc, with a particular pregnancy outcomes and sexual function. Finally, patients focus on PAH and right ventricular failure. The first paper in with SSc can rarely suffer from inflammatory vasculitis, and this section describes a case report of a patient with mixed recognition of this has a significant impact on therapy. In connective tissue disease and severe, refractory PAH who this special issue on vascular disease in systemic sclerosis, we experienced dramatic improvement in functional ability and invited original research articles and review articles regarding hemodynamics in response to treatment with tocilizumab, a the pathogenesis, epidemiology, natural history, evaluation, humanized monoclonal antibody to the human interleukin- and/or management of vascular complications in patients 6 receptor. This is followed by an original research article with SSc. evaluating the relationship of serum endoglin levels in The first paper of this special issue describes the vascular patients with and without elevated systolic pulmonary microenvironment that likely contributes to the pathogenesis arterial pressures (sPAP) on echocardiography. This study of vasculopathy in SSc. This is followed by two papers of the found that SSc patients with and without elevated sPAP current knowledge regarding the pathogenesis, evaluation, had much higher levels of serum endoglin compared and management of digital ulcers and digital ischemic loss in with healthy controls, suggesting that endoglin may be a patients with SSc. The fourth paper of the special issue is an potential biomarker of vasculopathy that is not specific original research article that describes the prevalence of and to the pulmonary vasculature. The next paper describes risk factors for nondigital lower extremity ulcers in SSc. The a histopathogic comparison of samples from the right authors found that antiphospholipid antibodies and genetic ventricle of patients with SSc-associated PAH and idiopathic prothrombotic mutations are highly prevalent in