Review
Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Rheumatol. Nov 12, 2015; 5(3): 131-141
Published online Nov 12, 2015. doi: 10.5499/wjr.v5.i3.131
Scleroderma: Not an orphan disease any more
Durga Prasanna Misra, Abhra Chandra Chowdhury, Sanat Phatak, Vikas Agarwal
Durga Prasanna Misra, Abhra Chandra Chowdhury, Sanat Phatak, Vikas Agarwal, Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226014, India
Author contributions: Misra DP, Chowdhury AC, Phatak S and Agarwal V contributed to the conception and design, acquisition of the data, analysis and interpretation of the data, and final approval of the version to be published; Misra DP, Chowdhury AC and Phatak S drafted the article; Agarwal V critically revised the article for important intellectual content.
Conflict-of-interest statement: Authors do not declare any conflict of interest.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Dr. Vikas Agarwal, Additional Professor, Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow 226014, India. vikasagr@yahoo.com
Telephone: +91-52-22494318 Fax: 91-52-22668812
Received: March 30, 2015
Peer-review started: March 31, 2015
First decision: April 27, 2015
Revised: June 26, 2015
Accepted: July 11, 2015
Article in press: July 14, 2015
Published online: November 12, 2015
Core Tip

Core tip: Scleroderma is a rare disease associated with significant morbidity and mortality. Better understanding regarding its pathogenesis has led to exploration of various newer therapeutic targets. Anti B cell therapy, endothelin receptor antagonists, phosphodiesterase-5 (PDE-5) inhibitors and autologous stem cell transplant holds promise in the management of systemic sclerosis. PDE-5 inhibitors in particular have potential to be disease modifying agents as they not only improve Raynaud’s phenomenon, but also heal digital ulcers, improve pulmonary hypertension and in addition by virtue of antifibrotic properties may have beneficial effect on skin and lung fibrosis.