Misra DP, Chowdhury AC, Phatak S, Agarwal V. Scleroderma: Not an orphan disease any more. World J Rheumatol 2015; 5(3): 131-141 [DOI: 10.5499/wjr.v5.i3.131]
Corresponding Author of This Article
Dr. Vikas Agarwal, Additional Professor, Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow 226014, India. vikasagr@yahoo.com
Research Domain of This Article
Rheumatology
Article-Type of This Article
Review
Open-Access Policy of This Article
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/
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
Abstract
Scleroderma (or systemic sclerosis) is a rare disease associated with significant morbidity and mortality. Although previously thought to have a uniformly poor prognosis, the outlook has changed in recent years. We review recent insights into the pathogenesis, clinical features, assessment and management of scleroderma.
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.
