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Preface
Scleroderma and Related Disorders
Anupam Wakhlu
Durga Prasanna Misra
Vikas Agarwal
Guest Editors Scleroderma, also called systemic sclerosis, is a rare disease predominantly affecting young females, associated with significant morbidity and mortality due to the involvement of major organs such as the lungs and the kidney. The major pathogenic feature of scleroderma is excessive collagen deposition and fibrosis in the skin and other internal organs of the body. Lack of potent antifibrotic therapy is a major hindrance towards disease modifying therapy in this condition, with most of the therapy being directed towards relief of symptoms such as Raynaud’s phenomenon and gastro-esophageal reflux. This disease is also associated with increased risk of cardiovascular events due to accelerated atherogenesis. In this supplement of the Indian Journal of Rheumatology on “Scleroderma and related disorders”, we bring forth a collection of review articles written by experts in this field, both national and international, discussing recent advances in the diagnosis, pathogenesis and treatment of various clinical features of scleroderma. Gupta and colleagues have lucidly discussed the pathogenesis of scleroderma starting from endothelial injury finally culminating in fibrosis. Khanna and colleagues have elucidated on pulmonary hypertension in this condition whereas Makol and colleagues have reviewed interstitial lung disease in scleroderma. Two separate reviews by Larche et al and Kedia et al discuss gastrointestinal involvement in scleroderma. Mimics of scleroderma have been discussed separately by Shenoy and colleagues, Ghosh and colleagues and Jyoti Parida. Wakhlu and colleagues have shared a unique presentation of epidemic dropsy (adulteration of mustard oil, a peculiar problem in some parts of India) mimicking scleroderma. Misra et al discuss the emerging antifibrotic role of phosphodiesterase inhibitors whereas Negi et al has presented the current perspectives on the emerging role of hematopoietic stem cell transplant in the management of scleroderma. Dimitroulas et al have provided an excellent overview of cardiac involvement and cardiovascular risk in scleroderma. Lastly, there are not many biomarkers to assess disease activity or damage S141
in scleroderma, Sukesh and colleagues have presented the current scenario and utility of presently available and emerging biomarkers in scleroderma. We hope that this supplement shall serve to enhance the knowledge of the practicing Rheumatologist and well as motivate youngsters to undertake further research in this very important but somewhat less cared for area in the field of Rheumatology.
Financial support and sponsorship Nil.
Conflicts of interest There are no conflicts of interest.
Anupam Wakhlu, Durga Prasanna Misra1, Vikas Agarwal1 Department of Rheumatology, King George Medical University, Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
1
Address for correspondence: Prof. Vikas Agarwal, Department of Clinical Immunology, SGPGIMS, Lucknow, India E‑mail:
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DOI: 10.4103/0973-3698.219079
How to cite this article: Wakhlu A, Misra DP, Agarwal V. Scleroderma and related disorders. Indian J Rheumatol 2017;12:S141.
© 2017 Indian Journal of Rheumatology | Published by Wolters Kluwer - Medknow
