466, Sector 28, Noida, UP - 201 303
|How to cite this article:
Sardana K, Garg V K. Authors' reply. Indian J Dermatol Venereol Leprol 2009;75:511-513
- Firstly we focused on newer approaches in scleroderma. Dexamethasone-cyclophosphamide pulse (DCP) therapy is by no means a new approach.
- The evidence against use of steroids is overwhelming. The mountain of evidence from textbooks, and guidelines of medicine, rheumatolology and dermatology detailing the evidence against the use of steroids except for alveolitis, mycocarditis and sometimes for renal involvement ,,,,,,,,,,,,,,,,,, [Table - 1].
- Steroids have multitude of side effects which add to the already multisystem damage of scleroderma. ,,
- Skin improvement, which is a tool observed by most Indian case reports, is the most nonspecific tool to monitor improvement.Steroids per se have no role to play in the skin pathology of progressive systemic sclerosis (PSS). ,,,, Glucocorticoids are not effective in improving or preventing skin induration and the progression of systemic sclerosis (SSc; also known as scleroderma). 
- The most crucial aspects is that evidence-based double blinded trial has never shown steroids to be disease modifying [Table - 1]. ,,,,,,,,,,,,,,,,,,
- To complicate the matter, the disease has a well known spontaneous resolution and the trial has to be factored in any reported trial that shows results. ,,,, In other words, a disease in the resolving phase will show a false response to any drug.
- The evidence for cyclophosphamide is there, but as yet the results of the largest multicentric, multiregional (SCOT) trial is awaited and only after that we can comment on the therapeutic role of cyclophosphamide. ,,,,,,
Secondly, the indexed literature does not contain references 4, 6, and 8, referred by the author.
And case reports are not in any way considered as evidence even in the Cochrane registry of controlled trials, and most of the data reported by the author do not meet the standards of the Cochrane guidelines.
Lastly, the author has excluded two articles reporting the side-effects, which have been reported from India. , This highlights the risks involved in the indiscriminate use of this form of therapy.
In view of the huge data from scientific journals, specialty books and international guidelines, ,,,,,,,,,,,,,,, the obstinate persistence of DCP pulse in scleroderma is purely a individual perception which is beyond scientific purview as its role has not been mentioned in any evidence-based data.
The summary guidelines on steroids gleaned from the wealth of data are given below [Table - 2].
Pasricha JS. Systemic sclerosis and dexamethasone cyclophosphamide pulse therapy. Indian J Dermatol Venereol Leprol 2009;75:510.[Google Scholar]
Gupta R. Dexamethasome-cyclophosphamide pulse therapy for systemic sclerosis. Indian J Dermatol Venereol Leprol 2009;75:511.[Google Scholar]
Sardana K, Garg VK. Therapeutic trials for systemic sclerosis: An update. Indian J Dermatol Venereol Leprol 2008;74:436-45.[Google Scholar]
Sapadin AN, Fleischmajer R. Treatment of scleroderma. Arch Dermatol 2002;138:99-105.[Google Scholar]
Denton PC. Therapeutic targets in systemic sclerosis. Arthritis Res Ther. 2007;9:S10.[Google Scholar]
Rubin LJ, Black CM, Denton CP, Seibold JR . New therapeutic strategies for systemic sclerosis--a critical analysis of the literature. Clin Dev Immunol. 2005;12:165-73.[Google Scholar]
Charles C, Clements P, Furst DE. Systemic sclerosis: hypothesis-driven treatment strategies. Lancet 2006;367:1683-91.[Google Scholar]
Matucci-Cerinic M, Steen VD, Furst DE, Seibold JR. Clinical trials in systemic sclerosis: lessons learned and outcomes. Arthritis Res Ther 2007;9:S7. Review[Google Scholar]
Ruddy S, Harris ED, Sledge CB, Sergent JS, Budd RC. Kelley's Textbook of Internal Medicine, 6 TH E-edition, Philadelphia: W.B. Saunders Company; 2006. p. 1326.TH E-edition, Philadelphia: W.B. Saunders Company; 2006. p. 1326.'>[Google Scholar]
Austen KF, Frank MM., Atkinson JP, Harvey C. Samter's immunologic diseases, 6 th ed. Philadelphia: Lippincott Williams and Wilkins; 2001. p. 1264.th ed. Philadelphia: Lippincott Williams and Wilkins; 2001. p. 1264.'>[Google Scholar]
Dale DC, Federman DD. ACP Medicine, WebMD,2006. p. 2754-6.[Google Scholar]
Hoyles RK, Ellis RW, Wellsbury J, Lees B, Newlands P, Goh NS, et al. A multicenter, prospective, randomized, double-blind, placebo-controlled trial of corticosteroids and intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma Arthritis Rheum 2006;54:3962-70.[Google Scholar]
Vanthuyne M, Blockmans D, Westhovens R, Roufosse F, Cogan E, Coche E, et al. A pilot study of mycophenolate mofetil combined to intravenous methylprednisolone pulses and oral low-dose glucocorticoids in severe early systemic sclerosis. Clin Exp Rheumatol 2007;25:287-92.[Google Scholar]
Gilliland BC. Systemic Sclerosis (Scleroderma) and Related Disorders. In: Kasper DI, Braunwald E, Fauci AS, Hauser SI, editors, Harrison's principles of internal medicine, 16 th edition, New York: McGraw-Hill; 2005. p. 1979-99.th edition, New York: McGraw-Hill; 2005. p. 1979-99. '>[Google Scholar]
Nihtyanova SI, Denton CP. Current approaches to the management of early active diffuse scleroderma skin disease. Rheum Dis Clin North Am 2008;34:161-79; 8.[Google Scholar]
Herrick A. Diagnosis and management of scleroderma peripheral vascular disease. Rheum Dis Clin North Am 2008;34:89-114.[Google Scholar]
Rubin LJ. Treatment of pulmonary arterial hypertension due to scleroderma: challenges for the future. Rheum Dis Clin North Am 2008;34:191-7.[Google Scholar]
Distler J, Distler O. Novel treatment approaches to fibrosis in scleroderma. Rheum Dis Clin North Am 2008;34:145-59. .[Google Scholar]
Pasricha JS, Ramam M, Shah S. Reversal of systemic sclerosis with dexamethasone pulses. Indian J Dermatol Venereol Leprol 1990;56:40-2.[Google Scholar]
Saoji VA. Premature ovarian failure due to cyclophosphamide: a report of four cases in dermatology practice. Indian J Dermatol Venereol Leprol 2008;74:128-32.[Google Scholar]
Ahmad QM, Shah IH, Nauman Q, Sameem F, Sultan J. Increased incidence of tuberculosis in patients of systemic sclerosis on dexamethasone pulse therapy: A short communication from Kashmir. Indian J Dermatol 2008;53:24-5.[Google Scholar]
Maddison PJ, Isenberg DA, Woo P, Glass DN, Breedveld F. Oxford Textbook of Rheumatology, 3 rd Edition, USA: Oxford University Press, Section 6. Subsection 2004. p. 76.7.[Google Scholar]