Background Scleroderma is an autoimmune disease characterized mainly by skin manifestations and various visceral organs involvement especially lungs. Lung involvement is the leading cause of mortality in this patients.Various studies have demonstrated efficacy of cyclophosphamide (CYC) and mycophenolate mofetil (MMF) in SSc-ILD but no head to head comparative data is available till date.
Objectives To compare efficacy of CYC & MMF in patients with SSc-ILD in terms of improvement in lung function.
Methods Patients more than 18 years of age fulfilling ACR 2012 classification criteria for Systemic sclerosis with significant ILD as defined by Forced vital capacity (FVC) ≤80 and Presence of ILD on thoracic high resolution computerized tomography were included.MMF group received upto 3gm daily and CYC group received 600mg/m2 (Max 1.2 gm) for total duration of 6 months.Monthly clinical assessment and 3 monthly lung function (spirometry-FVC) were done for 6 months.Response was evaluated as per American Thoracic Society recommendations (improvement when increase in FVC ≥10%, stabilization when change in FVC <10% and worse reduction in FVC was ≥10%).Appropriate statistical tests were applied to analyse the data.
Results 23 patients (18 female/5 male) in CYC and 44 patients (40 female/4 male) in MMF group were included with mean age of 46±10.34 years (mean disease duration of 6.04±5.96 years) and 45.54± 13.74 years (mean disease duration of 4.84±4.08 years) in respective groups.After 6 months of induction therapy,increase in mean percentage of FVC observed was 10.84±13.81% (p=0.003) in CYC and 6.07±11.92% (p=0.006) in MMF group.When the change in FVC at 6 months in both groups were compared to each other,no significance difference was found (p=0.373).
Conclusions We support the use of mycophenolate for the patients for whom CYC is contraindicated or not tolerated, given the substantial toxicities and modest benefit of CYC as alternative.A prospective multicenter controlled trial of mycophenolate in SSc-ILD patients is warranted to support the above data.
Ferri C,Valentini G,Cozzi F et al.Systemic sclerosis:demographic,clinical,and serologic features and survival in 1,012 Italian patients. Medicine (Baltimore). 2002 Mar; 81(2):139–53
Tashkin DP, Elashoff R, Clements PJ et al. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med. 2006 Jun 22;354(25):2655–66.
Nihtyanova SI, Brough GM, Black CM et al.Mycophenolate mofetil in diffuse cutaneous systemic sclerosis – a retrospective analysis. Rheumatology (Oxford). 2007 Mar;46(3):442–5.
Tzouvelekis A, Galanopoulos N, Bouros E et al Effect and safety of mycophenolate mofetil or sodium in systemic sclerosis-associated interstitial lung disease: a meta-analysis. Pulm Med. 2012;2012:143637. doi: 10.1155/2012/143637.
Collard H, Loyd J, King T et al.Current Diagnosis and Management of Idiopathic Pulmonary Fibrosis: A Survey of Academic Physicians Respir Med. 2007 Sep; 101(9): 2011–2016.
Acknowledgement The authors are thankful to Ms Prasanna (PFT-Spirometry technician) Pulmonology department and Mr Unnikrishnan (Statistician)- Amrita institute of medical science, Kochi for their valuable contribution in performing Spirometry and data calculations respectively.
Disclosure of Interest None declared
Statistics from Altmetric.com
If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.