Treatments for Pulmonary Fibrosis: Oral and IV Cyclophosphamide

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Oral Cyclophosphamide
IV Cyclophosphamide


Cyclophosphamide was also formerly known as the brand name Cytoxan.

Cyclophsophamide is given either intravenously (IV) or orally for pulmonary fibrosis. Studies for both treatments have been completed and some studies are still on-going. Each treatment has its advantages and disadvanatges to the patient. As with all treatments for scleroderma, patients respond differently to oral and IV cyclophosphamide treatments.

Cyclophosphamide in systemic sclerosis: still in search of a 'real life' scenario. In systemic sclerosis (SSc), there is no proven treatment to prevent disease progression. In a recent meta-analysis of three randomised controlled trials (RCTs) and six open prospective studies on cyclophosphamide (CYC), no significant changes in lung function were observed. Further RCTs on early SSc are needed to assess the real efficacy of CYC in inducing remission and increasing survival. I. Miniati. Arthritis Research & Therapy 23 January 2009 (Also see Cyclophosphamide)

Oral Cyclophosphamide

Correlation of Cough With Disease Activity and Treatment With Cyclophosphamide in Scleroderma Interstitial Lung Disease (SSc-ILD). Findings from the Scleroderma Lung Study. Cough is a common symptom in SSc-ILD and correlates with the extent of fibrosis. It may be a symptom of ongoing fibrosis. CHEST Journal, Vol 142, No 3, September 2012. (Also see Lung Involvement)

Still recruiting, as of 10/15/12.

Comparison of Therapeutic Regimens for Scleroderma Interstitial Lung Disease (The Scleroderma Lung Study II) (SLSII). The purpose of this study is to determine whether people with symptomatic scleroderma-related interstitial lung disease experience more respiratory benefits from treatment with a 2-year course of mycophenolate mofetil or treatment with a 1-year course of oral cyclophosphamide.

IV Cyclophosphamide

Efficacy and safety of intravenous cyclophosphamide pulse therapy with oral prednisolone in the treatment of interstitial lung disease (ILD) with systemic sclerosis: 4-year follow-up This long observational study for 48 months showed the efficacy of intravenous cyclophosphamide (IVCY) pulse therapy with prednisolone (PSL) for active alveolitis in the first year. However, because five patients had recurrence of ILD more than 1 year after the treatment, it would be necessary to consider maintenance therapy for ILD beyond 1 year. Modern Rheumatology, 15 January 2011.

Effects of micronutrient antioxidants (alpha-tocopherol and ascorbic acid) on skin thickening and lung function in patients with early diffuse systemic sclerosis. After 6 months of therapy, patients treated with CyP (intravenous cyclophosphamide) and antioxidants had a significantly lower STPR (skin thickening progression rate), compared to patients treated with CyP only. Lung function parameters remained stable in both subgroups. However, lung diffusing capacity improved slightly, without statistical significance, in patients treated with CyP and antioxidants, and it deteriorated in patients without antioxidants. Predrag Ostojic. (SpringerLink) Rheumatology International. 23 March 2010. (Also see Antioxidants)

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