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.

Using transitional changes on HRCT to monitor the impact of cyclophosphamide or mycophenolate on systemic sclerosis-related interstitial lung disease (SSc-ILD). Significantly favorable transitions from both ground glass and lung fibrosis ILD patterns to normal lung were found in patients undergoing immunosuppressive treatment for Ssc-ILD. PubMed, Arthritis Rheumatol, 08/20/2019. (Also see Cellcept)

Long–term effects of immunosuppressive therapy on lung function in scleroderma patients. Cyclophosphamide does not appear to result in clinically significant improvement of pulmonary function but fulfilled criteria of stable disease. PubMed, Clin Rheumatol, 2018 Nov;37(11):3043-3050. (Also see Immunosuppressants)

Cyclophosphamide for connective tissue disease–associated interstitial lung disease. Researchers may consider comparing cyclophosphamide versus antifibrotic agents, or comparing both versus placebo, in particular, for those with evidence of rapidly progressive fibrotic disease, who may benefit the most. PubMed, Cochrane Database Syst Rev, 01/03/2018. (Also see Cyclophosphamide)

Oral Cyclophosphamide

Cough is a common symptom in scleroderma interstitial lung disease and correlates with the extent of fibrosis. (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

Prediction of therapeutic response before and during i.v. cyclophosphamide (IVCY) pulse therapy for interstitial lung disease in systemic sclerosis (SSc–ILD): A longitudinal observational study. ILD severity/activity before treatment and variability of serum Krebs von den Lungen-6, surfactant protein D levels during treatment may be useful to predict therapeutic effects of IVCY on SSc–ILD. PubMed, J Dermatol, 10/05/2018.

Long-term follow-up of patients with scleroderma interstitial lung disease (SSc-ILD) treated with intravenous cyclophosphamide(CYC) pulse therapy: a single–center experience. In patients with ILD-SSc, CYC stabilized the reduction of FVC during treatment, but this effect was not persistent. The vascular characteristic of ILD-SSc (DLCO) was not affected by CYC treatment. PubMed, Isr Med Assoc J, 2015 Mar;17(3):150-6.

Low-dose pulse cyclophosphamide (CYC) in interstitial lung disease associated with systemic sclerosis (SSc-ILD): efficacy of maintenance immunosuppression in responders and non-responders. Our study supports the use of low-dose pulse CYC as induction therapy of recently deteriorated SSc-ILD. PubMed, Semin Arthritis Rheum, 2015 Feb;44(4):437-44.

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