Therapeutic strategy combining intravenous cyclophosphamide followed by oral azathioprine to treat worsening interstitial lung disease associated with systemic sclerosis: a retrospective multicenter open-label study

Alice Bérezné, Brigitte Ranque, Dominique Valeyre, Michel Brauner, Yannick Allanore, David Launay, Véronique Le Guern, Jean-Emmanuel Kahn, Louis-Jean Couderc, Joël Constans, Pascal Cohen, Alfred Mahr, Christian Pagnoux, Eric Hachulla, André Kahan, Jean Cabane, Loïc Guillevin, Luc Mouthon
Journal of Rheumatology 2008, 35 (6): 1064-72

OBJECTIVE: To evaluate the effects and safety of 6-month intravenous cyclophosphamide (CYC) followed by 18-month oral azathioprine (AZA) therapy in patients with systemic sclerosis (SSc) and worsening interstitial lung disease (ILD).

METHODS: All patients presented with ILD and worsened forced vital capacity (FVC) and/or total lung capacity of more than 10% and/or DLCO of more than 15% during the previous year. Treatment was 6 monthly pulses of 0.6 g/m(2) CYC followed by oral AZA for 18 months on disease stabilization or improvement. The endpoint was the rate of percentage change in pulmonary function tests (PFT) after 6 and 24 months.

RESULTS: Twenty-seven patients with SSc (20 females) were recruited. Age and disease duration before CYC therapy were (mean +/- SD) 49.4 +/- 15 years and 75.5 +/- 87.8 months, respectively. Mean baseline FVC was 67% +/- 19% of predicted value. At 6 months, in 7 (26%) patients disease was improved, in 12 (44%) stabilized, and in 8 (30%) worsened. Among the 19 (70%) responders, 15 received AZA and 4 declined. Twenty-three completed 2-year followup, 3 died, and one dropped out. Six (22.2%) had improved, 8 (29.6.%) were stable, and 13 (48.2%) had worsened. Evolution of the slope of FVC (in % per year) varied from -15.5 prior to treatment to +3 (p = 0.004) at 6 months and to +1 (p < 5 x 10(-5)) at 24 months.

CONCLUSION: Intravenous CYC followed by oral maintenance immunosuppressive therapy for worsening ILD was well tolerated and was associated with stable or improved PFT in 70% and 51.8% of SSc patients at 6 months and 2 years, respectively.

Full Text Links

Find Full Text Links for this Article


You are not logged in. Sign Up or Log In to join the discussion.

Related Papers

Remove bar
Read by QxMD icon Read

Save your favorite articles in one place with a free QxMD account.


Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"