What's new in the other general journalsBMJ 2006; 333 doi: https://doi.org/10.1136/bmj.333.7557.37 (Published 29 June 2006) Cite this as: BMJ 2006;333:37
All rapid responses
Tashkin and colleagues (1) showed a significant but modest beneficial
effect on lung function and dyspnea in patients with symptomatic
scleroderma-related interstitial lung disease (ILD) after one year of
cyclophosphamide. Although they stated that pulmonary hypertension (PH) is
a leading cause of death in such patients, "clinically significant" PH (p.
2656; "Severe pulmonary hypertension requiring specific treatment" in
Appendix 1) was an exclusion criterion in this trial and PH was not
evaluated as an end point. The relationship between ILD and PH in
scleroderma is largely unknown and considered worthy of study (2, 3).
Recently a role for PH in mortality, independently of ILD, has been
suggested (3). Moreover, it has been shown (albeit in a small
retrospective series) that in patients treated with cyclophosphamide,
while respiratory parameters remained stable, PH may indeed deteriorate
(3). The overall clinical significance of treatment with cyclophosphamide
in the Tashkin's et al. study could have been better appreciated including
1. Tashkin DP, Elashoff R, Clements PJ, et al. Cyclophosphamide versus
Placebo in Scleroderma Lung Disease. N Engl J Med 2006;354:2655-66.
2. Ioannidis JP, Vlachoyanninopoulos PG, Haidich AB et al. Mortality in
systemic sclerosis: an international meta-analysis of individual patient
data. Am J Med 2005;118;2-10.
3. Trad S, Amoura Z, Beigelman C, et al. Pulmonary Arterial Hypertension
is a Major Mortality Factor in Diffuse Systemic Sclerosis, Independent of
Interstitial Lung Disease. Arthritis Rheum 2006:54:184-91.
Competing interests: No competing interests