OBJECTIVE: Pulmonary fibrosis, a frequent manifestation of systemic sclerosis (SSc), is considered incurable. Our aim was to assess the effect of therapy with intravenous (i.v.) cyclophosphamide on the course of pulmonary fibrosis in patients with SSc. METHODS: Five patients with SSc and clinical, laboratory, or radiographic findings of interstitial lung disease were treated with cyclophosphamide (1 g) administered i.v. monthly for 48 weeks. The dyspnea score, pulmonary function tests, arterial blood oxygen content, radiologic abnormalities, and bronchoalveolar lavage (BAL) fluid cellularity were determined before and after therapy. RESULTS: The dyspnea score decreased by 42% after 48 weeks of therapy. Forced vital capacity (FVC, percentage of predicted) increased by 7%, and carbon monoxide diffusing capacity (DLCO) decreased by 12%, but these changes were not statistically significant. Arterial blood oxygenation remained unchanged. At baseline, high resolution computed tomography of the lungs showed honeycombing, reticulonodular, or ground glass patterns in each patient examined. These radiologic abnormalities did not improve during treatment. A marked decrease in BAL fluid cell number, but not in the percentage of neutrophils, was observed after therapy. Nausea and leukopenia were frequent but mild side effects. One patient developed hemorrhagic cystitis. CONCLUSION: The results suggest that intermittent treatment with i.v. cyclophosphamide reduces the severity of dyspnea, but fails to improve FVC or DLCO, or cause resolution of radiologic abnormalities, in patients with SSc.
OBJECTIVE:Pulmonary fibrosis, a frequent manifestation of systemic sclerosis (SSc), is considered incurable. Our aim was to assess the effect of therapy with intravenous (i.v.) cyclophosphamide on the course of pulmonary fibrosis in patients with SSc. METHODS: Five patients with SSc and clinical, laboratory, or radiographic findings of interstitial lung disease were treated with cyclophosphamide (1 g) administered i.v. monthly for 48 weeks. The dyspnea score, pulmonary function tests, arterial blood oxygen content, radiologic abnormalities, and bronchoalveolar lavage (BAL) fluid cellularity were determined before and after therapy. RESULTS: The dyspnea score decreased by 42% after 48 weeks of therapy. Forced vital capacity (FVC, percentage of predicted) increased by 7%, and carbon monoxide diffusing capacity (DLCO) decreased by 12%, but these changes were not statistically significant. Arterial blood oxygenation remained unchanged. At baseline, high resolution computed tomography of the lungs showed honeycombing, reticulonodular, or ground glass patterns in each patient examined. These radiologic abnormalities did not improve during treatment. A marked decrease in BAL fluid cell number, but not in the percentage of neutrophils, was observed after therapy. Nausea and leukopenia were frequent but mild side effects. One patient developed hemorrhagic cystitis. CONCLUSION: The results suggest that intermittent treatment with i.v. cyclophosphamide reduces the severity of dyspnea, but fails to improve FVC or DLCO, or cause resolution of radiologic abnormalities, in patients with SSc.
Authors: D Farge; J Passweg; J M van Laar; Z Marjanovic; C Besenthal; J Finke; H H Peter; F C Breedveld; W E Fibbe; C Black; C Denton; I Koetter; F Locatelli; A Martini; A V N Schattenberg; F van den Hoogen; L van de Putte; F Lanza; R Arnold; P A Bacon; S Bingham; F Ciceri; B Didier; J L Diez-Martin; P Emery; W Feremans; B Hertenstein; F Hiepe; R Luosujärvi; A Leon Lara; A Marmont; A M Martinez; H Pascual Cascon; C Bocelli-Tyndall; E Gluckman; A Gratwohl; A Tyndall Journal: Ann Rheum Dis Date: 2004-08 Impact factor: 19.103
Authors: Donald P Tashkin; Robert Elashoff; Philip J Clements; Michael D Roth; Daniel E Furst; Richard M Silver; Jonathan Goldin; Edgar Arriola; Charlie Strange; Marcy B Bolster; James R Seibold; David J Riley; Vivien M Hsu; John Varga; Dean Schraufnagel; Arthur Theodore; Robert Simms; Robert Wise; Fred Wigley; Barbara White; Virginia Steen; Charles Read; Maureen Mayes; Ed Parsley; Kamal Mubarak; M Kari Connolly; Jeffrey Golden; Mitchell Olman; Barri Fessler; Naomi Rothfield; Mark Metersky; Dinesh Khanna; Ning Li; Gang Li Journal: Am J Respir Crit Care Med Date: 2007-08-23 Impact factor: 21.405