Improvement of endothelial function in parallel with the amelioration of dry cough and dyspnea due to interstitial pneumonia by intravenous cyclophosphamide pulse therapy in patients with systemic sclerosis: a preliminary report of two cases

Intravenous cyclophosphamide pulse therapy (IVCY) exerts its efficacy against interstitial lung disease (ILD) associated with systemic sclerosis (SSc) by restoring vascular injuries as well as aberrant immune activation. We recently experienced two patients with SSc-ILD in whom the values of brachial flow-mediated dilation (FMD) reflected the efficacy of IVCY. We herein report the details of these cases and discuss the potential of FMD to predict and evaluate the effect of IVCY on SSc-ILD.

[1]  W E Haefeli,et al.  Nitric oxide is responsible for flow-dependent dilatation of human peripheral conduit arteries in vivo. , 1995, Circulation.

[2]  P. Emery,et al.  A multicenter, prospective, randomized, double-blind, placebo-controlled trial of corticosteroids and intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma. , 2006, Arthritis and rheumatism.

[3]  A. Sasseville,et al.  Novel Fibrogenic Pathways Are Activated in Response to Endothelial Apoptosis: Implications in the Pathophysiology of Systemic Sclerosis1 , 2005, The Journal of Immunology.

[4]  N. Sepp,et al.  Endothelial cell apoptosis in systemic sclerosis is induced by antibody-dependent cell-mediated cytotoxicity via CD95. , 2000, Arthritis and rheumatism.

[5]  T. Medsger,et al.  Changes in causes of death in systemic sclerosis, 1972–2002 , 2007, Annals of the rheumatic diseases.

[6]  E. Leroy.  SYSTEMIC SCLEROSIS : A Vascular Perspective , 1996 .

[7]  M. Gershwin,et al.  Endothelial cell apoptosis is a primary pathogenetic event underlying skin lesions in avian and human scleroderma. , 1996, The Journal of clinical investigation.

[8]  L. Mouthon,et al.  Therapeutic options for systemic sclerosis related interstitial lung diseases. , 2010, Respiratory medicine.

[9]  A. Herrick,et al.  Vascular function in systemic sclerosis , 2000, Current opinion in rheumatology.

[10]  S. Jimenez,et al.  Systemic sclerosis: current views of its pathogenesis. , 2003, Autoimmunity reviews.

[11]  L. Mouthon,et al.  Interstitial lung disease in systemic sclerosis. , 2011, Autoimmunity reviews.

[12]  J. Piette,et al.  The binding of some human antiendothelial cell antibodies induces endothelial cell apoptosis. , 1998, The Journal of clinical investigation.

[13]  Charlie Strange,et al.  Cyclophosphamide versus placebo in scleroderma lung disease. , 2006, The New England journal of medicine.

[14]  J. K. Lloyd,et al.  Non-invasive detection of endothelial dysfunction in children and adults at risk of atherosclerosis , 1992, The Lancet.

[15]  Shinichi Sato,et al.  Mobilization of endothelial progenitor cells by intravenous cyclophosphamide in patients with systemic sclerosis. , 2010, Rheumatology.

[16]  M. Gershwin,et al.  In vivo analysis of the apoptosis-inducing effect of anti-endothelial cell antibodies in systemic sclerosis by the chorionallantoic membrane assay. , 2003, Arthritis and rheumatism.

[17]  I. Haznedaroglu,et al.  Effects of oral cyclophosphamide and prednisolone therapy on the endothelial functions and clinical findings in patients with early diffuse systemic sclerosis. , 2003, Arthritis and rheumatism.

[18]  M. Cutolo,et al.  Brachial Artery Endothelial-dependent Flow-mediated Dilation Identifies Early-stage Endothelial Dysfunction in Systemic Sclerosis and Correlates with Nailfold Microvascular Impairment , 2010, The Journal of Rheumatology.

[19]  D. Knight,et al.  The Assessment of Anti-Endothelial Cell Antibodies in Scleroderma-Associated Pulmonary Fibrosis A Study of Indirect Immunofluorescent and Western Blot Analysis in 49 Patients With Scleroderma , 2003 .

[20]  E. Parra,et al.  Collagen V and vascular injury promote lung architectural changes in systemic sclerosis , 2009, The clinical respiratory journal.

[21]  J. Tervaert,et al.  Anti-endothelial cell antibodies in systemic sclerosis , 2010, Annals of the rheumatic diseases.

[22]  F. Luppi,et al.  Pulse cyclophosphamide decreases sympathetic postganglionic activity, controls alveolitis, and normalizes vascular tone dysfunction (Raynaud's phenomenon) in a case of early systemic sclerosis. , 2004, Arthritis and rheumatism.

[23]  A. Volpe,et al.  Cyclophosphamide treatment improves microvessel damage in systemic sclerosis , 2009, Clinical Rheumatology.

[24]  I. Chikanza,et al.  Mechanisms of Disease: the role of immune cells in the pathogenesis of systemic sclerosis , 2006, Nature Clinical Practice Rheumatology.

[25]  C. Stefanadis,et al.  Improvement of vascular endothelial function using the oral endothelin receptor antagonist bosentan in patients with systemic sclerosis. , 2007, Arthritis and rheumatism.