Sildenafil Improved Pulmonary Hypertension and Peripheral Blood Flow in a Patient with Scleroderma-Associated Lung Fibrosis and the Raynaud Phenomenon
Authors: Stephan Rosenkranz, Frank Diet, Thomas Karasch, Julia Weihrauch, Klaus Wassermann, Erland Erdmann
Journal: Annals of Internal Medicine (2003)

Letters18 November 2003Sildenafil Improved Pulmonary Hypertension and Peripheral Blood Flow in a Patient with Scleroderma-Associated Lung Fibrosis and the Raynaud PhenomenonStephan Rosenkranz, Frank Diet, Thomas Karasch, Julia Weihrauch, Klaus Wassermann, and Erland ErdmannStephan RosenkranzFrom Klinik III fr Innere Medizin and Klinik und Poliklinik fr Dermatologie; Universitt zu Kln., Frank DietFrom Klinik III fr Innere Medizin and Klinik und Poliklinik fr Dermatologie; Universitt zu Kln., Thomas KaraschFrom Klinik III fr Innere Medizin and Klinik und Poliklinik fr Dermatologie; Universitt zu Kln., Julia WeihrauchFrom Klinik III fr Innere Medizin and Klinik und Poliklinik fr Dermatologie; Universitt zu Kln., Klaus WassermannFrom Klinik III fr Innere Medizin and Klinik und Poliklinik fr Dermatologie; Universitt zu Kln., and Erland ErdmannFrom Klinik III fr Innere Medizin and Klinik und Poliklinik fr Dermatologie; Universitt zu Kln.Author, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-139-10-200311180-00030 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail TO THE EDITOR:Background: Systemic scleroderma is a connective tissue disease involving several organ systems. Up to 90% of affected patients have the Raynaud phenomenon, and mortality rates largely depend on involvement of the lungs. In patients without pulmonary abnormalities, the 5-year survival rate is 90%, whereas in patients with systemic sclerodermarelated pulmonary arterial hypertension, median survival is limited to 1 year (1). Since only a few patients with pulmonary arterial hypertension respond to current medical therapy and the possibility of lung transplantation is limited (2), better treatment options are desirable.Objective: Recent studies indicated that phosphodiesterase type V inhibitors ...References1. Koh ET, Lee P, Gladman DD, Abu-Shakra M. Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients. Br J Rheumatol. 1996;35:989-93. [PMID: 8883438] CrossrefMedlineGoogle Scholar2. Gibbs JS, Higenbottam TW, Black C, Corris PA, Haworth SG, McNeil K, . Recommendations on the management of pulmonary hypertension in clinical practice. Heart. 2001;86 Suppl I I1-I13. MedlineGoogle Scholar3. Watanabe H, Ohashi K, Takeuchi K, Yamashita K, Yokoyama T, Tran QK , . Sildenafil for primary and secondary pulmonary hypertension. Clin Pharmacol Ther. 2002;71:398-402. [PMID: 12011826] CrossrefMedlineGoogle Scholar4. Prasad S, Wilkinson J, Gatzoulis MA. Sildenafil in primary pulmonary hypertension [Letter]. N Engl J Med. 2000;343:1342. [PMID: 11183578] CrossrefMedlineGoogle Scholar5. Ghofrani HA, Wiedemann R, Rose F, Olschewski H, Schermuly RT, Weissmann N, . Combination therapy with oral sildenafil and inhaled iloprost for severe pulmonary hypertension. Ann Intern Med. 2002;136:515-22. [PMID: 11926786] LinkGoogle Scholar6. Ghofrani HA, Wiedemann R, Rose F, Schermuly RT, Olschewski H, Weissmann N, . Sildenafil for treatment of lung fibrosis and pulmonary hypertension: a randomised controlled trial. Lancet. 2002;360:895-900. [PMID: 12354470] CrossrefMedlineGoogle Scholar7. Wilkens H, Guth A, Konig J, Forestier N, Cremers B, Hennen B, . Effect of inhaled iloprost plus oral sildenafil in patients with primary pulmonary hypertension. Circulation. 2001;104:1218-22. [PMID: 11551870] CrossrefMedlineGoogle Scholar8. Michelakis E, Tymchak W, Lien D, Webster L, Hashimoto K, Archer S. Oral sildenafil is an effective and specific pulmonary vasodilator in patients with pulmonary arterial hypertension: comparison with inhaled nitric oxide. Circulation. 2002;105:2398-403. [PMID: 12021227] CrossrefMedlineGoogle Scholar9. Nagaya N, Nishikimi T, Okano Y, Uematsu M, Satoh T, Kyotani S, . Plasma brain natriuretic peptide levels increase in proportion to the extent of right ventricular dysfunction in pulmonary hypertension. J Am Coll Cardiol. 1998;31:202-8. [PMID: 9426041] CrossrefMedlineGoogle Scholar10. Nagaya N, Nishikimi T, Uematsu M, Satoh T, Kyotani S, Sakamaki F, . Plasma brain natriuretic peptide as a prognostic indicator in patients with primary pulmonary hypertension. Circulation. 2000;102:865-70. [PMID: 10952954] CrossrefMedlineGoogle Scholar Author, Article, and Disclosure InformationAffiliations: From Klinik III fr Innere Medizin and Klinik und Poliklinik fr Dermatologie; Universitt zu Kln. 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