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Open Access Research article

Long-term effects of intravenous iloprost in patients with idiopathic pulmonary arterial hypertension deteriorating on non-parenteral therapy

Lars Knudsen1*, Alexander Schurawlew1, Nils Nickel1, Henning Tiede2, Hossein A Ghofrani2, Heinrike Wilkens3, Ralf Ewert4, Michael Halank5, Hans Klose6, Carlos Bäzner7, Jürgen Behr78 and Marius M Hoeper1

Author Affiliations

1 Department of Respiratory Medicine, Hannover Medical School, Hanover, Germany

2 Medical Clinic II/V, University Hospital Giessen and Marburg, Justus-Liebig University, Giessen, Germany

3 Department of Pneumology, University Hospital Homburg, Homburg/Saar, Germany

4 Department of Cardiology and Respiratory Medicine, University Hospital Greifswald, Greifswald, Germany

5 Department of Internal Medicine I, Carl-Gustav-Carus University, Dresden, Germany

6 Department of Pneumology, University Hospital Hamburg-Eppendorf, Hamburg, Germany

7 Division of Pulmonary Diseases, Department of Internal Medicine I, Ludwig Maximilans University, Klinikum Grosshadern, Munich, Germany

8 Department of Internal Medicine III, Pneumology, University Hospital Bergmannsheil, Bochum, Germany

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BMC Pulmonary Medicine 2011, 11:56  doi:10.1186/1471-2466-11-56

Published: 1 December 2011

Abstract

Background

The majority of patients with idiopathic pulmonary arterial hypertension (IPAH) in functional classes II and III are currently being treated with non-parenteral therapies, including endothelin receptor antagonists (ERA), phosphodiesterase (PDE)-5 inhibitors, inhaled iloprost or combinations of these substances. If these treatments fail, current guidelines recommend the addition of parenteral prostanoid therapy. There is, however, limited evidence for the efficacy of parenteral prostanoids when added to combinations of non-parenteral therapies.

Methods

In this retrospective, multicentre study we collected data from consecutive IPAH patients receiving intravenous iloprost in addition to optimized non-parenteral therapy between Jan 2002 and Dec 2009. Analyses included 6 min walk distance (6MWD), functional class, need for transplantation, and survival.

Results

During the observation period, 50 patients were treated with intravenous iloprost in addition to non-parenteral therapy; 44% of the patients were on dual combination therapy and 52% on triple combination. Three months after initiation of iloprost, functional class had improved in 24% of the patients and the median 6MWD had increased from 289 m to 298 m (n.s.). During the observation period, 22 patients (44%) died and 14 (28%) underwent lung transplantation. The probabilities of LuTx-free survival at 1, 3 and 5 years following iloprost initiation were 38%, 17% and 17%, respectively. A 6MWD < 300 m and persistent functional class IV at 3 months after initiation of intravenous iloprost were predictors of an adverse outcome.

Conclusion

In essence, late initiation of intravenous iloprost in IPAH patients who previously failed to respond to non-parenteral therapies appears to be of limited efficacy in the majority patients. Alternative therapeutic options are currently not available, underlying the need for the development of new drugs.