Inhaled Iloprost for Sarcoidosis-associated Pulmonary Hypertension

Inhaled Iloprost for Sarcoidosis Associated Pulmonary Hypertension

This trial will study the treatment of sarcoidosis-associated pulmonary arterial hypertension with inhaled iloprost, a drug approved for primary pulmonary arterial hypertension.

Study Overview

• Status: Completed
• Conditions: Pulmonary Arterial Hypertension; Sarcoidosis
• Intervention / Treatment: Drug: Iloprost

Detailed Description

Pulmonary hypertension has been described in sarcoidosis. It can be a significant problem, not responsive to treatment with anti-inflammatory drugs for the sarcoidosis (1;2). Inhaled iloprost has been approved for treatment of pulmonary hypertension (3). We propose to study the effectiveness of inhaled iloprost for sarcoidosis associated pulmonary hypertension (SAPAH). This is an open label trial, with patients receiving 16 weeks of therapy. Clinical and hemodynamic outcome of therapy will be assessed.

• Study Type: Interventional
• Enrollment (Actual): 20
• Phase: Phase 4

Contacts and Locations

Study Locations

• United States
  • Ohio
    • Cincinnati, Ohio, United States, 45267
      • University of Cincinnati

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years to 90 years (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

Description

Inclusion Criteria:

• Patients with known sarcoidosis 17
• Age 18 or greater
• Patients with documented pulmonary hypertension with a PA mean > 25 mm as measured by cardiac catheterization within six months of entry into the study
• Patients with dyspnea
• Six minute walk distance of between 100 to 500 meters
• Patients on stable immunotherapy for their sarcoidosis, including prednisone, methotrexate, azathioprine, hydroxychloroquine, cyclophosphamide, thalidomide, and/or infliximab
• Patients able to provide written consent

Exclusion Criteria:

• Patients on pulmonary vasodilator drugs (flolan, remodulin, bosentan, sildenafil) in the prior 28 days (patients on stable dose of calcium channel blocker for more than 1 month prior to right heart catheterization can be continued on the calcium channel blocker)
• Patients with severe airway obstruction as defined by FEV1/FVC of less than 35%
• Patients with World Health Organization (WHO) class IV status
• Patients who are pregnant or breast feeding
• Patients with significant left ventricular dysfunction with a left ventricular ejection fraction of less than 35%
• Significant liver dysfunction not due to sarcoidosis
• Patients with severe other organ disease felt by investigators to impact survival during the course of the study
• Patients unable to perform the 6 inhalation treatments required for therapy
• Patients with < 90 mm Hg Systolic systemic blood pressure will be excluded

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Treatment
• Allocation: Non-Randomized
• Interventional Model: Single Group Assignment
• Masking: None (Open Label)

Number of Arms

1

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
Experimental: 1	Drug: Iloprost; Iloprost 2.5-5 mg inhaled via nebulizer up to 6 times a day; Other Names: Ventavis

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Time Frame
Change in six minute walk distance	24 weeks

Secondary Outcome Measures

Outcome Measure	Time Frame
Quality of life	24 weeks
Respiratory function	24 weeks
Toxicity	24 weeks
Pulmonary artery hemodynamics	24 weeks

Collaborators and Investigators

• Sponsor: University of Cincinnati
• Investigators: Principal Investigator: Robert P. Baughman, MD, University of Cincinnati

Publications and helpful links

General Publications

• Baughman RP, Judson MA, Lower EE, Highland K, Kwon S, Craft N, Engel PJ. Inhaled iloprost for sarcoidosis associated pulmonary hypertension. Sarcoidosis Vasc Diffuse Lung Dis. 2009 Jul;26(2):110-20.

Study record dates

Study Major Dates

• Study Start: November 1, 2006
• Primary Completion (Actual): July 1, 2008
• Study Completion (Actual): September 1, 2008

Study Registration Dates

• First Submitted: November 24, 2006
• First Submitted That Met QC Criteria: November 24, 2006
• First Posted (Estimate): November 27, 2006

Study Record Updates

• Last Update Posted (Estimate): April 12, 2013
• Last Update Submitted That Met QC Criteria: April 10, 2013
• Last Verified: April 1, 2013

More Information

Terms related to this study

• Keywords: Interstitial lung disease; Sarcoidosis; Dyspnea
• Additional Relevant MeSH Terms: Cardiovascular Diseases; Vascular Diseases; Respiratory Tract Diseases; Lymphoproliferative Disorders; Lymphatic Diseases; Lung Diseases; Hypertension; Pulmonary Arterial Hypertension; Hypertension, Pulmonary; Sarcoidosis; Vasodilator Agents; Platelet Aggregation Inhibitors; Iloprost
• Other Study ID Numbers: Sarcoid 6