Clinical trial NCT00403650: Inhaled Iloprost for Sarcoidosis-associated Pulmonary Hypertension
Official title: Inhaled Iloprost for Sarcoidosis Associated Pulmonary Hypertension
Study type: INTERVENTIONAL
Overall status: COMPLETED
Phase: PHASE4
Conditions: Sarcoidosis
Pulmonary Arterial Hypertension
Keywords: Sarcoidosis
Dyspnea
Interstitial lung disease
Lead sponsor: University of Cincinnati
Sponsor class: OTHER
First posted: 2006-11-27
Enrollment: 20
Sex: ALL
Minimum age (years): 18
Maximum age (years): 90
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Allocation: NON_RANDOMIZED
Intervention model: SINGLE_GROUP
Primary purpose: TREATMENT

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

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.

Eligibility criteria: 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

Primary outcomes:
measure: Change in six minute walk distance
timeFrame: 24 weeks
Secondary outcomes:
measure: Quality of life
timeFrame: 24 weeks
measure: Respiratory function
timeFrame: 24 weeks
measure: Toxicity
timeFrame: 24 weeks
measure: Pulmonary artery hemodynamics
timeFrame: 24 weeks
Other outcomes:
Locations:
city: Cincinnati
country: United States
facility: University of Cincinnati
state: Ohio
Study officials:
affiliation: University of Cincinnati
name: Robert P. Baughman, MD
role: PRINCIPAL_INVESTIGATOR
References:
citation: 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.