Saves the questions and what to expect into your notes, next to the visit they belong to.
Keep this study
Lead
Daniel Doberer
Age
18–70
Sex
ALL
Healthy volunteers
Not accepted
You may be eligible if
Signed informed consent prior to any study-mandated procedure.
Male and female patients aged \> 18 and \< 70 years.
Histologically proven sarcoidosis diagnosed at least one year before screening.
Diagnosis of sarcoidosis and with evidence of pulmonary parenchymal disease on chest X-ray or CT (radiological stage II, III) with or without pulmonary hypertension. Subjects with concurrent extrapulmonary sarcoidosis are encouraged to be enrolled.
Progressive disease, defined as follows:
* Deterioration in the 3-12 month period prior to screening in at least two of the following criteria:
* increase in clinical symptoms (cough, shortness of breath, chest pain, fatigue or hemoptysis).
* lung function: decrease of 10% in TLC, FVC or DLCO.
* worsening of radiographic opacities.
* Have been receiving pre-study treatment with prednisolone (or equivalent dose of corticosteroid) as a single agent (≥ 10 mg/day) or other immunosuppressants (methotrexate, azathioprine, cyclophosphamide, TNF inhibitors, etc.) within the 3-month period immediately prior to screening. Patients must be on a stable dose of these medications for \> 4 weeks before starting the study medication.
AST and ALT values within three times upper limit of normal.
Ability to communicate well with the investigator, in the local language, and to understand and comply with the requirements of the study.
Negative pregnancy test in female patients.
Adequate contraception in female patients of childbearing age.
You may not be if
Known hypersensitivity to any excipients of the drug formulation or to bosentan.
Treatment with another investigational drug within 3 months prior to screening.
Pulmonary sarcoidosis:
* without disease progression as defined above
* with radiological stage I
* with radiological stage IV (pulmonary fibrosis with evidence of honey-combing, hilar retraction, bullae and cysts)
Other cause of pulmonary disease:
* Active tuberculosis (or positive Quantiferon test), fungi infection, lymphoma.
* Chronic obstructive pulmonary disease, asthma, interstitial lung disease other than sarcoid-related
Anamnesis of beryllium or asbestos exposition
Previous smoking (\> 10 PY), or active smoker
Previous administration of bosentan
Positive results from the hepatitis serology, except for vaccinated subjects, at screening.
Positive results from the HIV serology at screening.
Malignancy requiring chemotherapy or radiation
Uncontrolled other disease like
* Chronic heart failure (NYHA III, IV)
* Diabetes mellitus (blood glucose 2x per day \> 250 mg/dl , HbA1c \> 10 %)
* Arterial hypertension (SBP \> 180 mmHg)
Concomitant treatment with cyclosporine A
Concomitant treatment with tacrolimus or sirolimus
Concomitant treatment with glibenclamide
Are pregnant, nursing, or planning pregnancy during the trial or within six month period thereafter.
Have a known substance dependency (drug or alcohol within 3 years of screening).
Presumed non-compliance.
Legal incapacity or limited legal capacity at screening.
