BOSSA Study: Bosentan for the treatment of Steroid-resistant Pulmonary Sarcoidosis

Randomised placebo-controlled, double-blind, exploratory trial of Bosentan for Steroid-resistant Pulmonary Sarcoidosis: the BOSSA Study

Status

Active, not recruiting

Phases

Phase 2

Study type

Interventional

Source

ISRCTN

Registry ID

ISRCTN73579020

Enrollment

Registered

2010-09-27

Start date

2007-10-01

Completion date

Unknown

Last updated

2018-12-04

For informational purposes only — not medical advice. Sourced from public registries and may not reflect the latest updates. Terms

Conditions

Sarcoidosis with pulmonary involvement Haematological Disorders Sarcoidosis

Interventions

Patients will be randomised to receive 1. Bosentan 2. Placebo 62.5 mg Twice daily (BID) for 4 weeks followed by 125 mg BID for 11 months.

Eligibility

Sex/Gender

All

Inclusion criteria

Inclusion criteria: 1. Biopsy-proven sarcoidosis with pulmonary involvement stages II, III, (IV) according to Silzbach 2. Persistant symptoms on long-term oral corticosteroids (greater than 2 months; 5 mg prednisone or equivalent and/or other immunosuppressive agents 3. Aged greater than 18 years 4. Informed written consent 5. Impaired exercise capacity (oxygen uptake [VO2] peak less than 80%) or resting lung functions (forced expiratory volume in one second [FEV1], forced vital capacity [FVC] or diffusing capacity of the lung for carbon monoxide [DLCO] less than 80%)

Exclusion criteria

Exclusion criteria: 1. Systemic illness other than sarcoidosis requiring immunosuppressive therapy 2. Honey combing greater than 10% on High Resolution Computed Tomography [HRCT] scan 3. Marked disturbance of liver enzymes at baseline 4. Pregnancy 5. Relevant psychiatric illness or addictive disorder 6. Previous or current treatment with bosentan 7. Therapry with cyclosporine A

Design outcomes

Primary

Measure	Time frame
1. Safety as measured by severe adverse events (SAEs) and necessity to stop bosentan due to increases liver enzymes 2. Efficacy at 12 months as measured by: 2.1. Overall response rate as defined by a 10% improvement of either TLC, DLCO, VO2 peak, endurance time at 75% of VO2 peak or 6-min walk distance (6MWD) or 2.2. A decrease in the HRCT-score greater than or equal to and absence of worsening by at least 10% in any functional parameters and absence of an increase in the HRCT-score greater than or equal to 2	—

Measure

Time frame

1. Safety as measured by severe adverse events (SAEs) and necessity to stop bosentan due to increases liver enzymes 2. Efficacy at 12 months as measured by: 2.1. Overall response rate as defined by a 10% improvement of either TLC, DLCO, VO2 peak, endurance time at 75% of VO2 peak or 6-min walk distance (6MWD) or 2.2. A decrease in the HRCT-score greater than or equal to and absence of worsening by at least 10% in any functional parameters and absence of an increase in the HRCT-score greater than or equal to 2

—

Secondary

Measure	Time frame
1. Overall adverse events, reported during the regular visits of the patients at the centres 2. Changes in QoL, measured by SF-36 questionnaire 3. Decrease in expression of genes associated with firbroproliferation 4. Efficacy at 3, 6 and 9 months	—

Countries

Switzerland

Outcome results

None listed

Source: ISRCTN (via WHO ICTRP) · Data processed: Feb 23, 2026