Study of Ambrisentan With Antifibrotic Agent Combination Therapy in Diffuse Systemic Sclerosis

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Brief Title

Study of Ambrisentan With Antifibrotic Agent Combination Therapy in Diffuse Systemic Sclerosis

Official Title

An Open Label Study of Ambrisentan With Antifibrotic Agent Combination Therapy in Treatment of Diffuse Systemic Sclerosis

Brief Summary

      Systemic sclerosis is a chronic autoimmune connective tissue disorder with no universally
      accepted disease modifying regimen. Recruiting patients for systemic sclerosis treatment
      studies is difficult due to the limited availability of such patients and furthermore the use
      of a placebo arm is often deemed unethical due to the poor survival of diffuse systemic
      sclerosis patients.

      Long-term controlled trials examining functional outcomes and survival from novel therapeutic
      agents for systemic sclerosis are often difficult to undertake because of costs, rarity of
      the disease and ethical issues with the use of a true placebo. Open label single center
      studies while inferior to multicenter placebo controlled studies, have helped establish the
      benefits of certain pharmaceutical agents in systemic sclerosis, and while not universally
      accepted as disease modifying agents, have been used with some success to treat systemic
      sclerosis.

      The hypothesis on which we are basing this study is that an endothelin receptor antagonist
      and disease modifying agent with antifibrotic properties will have additive influence on
      fibrosis, inhibit cellular and humoral hyperactivity and interfere with smooth muscle
      proliferation in the vessel wall. The combination of these two agents will also be the first
      regimen to address the heterogeneity of scleroderma manifestations including ILD, pulmonary
      arterial hypertension and skin manifestations
    

Detailed Description

      This is an open label, single center study to determine the efficacy and safety of
      ambrisentan and antifibrotic agent combination in systemic sclerosis. Up to twenty patients
      will be recruited within the next year who have early diffuse systemic sclerosis and are
      presently receiving treatment with any of the following antifibrotic agents - cellcept,
      colchicine, azathioprine, D-penicillamine, methotrexate or cyclophosphamide. Ambrisentan will
      be added to the present agent and then followed for 12 months.

      Patients, male or female, > 18 years with a clinical diagnosis of systemic sclerosis
      fulfilling the criteria of the American College of Rheumatology (formerly the American
      Rheumatism Association) classification criteria for systemic sclerosis (24), and diffuse
      cutaneous involvement based on the criteria of LeRoy et al

      A thorough baseline evaluation will determine the extent and severity of systemic sclerosis
      in the individual patients using laboratory studies and the clinical evaluation. Monthly
      follow-ups will capture any safety issues related to the combination therapy based again on
      laboratory studies and clinical evaluation. At the six month and twelve month follow-up a
      thorough evaluation will again be undertaken to evaluate the extent and severity of the
      disorder. Event driven follow-ups will also take place to record and establish any safety
      issues that may arise. Clinical end-points will be the focus of this study.

      Dosing of ambrisentan will begin at 5mg daily for the first month. Half the patients will
      remain at 5mg daily, while the remaining patients will be increased to a maintenance dose of
      10mg daily on the fourth week. Subjects will continue their present dose and schedule of
      disease modifying/antifibrotic medication for the duration of the study.
    


Study Type

Interventional


Primary Outcome

The Benefit That an Antifibrotic Agent and Ambrisentan Combination Have on the Cutaneous Involvement of Patients With Early Diffuse Systemic Sclerosis by Utilizing the MRSS

Secondary Outcome

 Systemic Sclerosis Quality of Life Assessed by the SF-36.

Condition

Systemic Sclerosis

Intervention

Ambrisentan

Study Arms / Comparison Groups

 open label: medication Ambrisentan
Description:  Open label study of Ambrisentan.
Ambrisentan will begin at 5mg daily for the first month.
Half the patients will remain at 5mg daily, while the remaining patients will be increased to a maintenance dose of 10mg daily on the fourth week. Subjects will continue their present dose and schedule of disease modifying/antifibrotic medication for the duration of the study.
** Dose escalation was attempted however none of the patients were able to increase. Therefore all subjects remained on 5 mg daily throughout the study. 12 patients on mycophenolate mofetil, 2 on mycophenolic acid and one on methotrexate

Publications

* Includes publications given by the data provider as well as publications identified by National Clinical Trials Identifier (NCT ID) in Medline.

Recruitment Information


Recruitment Status

Drug

Estimated Enrollment

15

Start Date

February 2010

Completion Date

July 2016

Primary Completion Date

July 2016

Eligibility Criteria

        Inclusion Criteria:

        Patients, male or female, greater than 18 years with a clinical diagnosis of systemic
        sclerosis fulfilling the criteria of the American College of Rheumatology (formerly the
        American Rheumatism Association) classification criteria for systemic sclerosis, and
        diffuse cutaneous involvement based on the criteria of LeRoy et al

          -  Onset of skin sclerosis less than or equal to 48 months before study entry.

          -  Extent of skin sclerosis involving the trunk and/or arms and legs proximally to the
             elbows and/or knees.

          -  Present regimen consisting of one of the following: cellcept, D-penicillamine,
             methotrexate or cyclophosphamide.

          -  Previous history of using an alternative antifibrotic agent prior to present regimen
             will be permitted.

          -  Total antifibrotic treatment regimen duration should be less than or equal to 48
             months.

        Exclusion Criteria:

          -  Systemic sclerosis with skin involvement confined to face or acral regions of the
             body.

          -  Chemically induced scleroderma.

          -  Diffuse fasciitis.

          -  Mixed connective tissue disease and overlap syndromes.

          -  Pregnancy or nursing.

          -  Use of non-reliable method of contraception.

          -  Major surgery in the past month.

          -  Inability or unwillingness to provide written informed consent.

          -  Inability or unwillingness to comply with the requirements of the protocol as
             determined by the investigator.

          -  Known hypersensitivity or contraindication to ambrisentan
      

Gender

All

Ages

19 Years - 90 Years

Accepts Healthy Volunteers

No

Contacts

Chris Derk, MD, MSc, , 

Location Countries

United States

Location Countries

United States

Administrative Informations


NCT ID

NCT01093885

Organization ID

Ambrj55501.


Responsible Party

Sponsor

Study Sponsor

University of Pennsylvania

Collaborators

 Gilead Sciences

Study Sponsor

Chris Derk, MD, MSc, Principal Investigator, University of Pennsylvania


Verification Date

November 2018