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Digital Ischemic Lesions in Scleroderma Treated With Oral Treprostinil Diethanolamine

Phase 2
Completed
Conditions
Scleroderma
Systemic Sclerosis
Interventions
Registration Number
NCT00775463
Lead Sponsor
United Therapeutics
Brief Summary

This study will evaluate the effect of treprostinil diethanolamine (UT-15C) sustained release tablets(compared to placebo) on digital ulcers in patients with scleroderma. Treprostinil diethanolamine is an analog of prostacyclin. Prostacyclin is a naturally occuring substance produced by the cells of blood vessels that inhibits platelet aggregation, induces vasodilation, and suppresses smooth muscle proliferation. Improvement in blood flow in lower limbs and fingers would be anticipated to result in a reduction in ischemic pain, Raynaud's phenomenon and promote healing of digital ulcers and other ischemic wounds.

Detailed Description

Not available

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
148
Inclusion Criteria
  • Subject gave voluntary written informed consent to participate in the study
  • Diagnosis of systemic sclerosis (SSc) as defined by American College of Rheumatology (ACR) criteria
  • Males and females age greater than 18 years at Screening
  • Presence of at least one active digital ulcer (met protocol defined qualifications for active digital ulcer) at Baseline
  • Females of childbearing potential willing to use a reliable form of medically acceptable contraception and have a negative pregnancy test at Screening and Baseline
  • Able to communicate effectively with study personnel and willing to comply with protocol requirements
Exclusion Criteria
  • Diagnosis of pulmonary arterial hypertension (PAH)
  • Body weight less than 40 kg at Screening and confirmed at Baseline
  • History of postural hypotension, unexplained syncope, a blood pressure that is less than 90 mmHg systolic or 50 mmHg diastolic at Screening and Baseline
  • Hemoglobin concentration less than 75% of the lower limit of the normal range at Screening
  • Moderate to severe hepatic impairment, i.e., Child-Pugh Class B or C, or ALT greater than three times upper limit of normal
  • Intractable diarrhea, or severe malabsorption, defined as greater than 15% unintentional loss of body weight in the last 6 months prior to Screening; any severe organ failure (e.g., lung, kidney), bleeding diathesis or platelet disorder, or any life-threatening condition
  • Pregnant or breast-feeding
  • Simultaneously fulfilled criteria for a second connective tissue disease including systemic lupus erythermatosus, rheumatoid arthritis or inflammatory myopathy
  • Sympathectomy of the upper limb, involving the hand, performed within 12 months of Baseline. Sympathectomy performed on the non-target limb (hand not presenting with qualifying ulcers) or which did not include the hand, performed within 6 months of Baseline
  • Receipt of prostanoid treatment (epoprostenol, treprostinil sodium, or other prostacyclin analog) within the previous 3 months of Baseline for conditions including Raynaud's phenomenon, rest pain and / or digital ulcers
  • Required systemic antibiotics for infected digital ulcers within 2 weeks of Screening
  • Local injection of botulinum toxin in an affected finger within 1 month prior to Baseline
  • Treatment with endothelin receptor antagonists within 1 month prior to Baseline
  • Patients on phosphodiasterase inhibitors, such as sildenafil or tadalafil, who have received treatment for less than 6 months prior to Baseline (unless for intermittent treatment of male erectile dysfunction)
  • Treatment with statin within 1 month prior to Screening, unless for management of hyperlipidemia
  • Received an investigational product within 1 month preceding Screening
  • Known hypersensitivity to treprostinil diethanolamine or any of the excipients
  • Tobacco or nicotine use at any level within the past 6 months prior to Screening
  • Any condition or laboratory that in the opinion of the investigator might interfere with subject's participation, pose an additional risk for the subject, could prevent understanding the objectives, nature or consequences of the trial, compliance with the protocol, adherence to therapy, or that would interfere with interpretation of study assessments

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
treprostinil diethanolaminetreprostinil diethanolamineTreprostinil diethanolamine sustained release tablet initiated at 0.25 mg BID and titrated up to a maximum dose of 16 mg BID or the individual's maximum tolerated dose.
placebo (sugar pill)placeboMatching placebo sustained release tablet initiated at 0.25 mg BID and titrated up to a maximum dose of 16 mg BID or the individual's maximum tolerated dose.
Primary Outcome Measures
NameTimeMethod
Net Ulcer BurdenWeek 20

Net ulcer burden was defined as the number of "new" or "active" digital ulcers (DU), plus the number of "indeterminate" DUs at that assessment that have previously been classified as either "active" or "new" at any earlier assessment during the study. A DU was defined as an area with visually discernable depth and a loss of continuity of epithelial coverage, which could be denuded or covered by a scab or necrotic tissue. If denuded, the DU was pronounced "active." If denudation could not be judged because of the presence of scab or necrotic tissue, DU presenting with features, including underlying pain, based on Investigator clinical judgment to be consistent with loss of epithelialization, epidermis, or dermis, and requiring treatment were designated as "active." Otherwise, the DU was pronounced "indeterminate." Only DUs distal to the proximal interphalangeal joints, volar to the equator of the finger, not localized in creases and vascular in origin were assessed.

Secondary Outcome Measures
NameTimeMethod
Short-Form McGill Pain QuestionnaireWeek 20

The SF-MPQ assessment has three component scores: the pain rating index (PRI), a pain visual analogue numerical scale (Pain VAS) and the present pain intensity (PPI). PRI is calculated by summing the responses (0=None to 3=Severe) to the 15 questions describing pain during the previous week and rated on an intensity scale as 0= none, 1= mild, 2= moderate or 3= severe and has possible values ranging from 0 to 45. The Pain VAS is a 100 mm VAS on which subjects were asked to rate pain during the previous week with values ranging from no pain (0.0) to worst possible pain (10.0). The PPI rated pain on a 6-point category scale from 0 (no pain) to 5 (excruciating pain).

Time to Ulcer HealingWeek 20

A subject was counted as having all ulcers completely healed at the earliest assessment for which all ulcers are designated as "healed" and no new ulcers appeared for the remainder of the trial. The time to complete healing of all ulcers were calculated as the number of days from randomization to the date of these respective assessments, provided that complete healing was achieved during the study.

Patient Global Assessment of Digital Ulcer Severity VASWeek 20

Patients rated their global impression of digital ulcer severity on a 15-cm VAS from scaled 0 (no disease activity) to 100 (very severe disease).

The term "severity" was used to measure the extent of disease activity and associated disability or discomfort the patient experienced during the indicated time period.

Modified Rodnan Skin Score (mRSS)Week 20

The skin thickening was assessed by the Investigator in 17 body areas: fingers, hands, forearms, arms, feet, legs, and thighs (bilaterally) and face, chest, and abdomen (singly). Each area was scored 0-3; 0 representing normal skin and 3 being severe thickening. The mRSS was the sum of the individual skin assessment scores: possible range of 0-51; 0 (no thickening) to 51 (severe thickening in all 17 areas) .

Time to Ulcer Healing- Percentage of Subjects With Complete HealingWeek 20

A subject was counted as having all ulcers completely healed at the earliest assessment for which all ulcers are designated as "healed" and no new ulcers appeared for the remainder of the trial.

Cochin Hand Function Scale (CHFS)Week 20

The CHFS has been demonstrated as a reliable and valid assessment of hand function at the activity level in persons with SSc. It is comprised of 18 questions with possible integer responses of 0 (without difficulty) to 5 (impossible). The CHFS Score is simply the sum of all 18 questions, divided by the number of questions actually answered, multiplied by 18. At least 10 of the 18 questions must have been answered in order for CHFS to be calculated. Therefore, CHFS Score values can range from 0 (least limitation) to 90 (most limitation). A higher score indicates more difficulty in hand function or greater disability.

Digital Ulcer Pain VASWeek 20

Digital ulcer pain was rated on a 100-mm VAS on which subjects were asked to rate their average overall hand pain during the last week. The recorded value was divided by 10, with values ranging from 0.0 (no pain) to 10.0 (unbearable pain), expressed to one decimal.

Physician Global Assessment of Digital Ulcer Severity VASWeek 20

Physicians rated their global impression of digital ulcer severity on a 15-cm VAS from scaled 0 (no disease activity) to 100 (very severe disease).

The term "severity" was used to measure the extent of disease activity and associated disability or discomfort the patient experienced during the indicated time period.

Scleroderma Health Assessment Questionnaire (SHAQ)Week 20

The SHAQ is a patient self-administered instrument which has been previously validated in SSc and demonstrates meaningful clinical changes in the course of the disease over time. It is comprised of a 20 question instrument pertaining to specific activities with possible integer responses of 0 (without any difficulty) to 3 (unable to do), and five additional scleroderma-specific visual analog scale (VAS) domains (Overall Disease Activity, Raynaud's Phenomenon, Finger Ulcers, Breathing, and Intestinal Problems) with possible values ranging from 0.0 to 15.0. The 20 questions are divided into eight domains. A mean score is calculated for each domain ranging from 0 to 3. A composite HAQ DI score is calculated by dividing the summed domain scores by the number of domains answered. The composite score is reported, falling between 0 and 3 on an ordinal scale. The scores are interpreted as 0 (no impairment in function) to 3 (maximal impairment of function).

Patient Impression of Change (PIC) QuestionnaireWeek 20

The PIC questionnaire consisted of three Likert items that asked the subject to rate changes in their digital ulcer, Raynaud's phenomenon and disease status since their last visit on a seven-level scale (very much improved, much improved, somewhat improved, same, somewhat worse, much worse and very much worse).

Short Form 36Week 20

Change in patient quality of life was measured by the Medical Outcomes Study 36-Item Short Form Health Survey (SF-36), a self-administered questionnaire covering eight areas: physical function, physical role, bodily pain, general health, vitality, social function, emotional role, and mental health. For each area, the score range from 0 (poorer health status) to 100 (better health status). The SF-36 is one of the most widely used and validated instruments to assess quality of life in patients with systemic illnesses. A decrease (negative change) in a domain score corresponds to deterioration.

Trial Locations

Locations (30)

University of Pittsburgh

🇺🇸

Pittsburgh, Pennsylvania, United States

University of Indiana School of Medicine

🇺🇸

Indianapolis, Indiana, United States

University of Connecticut Health Center

🇺🇸

Farmington, Connecticut, United States

Georgetown University

🇺🇸

Washington, District of Columbia, United States

Mayo Clinic Scottsdale

🇺🇸

Scottsdale, Arizona, United States

UMDNJ Clinical Research Center

🇺🇸

New Brunswick, New Jersey, United States

Dalhousie University - QEII Health Science Center

🇨🇦

Halifax, Nova Scotia, Canada

University of Alabama - Arthritis Clinical Intervention Program

🇺🇸

Birmingham, Alabama, United States

University of Utah

🇺🇸

Salt Lake City, Utah, United States

Medical College of Wisconsin

🇺🇸

Milwaukee, Wisconsin, United States

North Shore-LIJ Health System

🇺🇸

Lake Success, New York, United States

Stanford University School of Medicine/Palo Alto VA Health Care System

🇺🇸

Palo Alto, California, United States

Northwestern University - Feinberg School of Medicine

🇺🇸

Chicago, Illinois, United States

Johns Hopkins University - Division of Rheumatology

🇺🇸

Baltimore, Maryland, United States

University of Minnesota

🇺🇸

Minneapolis, Minnesota, United States

The Hospital for Special Surgery

🇺🇸

New York, New York, United States

University of Toledo

🇺🇸

Toledo, Ohio, United States

Virginia Mason Medical Center

🇺🇸

Seattle, Washington, United States

Clinical Sciences Center - University Hospital

🇬🇧

Liverpool, United Kingdom

St Joseph's Health Care

🇨🇦

London, Ontario, Canada

McGill University

🇨🇦

Montreal, Quebec, Canada

Royal Free Hospital - Center for Rheumatology

🇬🇧

London, United Kingdom

Salford Royal Hospital

🇬🇧

Manchester, United Kingdom

UCLA

🇺🇸

Los Angeles, California, United States

Boston University School of Medicine

🇺🇸

Boston, Massachusetts, United States

University of Texas - Houston

🇺🇸

Houston, Texas, United States

Cleveland Clinic Foundation

🇺🇸

Cleveland, Ohio, United States

Denver Medical Center

🇺🇸

Aurora, Colorado, United States

University of Michigan Scleroderma Program

🇺🇸

Ann Arbor, Michigan, United States

Medical University of South Carolina

🇺🇸

Charleston, South Carolina, United States

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