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Pilot Efficacy and Safety Study of Oral DF2156A in Patients With Active Bullous Pemphigoid

Phase 2
Terminated
Conditions
Bullous Pemphigoid
Interventions
Registration Number
NCT01571895
Lead Sponsor
Dompé Farmaceutici S.p.A
Brief Summary

The objective of this clinical trial was to evaluate whether DF2156A has a potential in improving the clinical outcome in patients with active blistering bullous pemphigoid (BP) to warrant its further development. The safety of DF2156A in the specific clinical setting was also evaluated.

Detailed Description

The study was a phase 2, multicentre, single arm, pilot study. It has been designed to determine if DF2156A has sufficient activity to warrant its further development.

A total of twelve (12) BP patients were planned to be involved. They were planned to receive DF2156A orally at the dose of 150 mg twice a day for a maximum of 14 days.

Recruitment was intended to be competitive among the study sites, until the planned number of patients is enrolled. Competitive recruitment has been chosen to increase the speed of recruitment and to account for any unexpected occurrence at a site that negatively impact enrolment rate.

The single arm design has been chosen as an appropriate tool for this pilot phase 2 study, considering that BP is a rare disease where a placebo control is not acceptable. Moreover, as there is no spontaneous acute recovery from the active blistering condition, any improvement in patient outcome can be attributed to a positive effect of the Investigational Product.

Each patient was intended to be involved in the study for a screening period, for 14 days of treatment, for all required measurements up to hospital discharge (planned on day 8+1 of treatment) and for one assessment occasion on day 15+1, either during hospital stay or after hospital discharge (out-patient visit). An optional post-treatment visit might be scheduled at day 30+3.

Due to the lack of efficacy observed at 1/3 of the enrollment at the investigated dosage, the patients' enrollment was interrupted and trial, hence, was early terminated. More precisely, only 1 of the 4 enrolled patients completed the study's 14-day treatment period. The remaining 3 patients were discontinued from the study early (1 patient due to treatment failure and 2 patients who were discontinued and admitted to rescue therapy).

While DF2156A appeared to be safe and was generally well-tolerated with only mild AEs reported in 3 patients (and no deaths, SAEs, or discontinuations from the study due to AEs), the limited sample size of the safety population prevents any overall conclusions of safety regarding the investigational product. For this reason no results other than listings are available.

See the MEX0111 synopsis on the EU Clinical Trial Register: https://www.clinicaltrialsregister.eu/ctr-search/rest/download/result/attachment/2011-000756-42/1/27931

Recruitment & Eligibility

Status
TERMINATED
Sex
All
Target Recruitment
4
Inclusion Criteria
  • Male and female patients aged >50 years.
  • Patients with newly diagnosed or relapsing bullous pemphigoid based on clinical diagnosis to be confirmed by direct immunofluorescence and indirect immunofluorescence on salt-spit skin (or BP180 and/or BP230 ELISA). Confirmation by laboratory tests will be obtained ideally before or anyway within one week after enrolment.

For the purpose of this study, clinical relapses are defined as re-appearance of clinical symptoms after the patient had attained remission lasting for more than 3 months without immunosuppressive treatment. In patients with relapsing BP, clinical diagnosis will be confirmed by indirect immunofluorescence or BP180 and/or BP230 ELISA only.

  • Patients with mild to moderate active blistering disease (total number of blisters between 1 and 30) whether associated or not with urticarial/eczematous lesions.

  • Patients with modified ABSIS score ≤50

  • Patients free from any systemic treatments that may affect the course of the disease with the following off-period prior to enrolment:

    1. 3 weeks: steroids, dapsone, tetracyclines, nicotinamide,
    2. 3 months: azathioprine, mycofenolate mofetil, cyclophosphamide, methotrexate, intravenous immunoglobulins, immunoadsorption, TNF antagonists
    3. 12 months: rituximab, leflunomide
  • Patients free from any topical treatments other than topical antibiotics and antiseptics in the 4 days prior to enrolment.

  • Patients able to comply with the protocol procedures for the duration of the study, including scheduled follow-up visits and examinations.

  • Patients able to provide informed consent.

Exclusion Criteria
  • Patients with a Karnofsky rating score <40%.
  • Patients with mucosal involvement.
  • Patients with moderate to severe renal impairment as per calculated creatinine clearance (CLcr) < 50 mL/min according to the Cockcroft-Gault formula (Cockcroft-Gault , 1976).
  • Patients with hepatic dysfunction defined by increased ALT/AST > 3 x upper limit of normal (ULN) and increased total bilirubin > 3 mg/dL [>51.3 μmol/L].
  • Patients with hypoalbuminemia defined as serum albumin < 3 g/dL.
  • Patients with a baseline (day 0/1, pre-dose) QTcF > 470 msec.
  • Patients who had a myocardial infarction in the 6 months prior to enrolment.
  • Patients on treatment with phenytoin, warfarin, sulphonylurea hypoglycemics (e.g. tolbutamide, glipizide, glibenclamide/glyburide, glimepiride, nateglinide) and high dose of amitriptyline (> 50 mg/day).
  • Patients with known hypersensitivity to non-steroidal antiinflammatory drugs.
  • Patients using any investigational agent within 12 months prior to enrolment.
  • Pregnant or breast feeding women. Unwillingness to use effective contraceptive measures up to 2 months after the end of study drug administration (females and males).

Additional Exclusion Criteria for Germany only:

  • Patients with hypokalemia defined as serum potassium < 3.5 mmol/L.
  • Patients with clinically relevant bradycardia (heart rate < 50 beats/min)
  • Patients with a complete left bundle branch block.
  • Patients with a history of uncontrolled or labile hypertension
  • Patients with a history of congestive heart failure.
  • Patients with a history of cardiomyopathy.
  • Patients with unstable angina pectoris.
  • Patients with a personal or family history of congenital or documented acquired QT interval prolongation.
  • Patients with a significant atrial or ventricular arrhythmia or symptomatic arrhythmia in the past.

Study & Design

Study Type
INTERVENTIONAL
Study Design
SINGLE_GROUP
Arm && Interventions
GroupInterventionDescription
DF2156A 150 mgDF2156A150 mg capsule twice a day (every 12 h) for a maximum of 14 days
Primary Outcome Measures
NameTimeMethod
Total number of blisters from baselineday 0/1 (pre-dose), 8 and 15

Total number of blisters from baseline

Modified ABSIS score change from baselineday 0/1 (pre-dose), 8 and 15

ABSIS score will be measured according to the pemphigus scoring sheet \[Rosenbach, 2009\] adjusted to the clinical manifestation in BP patients, as per specifications in protocol.

Physician Global Assessment (PGA) score measured on a 0-10 scale [Rosenbach, 2009]. Percent change from baselineday 0/1 (pre-dose), 8 and 15

PGA score will be measured according to the following scale:

0

1 2 3 4 5 6 7 8 9 10 Perfect Worst health skin condition imaginable

The following guidelines will help standardize PGA:

0: no lesions 2: almost cleared, no functional impairment 4: few lesions / low functional impairment 6: moderate 8: severe / extensive 10: life-threatening

Pruritus measured on a 10 cm visual analogue scale. Absolute value change from baselineday 0/1 (pre-dose), 8 and 15

Pruritus will be measured according to the following scale:

0 10 No pruritus Worst pruritus I can imagine

Eosinophil blood count. Percent change from baselinescreening and day 15

Eosinophil blood count. Percent change from baseline

Percentage of patients with treatment failure (drug discontinuation due to disease worsening)day 8

treatment failure (drug discontinuation due to disease worsening)

Percentage of patients completely free from blistersday 15

Percentage of patients completely free from blisters

Number of patients who are still free from blisters without requiring any systemic or topical rescue treatment - OptionalDay 30

Number of patients who are still free from blisters without requiring any systemic or topical rescue treatment - Optional

QTcF. Change from baselineItaly: day 0/1 (pre-dose), day 1, 5, 8 and 15___Ger:day 0/1 (pre-dose), day 1, 3, 5, 8 and 15

QTcF. Change from baseline

Incidence of Adverse Events and Serious Adverse Eventsthroughout the study up to day 15 or 30

Incidence of Adverse Events and Serious Adverse Events

Blisters percent change from baselineday 0/1 (pre-dose), 8 and 15

Blisters percent change from baseline

Modified ABSIS score percent change from baselineday 0/1 (pre-dose), 8 and 15

ABSIS score will be measured according to the pemphigus scoring sheet \[Rosenbach, 2009\] adjusted to the clinical manifestation in BP patients, as per specifications in protocol.

Physician Global Assessment (PGA) score measured on a 0-10 scale [Rosenbach, 2009]. Absolute value change from baselineday 0/1 (pre-dose), 8 and 15

PGA score will be measured according to the following scale:

0

1 2 3 4 5 6 7 8 9 10 Perfect Worst health skin condition imaginable

The following guidelines will help standardize PGA:

0: no lesions 2: almost cleared, no functional impairment 4: few lesions / low functional impairment 6: moderate 8: severe / extensive 10: life-threatening

Number of patients with treatment failure (drug discontinuation due to disease worsening)day 8

Number of patients with treatment failure (drug discontinuation due to diseas

Pruritus measured on a 10 cm visual analogue scale. Percent change from baselineday 0/1 (pre-dose), 8 and 15

Pruritus will be measured according to the following scale:

0 10 No pruritus Worst pruritus I can imagine

Number of patients completely free from blistersday 15

Number of patients completely free from blisters

QTcF. Absolute valueItaly: day 0/1 (pre-dose), day 1, 5, 8 and 15___Ger:day 0/1 (pre-dose), day 1, 3, 5, 8 and 15

QTcF. Absolute value

Eosinophil blood count. Absolute number change from baselinescreening and day 15

Eosinophil blood count. Absolute number change from baseline

Secondary Outcome Measures
NameTimeMethod
Plasma levels of DF2156A and its major metabolites (DF2227 and DF2108) at steady state conditionsday 5 and 8

Plasma levels of DF2156A and its major metabolites (DF2227 and DF2108) at steady state conditions

Trial Locations

Locations (4)

Klinik für Dermatologie, Allergologie und Venerologie - Universitätsklinikum Schleswig-Holstein, Campus Lübeck; Ratzeburger Allee 160

🇩🇪

Lübeck, Germany

Klinik für Dermatologie und Allergologie - Philips Universität; 35037

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Marburg, Germany

I Divisione di Dermatologia, Istituto Dermopatico dell'Immacolata, IRCCS;

🇮🇹

Roma, Italy

Department of Dermatology - Universitäts-Hautklinik; Hauptstraße 7

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Freiburg, Germany

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