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Role of Proteasomes in a Dermatological Autoimmune Disease: Bullous Pemphigoid

This study is currently recruiting participants. (see Contacts and Locations)
Verified November 2014 by Centre Hospitalier Universitaire de Nīmes
Sponsor:
Information provided by (Responsible Party):
Centre Hospitalier Universitaire de Nīmes
ClinicalTrials.gov Identifier:
NCT01559155
First received: March 19, 2012
Last updated: November 3, 2014
Last verified: November 2014

March 19, 2012
November 3, 2014
November 2013
November 2015   (final data collection date for primary outcome measure)
Plasmatic concentration of anti-proteasome autoantibodies (ng/ml) [ Time Frame: baseline ] [ Designated as safety issue: No ]
Same as current
Complete list of historical versions of study NCT01559155 on ClinicalTrials.gov Archive Site
  • the daily number of new lesions [ Time Frame: baseline ] [ Designated as safety issue: No ]
    For patients suffering from bullous pemphigoid or pemphigus: the daily number of new lesions for the 3 days preceding blood sampling
  • Presence/absence of mucosal disease [ Time Frame: baseline ] [ Designated as safety issue: No ]
    For patients suffering from bullous pemphigoid only
  • Disease duration (weeks) [ Time Frame: baseline ] [ Designated as safety issue: No ]
    For patients suffering from bullous pemphigoid or pemphigus or lupus
  • % surface area [ Time Frame: baseline ] [ Designated as safety issue: No ]
    For patients suffering from bullous pemphigoid or pemphigus or lupus: % of skin area affected in relation to total area
  • Puritis score [ Time Frame: baseline ] [ Designated as safety issue: No ]
    For patients suffering from bullous pemphigoid only: severity of itching on a analog scale varying from 0 to 6
  • concentration of anti-PB18 antibodies, measured by ELISA [ Time Frame: baseline ] [ Designated as safety issue: No ]
    For patients suffering from bullous pemphigoid only; U/ml
  • Immunohistochemistry [ Time Frame: baseline ] [ Designated as safety issue: No ]
    For the first 10 patients suffering from bullous pemphigoid included at the Nîmes University Hospital only; Skin biopsy immunohistochemistry scores for the pan-alpha, alpha6, beta1, beta2, beta1i, beta5i and rpt5 subunits (negative, weak, moderate, strong)
  • Tissue DNA expression [ Time Frame: baseline ] [ Designated as safety issue: No ]
    For the first 10 patients suffering from bullous pemphigoid included at the Nîmes University Hospital only; Skin biopsy, plasma and circulating mononuclear cell DNA expression for the pan-alpha, alpha6, beta1, beta2, beta1i, beta5i and rpt5 subunits (weighted by beta-actin)
  • presence/absence of oral lesions [ Time Frame: baseline ] [ Designated as safety issue: No ]
    For patients suffering from pemphigus
  • Presence/absence of Nikolsky's sign [ Time Frame: baseline ] [ Designated as safety issue: No ]
    For patients with pemphigus only
  • Pemphigus disease area index [ Time Frame: baseline ] [ Designated as safety issue: No ]
    For patients with Pemphigus only; score varying from 0 to 120.
  • Anti-desmogleine 1 and 3 antibody concentrations [ Time Frame: baseline ] [ Designated as safety issue: No ]
    For patients with Pemphigus only; ELISA (U/ml
  • CLASI score for lupus [ Time Frame: baseline ] [ Designated as safety issue: No ]
    for lupus patients only; score varying from 0 to 70
  • Karnofsky's score (%) [ Time Frame: baseline ] [ Designated as safety issue: No ]
  • Plasma proteasome concentration [ Time Frame: baseline ] [ Designated as safety issue: No ]
    ng/ml
  • % trypsin-like plasma proteasome proteolytic activity [ Time Frame: baseline ] [ Designated as safety issue: No ]
  • % chymotrypsin-like plasma proteasome proteolytic activity [ Time Frame: baseline ] [ Designated as safety issue: No ]
  • % caspase-like plasma proteasome proteolytic activity [ Time Frame: baseline ] [ Designated as safety issue: No ]
Same as current
Not Provided
Not Provided
 
Role of Proteasomes in a Dermatological Autoimmune Disease: Bullous Pemphigoid
Role of Proteasomes in a Dermatological Autoimmune Disease: Bullous Pemphigoid

The primary objective of this study is to describe and compare plasmatic anti-proteasome auto-antibody concentrations among three distinct groups: (1) patients suffering from bullous pemphigoide; (2) patients suffering from other dermatological auto-immune diseases; (3) an elderly control group.

The secondary objectives of this study are:

To compare the following parameters between the 3 groups:

  • plasmatic proteasome concentrations
  • plasmatic proteasome proteolytic activity

To explore the potential relationships between:

  • plasmatic proteasome concentrations
  • plasmatic proteasome proteolytic activity
  • plasmatic anti-proteasome auto-antibody concentrations
  • measures of disease severity for dermatological auto-immune diseases

To characterize plasmatic anti-proteasome auto-antibodies in patients suffering from bullous pemphigoide and other dermatological auto-immune diseases (other bullous auto immune diseases: pemphigus, cutaneous lupus, ...).

To characterize the expression and the activity of proteasomes in skin samples, in plasma and in circulating mononuclear cells in patients with bullous pemphigoide.

Observational
Observational Model: Cohort
Time Perspective: Cross-Sectional
Not Provided
Retention:   Samples With DNA
Description:

All left over plasma samples will be incorporated into the biological collection at the Nîmes University Hospital.

Non-Probability Sample

The study population is composed of three groups: (1) 50 patients with newly diagnosed (untreated) bullous pemphigoid, (2) 50 control patients matched for age and sex with the previous group and (3) 30 patients with other dermatological auto-immune disease (15 pemphigus + 15 cutaneous lupus).

  • Pemphigoid, Bullous
  • Pemphigus
  • Lupus Erythematosus, Cutaneous
Not Provided
  • Bullous pemphigoid
    Patients in this cohort are newly diagnosed (or have not started treatment) with bullous pemphigoid
  • Other bullous-like auto-immune
    Patients in this cohort are newly diagnosed (or have not started treatment) with pemphigus (15 patients) or cutaneous lupus (15 patients)
  • Control group
    Patients in this cohort are hospitalized at the Nîmes University Hospital, and have no history of autoimmune, inflammatory or neoplastic disease. Patients are matched for age and sex with patients in the bullous pemphigoid cohort.
Not Provided

*   Includes publications given by the data provider as well as publications identified by ClinicalTrials.gov Identifier (NCT Number) in Medline.
 
Recruiting
130
November 2015
November 2015   (final data collection date for primary outcome measure)

Inclusion Criteria:

  • The patient must have given his/her informed and signed consent
  • The patient must be insured or beneficiary of a health insurance plan
  • The patient is not taking systemic treatment
  • The patient has not been treated with topical steroids for more than 15 days.

For the bullous pemphigoid group:

  • clinical signs: erythematous-based lesions, especially on flexion areas of the arms and legs, not afflicting mucous membranes, and without atrophic scaring
  • histology: without epidermal acantholysis

For the pemphigus group:

  • patient with pemphigus

For the lupus group:

  • systemic lupus patients: presence of the 4 diagnostic criteria for systemic lupus erythematosus as defined by the American College of Rheumatology (amended 1997)
  • or characteristics of subacute cutaneous lupus: clinical, histological and immunological (anti-SSa)
  • or clinical and histological characteristics of chronic lupus

For the control group:

  • hospitalized patients with no history of auto-immune, inflammatory or evolving neoplastic disease

Exclusion Criteria:

  • The patient is participating in another study
  • The patient is in an exclusion period determined by a previous study
  • The patient is under judicial protection, under tutorship or curatorship
  • The patient refuses to sign the consent
  • It is impossible to correctly inform the patient
  • The patient is pregnant, parturient, or breastfeeding

For patients with bullous pemphigoid, pemphigus or lupus:

  • The patient is taking systemic treatment
  • The patient has been taking topical steroids for more than 15 days.

For the controls:

  • autoimmune disease
  • inflammatory disease
  • evolving neoplastic disease
  • surgery during the last 6 months
Both
18 Years and older
Yes
Contact: Pierre Stoebner, MD +33.(0)4.66.68.40.43 pierre.stoebner@chu-nimes.fr
Contact: Carey M Suehs, Ph D +33.(0)4.66.68.67.88 carey.suehs@chu-nimes.fr
France
 
NCT01559155
LOCAL/2011/PS-02, 2012-A00180-43
No
Centre Hospitalier Universitaire de Nīmes
Centre Hospitalier Universitaire de Nīmes
Not Provided
Principal Investigator: Pierre Stoebner, MD Centre Hospitalier Universitaire de Nîmes
Centre Hospitalier Universitaire de Nīmes
November 2014

ICMJE     Data element required by the International Committee of Medical Journal Editors and the World Health Organization ICTRP