Role of Proteasomes in a Dermatological Autoimmune Disease: Bullous Pemphigoid

Tracking Information
First Submitted Date	March 19, 2012
First Posted Date	March 21, 2012
Last Update Posted Date	May 10, 2018
Actual Study Start Date	November 5, 2013
Actual Primary Completion Date	June 9, 2015 (Final data collection date for primary outcome measure)
Current Primary Outcome Measures; (submitted: March 20, 2012)	Plasmatic concentration of anti-proteasome autoantibodies (ng/ml) [ Time Frame: baseline ]
Original Primary Outcome Measures	Same as current
Change History	Complete list of historical versions of study NCT01559155 on ClinicalTrials.gov Archive Site
Current Secondary Outcome Measures; (submitted: March 20, 2012)	the daily number of new lesions [ Time Frame: baseline ]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 ]For patients suffering from bullous pemphigoid only; Disease duration (weeks) [ Time Frame: baseline ]For patients suffering from bullous pemphigoid or pemphigus or lupus; % surface area [ Time Frame: baseline ]For patients suffering from bullous pemphigoid or pemphigus or lupus: % of skin area affected in relation to total area; Puritis score [ Time Frame: baseline ]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 ]For patients suffering from bullous pemphigoid only; U/ml; Immunohistochemistry [ Time Frame: baseline ]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 ]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 ]For patients suffering from pemphigus; Presence/absence of Nikolsky's sign [ Time Frame: baseline ]For patients with pemphigus only; Pemphigus disease area index [ Time Frame: baseline ]For patients with Pemphigus only; score varying from 0 to 120.; Anti-desmogleine 1 and 3 antibody concentrations [ Time Frame: baseline ]For patients with Pemphigus only; ELISA (U/ml; CLASI score for lupus [ Time Frame: baseline ]for lupus patients only; score varying from 0 to 70; Karnofsky's score (%) [ Time Frame: baseline ]; Plasma proteasome concentration [ Time Frame: baseline ]ng/ml; % trypsin-like plasma proteasome proteolytic activity [ Time Frame: baseline ]; % chymotrypsin-like plasma proteasome proteolytic activity [ Time Frame: baseline ]; % caspase-like plasma proteasome proteolytic activity [ Time Frame: baseline ]
Original Secondary Outcome Measures	Same as current
Current Other Outcome Measures	Not Provided
Original Other Outcome Measures	Not Provided
Descriptive Information
Brief Title	Role of Proteasomes in a Dermatological Autoimmune Disease: Bullous Pemphigoid
Official Title	Role of Proteasomes in a Dermatological Autoimmune Disease: Bullous Pemphigoid
Brief Summary	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.
Detailed Description	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.
Study Type	Observational
Study Design	Observational Model: Cohort; Time Perspective: Cross-Sectional
Target Follow-Up Duration	Not Provided
Biospecimen	Retention: Samples With DNA; Description: All left over plasma samples will be incorporated into the biological collection at the Nîmes University Hospital.
Sampling Method	Non-Probability Sample
Study Population	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).
Condition	Pemphigoid, Bullous; Pemphigus; Lupus Erythematosus, Cutaneous
Intervention	Not Provided
Study Groups/Cohorts	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.
Publications *	Not Provided
* Includes publications given by the data provider as well as publications identified by ClinicalTrials.gov Identifier (NCT Number) in Medline.
Recruitment Information
Recruitment Status	Terminated
Actual Enrollment; (submitted: May 4, 2018)	14
Original Estimated Enrollment; (submitted: March 20, 2012)	130
Actual Study Completion Date	June 9, 2015
Actual Primary Completion Date	June 9, 2015 (Final data collection date for primary outcome measure)
Eligibility Criteria	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
Sex/Gender	Sexes Eligible for Study: All
Ages	18 Years and older (Adult, Older Adult)
Accepts Healthy Volunteers	Yes
Contacts	Contact information is only displayed when the study is recruiting subjects
Listed Location Countries	France
Removed Location Countries
Administrative Information
NCT Number	NCT01559155
Other Study ID Numbers	LOCAL/2011/PS-02; 2012-A00180-43 ( Other Identifier: RCB number )
Has Data Monitoring Committee	No
U.S. FDA-regulated Product	Not Provided
IPD Sharing Statement	Not Provided
Responsible Party	Centre Hospitalier Universitaire de Nīmes
Study Sponsor	Centre Hospitalier Universitaire de Nīmes
Collaborators	Not Provided
Investigators	Principal Investigator: Pierre Stoebner, MD Centre Hospitalier Universitaire de Nîmes
PRS Account	Centre Hospitalier Universitaire de Nīmes
Verification Date	May 2018