Role of Proteasomes in a Dermatological Autoimmune Disease: Bullous Pemphigoid

Pemphigus Clinical Trial

Official title:

Role of Proteasomes in a Dermatological Autoimmune Disease: Bullous Pemphigoid

Clinical Trial Details — Status: Terminated

Administrative data

• NCT number: NCT01559155
• Other study ID #: LOCAL/2011/PS-02
• Secondary ID: 2012-A00180-43
• Status: Terminated
• Start date: November 5, 2013
• Est. completion date: June 9, 2015

Study information

• Verified date: May 2018
• Source: Centre Hospitalier Universitaire de Nimes
• Contact: n/a
• Is FDA regulated: No
• Study type: Observational

Clinical Trial 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.

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.

Recruitment information / eligibility

• Status: Terminated
• Enrollment: 14
• Est. completion date: June 9, 2015
• Est. primary completion date: June 9, 2015
• Accepts healthy volunteers: Accepts Healthy Volunteers
• Gender: All
• Age group: 18 Years and older

Eligibility

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

Related Conditions & MeSH terms

• Autoimmune Diseases
• Lupus Erythematosus, Cutaneous
• Pemphigoid, Bullous
• Pemphigus

Locations

Country	Name	City	State
France	CHU de Nîmes - Hôpital Universitaire Carémeau	Nîmes Cedex 09

Sponsors (1)

Lead Sponsor	Collaborator
Centre Hospitalier Universitaire de Nimes

Country where clinical trial is conducted

France, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Plasmatic concentration of anti-proteasome autoantibodies (ng/ml)		baseline
Secondary	the daily number of new lesions	For patients suffering from bullous pemphigoid or pemphigus: the daily number of new lesions for the 3 days preceding blood sampling	baseline
Secondary	Presence/absence of mucosal disease	For patients suffering from bullous pemphigoid only	baseline
Secondary	Disease duration (weeks)	For patients suffering from bullous pemphigoid or pemphigus or lupus	baseline
Secondary	% surface area	For patients suffering from bullous pemphigoid or pemphigus or lupus: % of skin area affected in relation to total area	baseline
Secondary	Puritis score	For patients suffering from bullous pemphigoid only: severity of itching on a analog scale varying from 0 to 6	baseline
Secondary	concentration of anti-PB18 antibodies, measured by ELISA	For patients suffering from bullous pemphigoid only; U/ml	baseline
Secondary	Immunohistochemistry	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)	baseline
Secondary	Tissue DNA expression	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)	baseline
Secondary	presence/absence of oral lesions	For patients suffering from pemphigus	baseline
Secondary	Presence/absence of Nikolsky's sign	For patients with pemphigus only	baseline
Secondary	Pemphigus disease area index	For patients with Pemphigus only; score varying from 0 to 120.	baseline
Secondary	Anti-desmogleine 1 and 3 antibody concentrations	For patients with Pemphigus only; ELISA (U/ml	baseline
Secondary	CLASI score for lupus	for lupus patients only; score varying from 0 to 70	baseline
Secondary	Karnofsky's score (%)		baseline
Secondary	Plasma proteasome concentration	ng/ml	baseline
Secondary	% trypsin-like plasma proteasome proteolytic activity		baseline
Secondary	% chymotrypsin-like plasma proteasome proteolytic activity		baseline
Secondary	% caspase-like plasma proteasome proteolytic activity		baseline