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Pemphigus is an autoimmune disease specific to the skin and mucous membranes characterized by the production of IgG4 isotype autoantibodies (AC) directed mainly against two proteins involved in interkeratinocyte adhesion: desmoglein 1 (Dsg1 ) and desmoglein 3 (Dsg3) (1-3). The binding of auto-AC on these proteins disrupts their adhesion function, resulting in inter-keratinocyte dysjunction called "acantholysis" responsible for the formation of intraepidermal bubbles.
Treatment of pemphigus is typically based on systemic corticosteroids. High doses are usually necessary because of the frequent cortico-resistance of the disease. In recent years, several studies have focused on the treatment of pemphigus with anti-CD20: rituximab. The "Ritux 3" study (NCT00784589), a randomized, multicentre, randomized, non-blind clinical trial involving 90 patients, found that the use of rituximab as first-line therapy in combination with short corticosteroid therapy was extremely effective and that cortisone sparing was thus obtained limited the occurrence of side effects of treatment. On the other hand, this study showed that the 2 rituximab maintenance infusions of 500 mg to M12 and M18 allowed the maintenance of a high rate of complete remission up to the 3rd year of follow-up.
Questions remain to explain the long-term action of rituximab, in particular that of the evolution of these auto-reactive B cells (specific DSG) away from lymphocyte reconstitution B, as well as the evolution of auto-AC. anti-DSG and total IgG CAs, so as to ensure that the disappearance of the auto-reactive compartment is not accompanied by a long-term overall immunosuppression (and therefore a possible risk of infection).
The immunological changes induced in the long term as well as the precise mechanism of action of these treatments and particularly rituximab which allows a complete remission 5 years after treatment in many patients remain little known.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Rituximab | Experimental | Rituximab in combination with reduced corticosteroids is administrated |
|
| Standard corticosteroid | Active Comparator | Standard corticosteroid is administrated |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Rituximab | Drug | Patient was enrolled into the RITUXIMAB 3 study in the arm Rituximab in association with low corticosteroids'therapy |
|
| Measure | Description | Time Frame |
|---|---|---|
| Evaluate the occurrence of long-term serious AEs and AEs in patients initially randomized in the rituximab arm and in those who have secondarily received the product during the course of their pemphigus | Collect of SAE and AE | 1 day: 5 to 7 years after the inclusion into RITUXIMAB 3 study |
| Evaluate the long-term relapse rate in patients initially randomized in the rituximab arm and in those who have secondarily received the product during the course of their pemphigus | Collect of relapses | 1 day: 5 to 7 years after the inclusion into RITUXIMAB 3 study |
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Inclusion Criteria:
Exclusion Criteria:
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Chu Angers | Angers | 49100 | France | |||
| Ap-Hp Avicenne |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 28342637 | Result | Joly P, Maho-Vaillant M, Prost-Squarcioni C, Hebert V, Houivet E, Calbo S, Caillot F, Golinski ML, Labeille B, Picard-Dahan C, Paul C, Richard MA, Bouaziz JD, Duvert-Lehembre S, Bernard P, Caux F, Alexandre M, Ingen-Housz-Oro S, Vabres P, Delaporte E, Quereux G, Dupuy A, Debarbieux S, Avenel-Audran M, D'Incan M, Bedane C, Beneton N, Jullien D, Dupin N, Misery L, Machet L, Beylot-Barry M, Dereure O, Sassolas B, Vermeulin T, Benichou J, Musette P; French study group on autoimmune bullous skin diseases. First-line rituximab combined with short-term prednisone versus prednisone alone for the treatment of pemphigus (Ritux 3): a prospective, multicentre, parallel-group, open-label randomised trial. Lancet. 2017 May 20;389(10083):2031-2040. doi: 10.1016/S0140-6736(17)30070-3. Epub 2017 Mar 22. |
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Patients included and treated in the RITUXIMAB 3 study (NCT00784589):
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| corticosteroids'therapy | Drug | Patient was enrolled into the RITUXIMAB 3 study in the arm Patient was enrolled into the RITUXIMAB 3 study in the arm standard corticosteroid |
|
| Bobigny |
| 93000 |
| France |
| Chu Bordeaux | Bordeaux | 33076 | France |
| Chu Brest | Brest | 29200 | France |
| Chu Clermont-Ferrand | Clermont-Ferrand | 63100 | France |
| Ap-Hp Henri Mondor | Créteil | 94010 | France |
| Chu Dijon | Dijon | 21000 | France |
| Ch Le Mans | Le Mans | 72037 | France |
| Chru Lille | Lille | 59000 | France |
| Chu Limoges | Limoges | 87000 | France |
| Hcl Edouard Herriot | Lyon | 69003 | France |
| Hcl Ch Lyon Sud | Lyon | 69310 | France |
| Ap-Hm La Timone | Marseille | 13385 | France |
| Chu Montpellier | Montpellier | 34090 | France |
| Chu Nantes | Nantes | 44000 | France |
| Ah-Hp Cochin | Paris | 75006 | France |
| Ap-Hp Saint-Louis | Paris | 75010 | France |
| Ap-Hp Cochin | Paris | 75014 | France |
| Ap-Hp Bichat | Paris | 75018 | France |
| Chu Reims | Reims | 51092 | France |
| Chu Rennes | Rennes | 35000 | France |
| Chu Rouen | Rouen | 76031 | France |
| Chu Saint-Etienne | Saint-Etienne | 42270 | France |
| Chu Toulouse | Toulouse | 31059 | France |
| Chu Tours | Tours | 37000 | France |
| ID | Term |
|---|---|
| D001327 | Autoimmune Diseases |
| ID | Term |
|---|---|
| D007154 | Immune System Diseases |
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| ID | Term |
|---|---|
| D000069283 | Rituximab |
| ID | Term |
|---|---|
| D058846 | Antibodies, Monoclonal, Murine-Derived |
| D000911 | Antibodies, Monoclonal |
| D000906 | Antibodies |
| D007136 | Immunoglobulins |
| D007162 | Immunoproteins |
| D001798 | Blood Proteins |
| D011506 | Proteins |
| D000602 | Amino Acids, Peptides, and Proteins |
| D012712 | Serum Globulins |
| D005916 | Globulins |
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