An observational study in Dermatology Disease, sponsored by University Hospital, Rouen. Completed at 1 site in France. Open to participants aged 18 Years and older. Per ClinicalTrials.gov, last updated 2025-03-30.
Sponsored by University Hospital, Rouen · Observational
In May 2018, the PNDS (national diagnosis and care protocol) for pemphigus drawn up by the autoimmune bullous diseases reference centre was amended to take into account the results of recent studies and proposed, in line with the rituximab as 1st-line treatment: 1g repeated at 15-day intervals, then in the event of complete clinical remission, at 6 months: an infusion of 500mg or 1g in patients with initially severe pemphigus and/or who still have a high level of anti desmogleins at 3 months. The primary objective of the study is to prospectively evaluate the relapse rate during the first year of patients with newly diagnosed pemphigus treated according to the recommendations of the revised PNDS.
Pemphigus is a rare, potentially life-threatening autoimmune bullous disease affecting both the skin and mucous membranes in which pathogenic IgG antibodies are directed against desmosomal transmembrane desmoglein (Dsg) 1 and 3. The initial severity of lesions in pemphigus correlates with levels of serum anti-Dsg antibodies anti-Dsg 1 and anti-Dsg 3 serum antibody levels, respectively. High doses of oral corticosteroids (OCs), sometimes combined with immunosuppressive drugs (azathioprine, mycophenolate mofetil) have been the mainstay of pemphigus treatment for many years for many years.
Over the last decade, more than 1,000 patients with recalcitrant or relapsing pemphigus have been treated pemphigus treated with rituximab (anti-CD20 monoclonal antibody) have been monoclonal antibody) have been reported in the literature.
The efficacy and safety of rituximab as a first-line treatment for patients with moderate to severe forms of pemphigus were recently evaluated in a large randomised controlled trial. Two predictive factors for early relapse that can be easily used in practice were identified, with a positive predictive value of 50% and a negative predictive value of 94% (initial PDAI severity score >45 and anti-desmoglein 1 >20 IU/ml and/or anti-desmoglein 3 >130 IU/ml at M3 after the first rituximab infusion).
Thus, the probability of an early relapse in patients presenting at least one of these 2 clinico-immunological criteria is 50%, whereas the probability of not relapsing in patients with none of these criteria is 94%.
In May 2018, the PNDS (national diagnosis and care protocol) for pemphigus drawn up by the autoimmune bullous diseases reference centre was amended to take into account the results of recent studies and proposed, in line with the rituximab as 1st-line treatment: 1g repeated at 15-day intervals, then in the event of complete clinical remission, at 6 months: an infusion of 500mg or 1g in patients with initially severe pemphigus and/or who still have a high level of anti desmogleins at 3 months. The primary objective of the study is to prospectively evaluate the relapse rate during the first year of patients with newly diagnosed pemphigus treated according to the recommendations of the revised PNDS.
76 studies on the registry are indexed under Pemphigus; 18 are open to participants now.
This study's enrollment of 100 is above the median of 60 across 21 observational studies indexed under Pemphigus.
Browse Pemphigus studies →University Hospital, Rouen is the lead sponsor of 418 studies on the registry; 106 are open to participants now.
Counted across the registry records on this site, refreshed daily.
Consecutive patients with newly diagnosed pemphigus treated as 1st-line patients with rituximab according to the recommendations of the revised PNDS,
Go through the criteria one by one — yes, no or not sure — and take the list to your doctor or the study team. Kept in this browser only.
Pemphigus newly diagnosed according to the following criteria:
Exclusion Criteria:
Underlined terms in the criteria open these too. Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
A line of therapy is one course of treatment, which may combine several drugs. First-line is the first course given; second-line follows when that stops working or is not tolerated.
“Two prior lines” means two courses that count separately under the study’s rules — not simply two drugs. Ask the study team if you are unsure how yours are counted.
Anti-inflammatory medicines such as prednisone, prednisolone and dexamethasone — not the anabolic steroids used for muscle.
Studies differ in what they allow: check which steroid medicines, routes (tablets, injections, creams, inhalers, eye drops) and doses this study accepts.
Line of therapy
A line of therapy is one course of treatment, which may combine several drugs. First-line is the first course given; second-line follows when that stops working or is not tolerated.
“Two prior lines” means two courses that count separately under the study’s rules — not simply two drugs. Ask the study team if you are unsure how yours are counted.
Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
Corticosteroids (steroids)
Anti-inflammatory medicines such as prednisone, prednisolone and dexamethasone — not the anabolic steroids used for muscle.
Studies differ in what they allow: check which steroid medicines, routes (tablets, injections, creams, inhalers, eye drops) and doses this study accepts.
Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
Relapse rate over one year
The primary objective of the study was to prospectively evaluate the relapse rate during the first year of patients with newly diagnosed pemphigus treated according to the recommendations of the revised PNDS. Relapse' is defined as 'the reappearance of at least three new lesions per month, which do not heal spontaneously within one week or by the extension of old lesions in a patient who had previously achieved disease control.
Time frame: 1 year
Evaluation of prognostic factors for short-term relapse (no maintenance infusion at 6 month))
Determining no maintenance infusion at 6 month after after starting treatment by rituximab In patients with no risk factors for relapse .
Time frame: 6 month
Prognostic factors for short-term relapse (Tolerability of the treatment regimen proposed in the revised PNDS)
Assessment of severe and non-severe side effects during the 1st year of treatment
Time frame: 1 year
Plan to share: No — The data provided will be the property of the sponsor and will be used solely for its own research activities.
No publications or documents are linked to this record.
This study is completed, as verified in Mar 2024. You cannot join it, but the record below documents what was studied.
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