Evaluation of Glucocorticoids Plus Rituximab in Patients with Newly-Diagnosed or Relapsing IgA Vasculitis

Inclusion Criteria:

• Biopsy-proven diagnosis of IgAV according to Chapel Hill Consensus Conferencedefinitions

• Patient aged of 18 years or older

• Patients with newly-diagnosed disease or relapsing disease at the time of screening,with an active disease defined by active manifestations attributable to IgAV

• Patients with severe involvement of at least one organ

• Patients within the first 21 days following initiation/increase of glucocorticoidsat a dose < 1 mg/kg/day

• Has signed an informed consent form prior to any study related procedures

• Affiliated to a national health insurance

Exclusion Criteria:

• Patients with ANCA-associated vasculitis, or other vasculitis, defined by the ACRcriteria and/or the Chapel Hill Consensus Conference,

• Patients with IgAV in remission of the disease,

• Patients with severe cardiac failure defined as class IV in New York HeartAssociation,

• Patients with severe, uncontrolled cardiac disease,

• Patients with acute infections or chronic active infections (including HIV, HBV orHCV),

• Patients with active cancer or recent malignancy (<5 years), except basocellularcarcinoma and prostatic cancer of low activity controlled by hormonal treatment,

• Pregnant women and breastfeeding. Patients with childbearing potential must usereliable contraceptive methods throughout the study and at least for 12 months afterthe last study drug administration,

• Patients with IgAV who have already been treated with rituximab within the previous 12 months,

• Patients treated with immunosuppressive therapy within the last 3 months,

• Patients with hypersensitivity to human or chimeric monoclonal antibodies,

• Patients with contraindication to use rituximab,

• Patients treated with any concomitant drugs contraindicated for use with therituximab according to its SmPC,

• Patients with contraindication to use routine care treatments (Glucocorticoids,Angiotensin-converting-enzyme (ACEis) or angiotensin receptor blockers (ARBs),dexchlorphéniramine),

• Patients in a severely immunocompromised state,

• Patients with other uncontrolled diseases, including drug or alcohol abuse, severepsychiatric disorders, that could interfere with his/her compliance to protocolrequirements,

• Patients currently participating in another clinical study or 3 months prior torandomization,

• Patients suspected not to be observant to the proposed treatments,

• Patients unable to give written informed consent prior to participation in the study

• Being deprived of liberty or under guardianship.