Intravenous Immunoglobulins for the Treatment of Primary Sjögren's Syndrome Associated Painful Sensory Neuropathies

Inclusion criteria

• Age ≥ 18 years and < 80 years
• Primary Sjögren's syndrome defined as per the European and American criteria (5)
• Peripheral neuropathy clinically defined:

• Pure sensitive (lymph node disease) or sensorimotor neuropathies
• Proved EMG
• Renal function, and viral evaluation (VIH and hepatitis serology) :

*Clairance > 50 (In case of biological abnormality, the second dosage can be scheduled within 2 weeks)

• Effective contraception during the study period
• Patient capable of understanding information about the study and of giving his/her consent
• Patient informed of the preliminary medical exam results
• Patient with healthcare insurance
• Written consent signed

Exclusion criteria

• Peripheral neurological damage of the type vascularitis-related multiplex mononeuropathy
• Small fibers neuropathy
• Neuropathy suspected of being related to alcohol, diabetes or post-chemotherapy
• Chronic viral infection (HCV, HBV, HIV, etc.)
• Prior treatment with polyvalent intravenous immunoglobulins in the 6 months preceding the study
• Corticosteroid treatment at a dose greater than 20 mg/d of prednisone equivalent or no stable dose for at least 1 month before inclusion
• Conventional immunosuppressant treatment with azathioprin, cyclophosphamide or mycophenolate mofetil on-going or interrupted less than one month before inclusion
• Rituximab or other biotherapy (belimumab, tocilizumab, …) less than 6 months before the start of the study treatment
• Immunomodulating treatment with methotrexate no stable dose for at least 2 months before inclusion
• Hydroxychloroquine no stable dose for at least 3 months before inclusion
• Pilocarpine hydrochloride secretagogue treatment no stable dose for at least one month before inclusion
• Treatment with amitriptyline, clomipramine, carbamazepine, clonazepam, pregabaline, duloxetine or gabapentine if the dose has not been stable for at least one month before inclusion (possible dose reduction to be documented).
• renal clairance < 50 ml/mn
• HIV seropositivity
• HBV, or HCV viral replication
• Contraindication to the use of IV Ig: h Hypersensitivity to the active substance or to any of the excipients; hypersensitivity to human immunoglobulins, especially in patients with antibodies against IgA; patients with hyperprolinaemia.
• Contraindication to the use of Nacl
• Immunization with live attenuated vaccine within 2 weeks prior to inclusion
• Participation in a clinical study with an investigational product with an exclusion period
• Women of child bearing potential or intends to become pregnant, unless they are using an effective method of birth control* and a βHCG blood test negative
• Pregnant or nursing (lactating) women
• Patient under legal guardianship
• Prisoners