title: Efficacy and Safety of Intravenous Immunoglobulin Treatment in Refractory Behcet's Disease with Different Organ Involvement: A Case Series.
abstract: Behçet's disease (BD) is a multi-systemic disorder of unknown etiology characterized by relapsing oral-genital ulcers, uveitis, and involvement of the articular, gastrointestinal, neurologic, and vascular systems. The choice of treatment is based on the severity of systemic involvement, clinical presentation and the site affected, and includes corticosteroids, azathioprine, interferon, cyclophosphamide, methotrexate or tumor necrosis factor-alpha and interleukin-1 blockers. We present a case series of four refractory BD patients successfully treated with intravenous immunoglobulins (IVIG). All patients fulfilled International Study Group criteria. The patients' mean age was 38.75 ± 12.09 years and mean disease duration 10.25 ± 8.5 years. Human leukocyte antigen B51 was positive in two of four patients. In addition to oral aphthosis, all patients suffered from genital ulcers and cutaneous BD-related manifestations; central nervous system involvement and arthralgia were found in two patients. Peripheral nervous system, gastrointestinal and eye involvement occurred in 25% of cases. In all patients, previously treated according to EULAR recommendations without reaching satisfactory results, IVIG induced immediate and sustained response over time without incurring any side effects. We propose IVIG administration as an additional effective and safe treatment option in patients with severe and resistant BD.
authors.forename: Luca
authors.initials: L
authors.lastname: Cantarini
authors.forename: Maria L
authors.initials: ML
authors.lastname: Stromillo
authors.forename: Antonio
authors.initials: A
authors.lastname: Vitale
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Lopalco
authors.forename: Giacomo
authors.initials: G
authors.lastname: Emmi
authors.forename: Elena
authors.initials: E
authors.lastname: Silvestri
authors.forename: Antonio
authors.initials: A
authors.lastname: Federico
authors.forename: Mauro
authors.initials: M
authors.lastname: Galeazzi
authors.forename: Florenzo
authors.initials: F
authors.lastname: Iannone
authors.forename: Nicola
authors.initials: N
authors.lastname: De Stefano
mesh_terms: D061605:Administration, Intravenous; D000328:Adult; D018501:Antirheumatic Agents / Q000145:classification / Q000627:therapeutic use; D001528:Behcet Syndrome / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology / Q000503:physiopathology; D004351:Drug Resistance; D005260:Female; D059949:HLA-B51 Antigen / Q000032:analysis; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage*; D008297:Male; D008875:Middle Aged; D055502:Secondary Prevention / Q000379:methods; D012720:Severity of Illness Index; D063189:Symptom Assessment; D016896:Treatment Outcome
chemical_list: D018501:Antirheumatic Agents; D059949:HLA-B51 Antigen; D016756:Immunoglobulins, Intravenous