title: An uncommon association of antiphospholipid syndrome, selective IgA deficiency and resistant-to-treatment relapsing polychondritis: efficacy of infliximab.
abstract: Autoimmune complications in the context of primary immunodeficiency diseases represent a well-known phenomenon, and this is widely recognized also for Selective Immunoglobulin A deficiency (IgAD), the most common primary antibody deficiency (PAD). Relapsing polychondritis (RP) is a rare immune-mediated, difficult to treat, disorder in which the cartilaginous tissues are the target for inflammation and damage. Ocular inflammatory manifestations in RP are frequent and often sight-threatening. Antiphospholipid syndrome (APS) is an acquired prothrombotic state related to circulating autoantibodies against phospholipids and/or their cofactors. Rare reports of APS associated to RP, PAD and APS or PAD and RP are available.
authors.forename: D
authors.initials: D
authors.lastname: Firinu
authors.forename: A
authors.initials: A
authors.lastname: Frau
authors.forename: M
authors.initials: M
authors.lastname: Pisanu
authors.forename: M M
authors.initials: MM
authors.lastname: Lorai
authors.forename: R
authors.initials: R
authors.lastname: Meleddu
authors.forename: F
authors.initials: F
authors.lastname: Musu
authors.forename: P E
authors.initials: PE
authors.lastname: Manconi
authors.forename: S R
authors.initials: SR
authors.lastname: Del Giacco
mesh_terms: D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D016736:Antiphospholipid Syndrome / Q000150:complications*; D005260:Female; D006801:Humans; D017098:IgA Deficiency / Q000150:complications*; D000069285:Infliximab; D008875:Middle Aged; D011081:Polychondritis, Relapsing / Q000188:drug therapy*; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors*
chemical_list: D000911:Antibodies, Monoclonal; D014409:Tumor Necrosis Factor-alpha; D000069285:Infliximab