An uncommon association of antiphospholipid syndrome, selective IgA deficiency and resistant-to-treatment relapsing polychondritis: efficacy of infliximab

D. Firinu, A. Frau, M. Pisanu, M.M. Lorai, R. Meleddu, F. Musu, P.E. Manconi, S.R.Del Giacco

Article ID: 6189
Vol 26, Issue 4, 2012
DOI: https://doi.org/10.54517/jbrha6189
Received: 8 January 2013; Accepted: 8 January 2013; Available online: 8 January 2013; Issue release: 8 January 2013

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.


Keywords

IgA deficiency;autoimmunity;relapsing polychondritis;Antiphospholipid syndrome;heparin-induced thrombocytopenia;infliximab;treatment


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