Lupus thrombocytopenia: pathogenesis and therapeutic implications

Systemic Lupus Erythematosus (SLE) is frequently complicated by cytopenias. Thrombocytopenia is usually non severe and its frequency ranges from 20% to 40%. It is mostly an autoimmune process caused by autoantibodies against platelet surface glycoproteins and it is associated with worse prognosis in SLE. It can also be a result of SLE treatment with azathioprine, methotrexate and rarely hydroxychloroquine or thrombotic microangiopathy or macrophage activation syndrome. If thrombocytopenia is mild (>50×10⁹/L) and there is no other evidence of disease there is no need of therapy. Severe thrombocytopenia is less frequent and needs therapeutic management. Corticosteroids are the cornerstone of therapy. Continuous high dose oral prednisolone or pulse high dose methylprednisolone (MP) with or without intravenous immune globulin are used in the acute phase. Second line agents (hydroxychloroquine, danazol, azathioprine, cyclosporine, mycophenolate mofetil, cyclophosphamide, rituximab) are usually needed. Splenectomy is indicated for recurrent or resistant cases. There are no evidence-based guidelines to facilitate selection of one drug over another but certainly the co-existence of other systemic SLE manifestations must be taken into account. Newer therapies are emerging although there is no consensus on the treatment of refractory lupus thrombocytopenia due to the absence of controlled randomized trials.