ABSTRACT
This report considers the rare situation in which primary antiphospholipid syndrome (PAPS) is linked with thrombotic thrombocytopenic purpura (TTP). It describes the case of a young lady with PAPS, characterized by recurring cerebro-vascular abnormalities and marked livedo reticularis, combined with circulating anticardiolipin and lupus anticoagulant antibodies. On follow-up, while on oral anticoagulation, she developed severe thrombocytopenia associated with hematuria, microangiophatic anaemia and neurological manifestations consistent with a diagnosis of TTP. The patient was treated with pulses of methylprednisolone and plasmapheresis with plasma exchange. The result was a favourable outcome. To our knowledge, this is the seventh report on this rare association in the English-language literature of this field.
Subject(s)
Antiphospholipid Syndrome/complications , Antiphospholipid Syndrome/diagnosis , Purpura, Thrombotic Thrombocytopenic/complications , Purpura, Thrombotic Thrombocytopenic/diagnosis , Adult , Antibodies/blood , Antiphospholipid Syndrome/therapy , Cardiolipins/immunology , Female , Glucocorticoids/therapeutic use , Humans , Lupus Coagulation Inhibitor/immunology , Methylprednisolone/therapeutic use , Plasmapheresis , Purpura, Thrombotic Thrombocytopenic/therapy , Treatment OutcomeABSTRACT
Systemic sclerosis (SS) is a chronic, multisystemic disease, characterized by inflammation associated with fibrosis. Calcinosis is one of the manifestations of this disorder, observed in 10 to 20% of cases. It is usually located on the extensor surface of the phalanges, peri-articular tissue and near the bone prominences. There are only a few cases reported of SS with vertebral column involvement and spinal cord compression by calcinosis causing serious neurological complications. We describe a fatal case of SS who showed tetraplegia secondary to ectopic calcinosis in the cervical medulla and present a review of the literature on the subject.
