Cases reported "Catastrophic Illness"

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1/4. Involvement of the entire spinal cord and medulla oblongata in acute catastrophic-onset transverse myelitis in SLE.

    A 30-year-old Caucasian male with systemic lupus erythematosus suffered acute catastrophic-onset transverse myelitis. Two years earlier aseptic meningitis, another rare CNS lupus manifestation, had been diagnosed. MRI showed involvement of the medulla oblongata and the entire spinal cord. Therapy with intravenous high-dose methylprednisolone and intravenous cyclophosphamide is discussed.
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ranking = 1
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus
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2/4. Catastrophic antiphospholipid antibody syndrome in systemic lupus erythematosus: an autopsy case report of a young woman.

    Catastrophic antiphospholipid syndrome (CAPS) is a severe variant of antiphospholipid syndrome (APS) characterized by disseminated microangiopathy that results in multiorgan failure. CAPS mainly occurs in association with systemic lupus erythematosus (SLE). Clinically, CAPS mimics disseminated SLE vasculitis, intravascular coagulation (DIC), and particularly thrombotic thrombocytopenic purpura (TTP). We describe an autopsy case of young woman with CAPS in SLE, which is difficult to differentiate from TTP secondary to SLE.
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ranking = 4.7771578385823
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus
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3/4. Catastrophic antiphospholipid syndrome; a "CATASTROPHIC" case of systemic lupus erythematosus.

    Less than 1% of patients with the antiphospholipid antibody syndrome (APS) can develop multiple, simultaneous organ-system thromboembolic disease, which is referred to as the catastrophic antiphospholipid antibody syndrome (CAPS). Roughly one-half of these patients have systemic lupus erythematosus (SLE). Factors known to precipitate CAPS include infection, surgery, trauma, neoplasia, anticoagulation withdrawal, obstetric complications, and SLE flares. Optimal treatment includes anticoagulation and high-dose corticosteroids, although IVIG and plasma exchange may also have a role. The overall mortality of CAPS is roughly 50%, but prompt clinical recognition of this rare but devastating syndrome may lead to improved outcomes.
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ranking = 4.7771578385823
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus
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4/4. Catastrophic antiphospholipid antibody syndrome.

    Antiphospholipid antibody syndrome (APS) is characterized by recurrent thrombosis with the presence of circulating antiphospholipid antibodies. A diagnosis of APS requires the presence of at least one clinical and one laboratory criteria (detection of aCL IgG or IgM antibodies or the presence of lupus anticoagulant on two or more consecutive occasions 6 weeks apart). A severe, rapidly progressive form characterized by clinical involvement of at least three different organ systems with histopathological evidence of small and large vessel occlusion is termed catastrophic antiphospholipid syndrome. Early recognition of APS is crucial since aggressive management can result in a favorable outcome. We present the case of a 12-year-old boy who presented with a devastating illness with multiple thrombotic episodes and rapidly progressive renal failure.
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ranking = 0.044568432283537
keywords = lupus
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