Lupus
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The objective of this study was to characterize risk factors for thrombotic events in lupus patients. A total of 272 lupus patients were followed up for five years during which the presence of aPL antibodies [anticardiolipin (aCL), anti-beta2-glycoprotein I (abeta2GPI) and lupus anticoagulant (LAC)] were determined, and all thrombotic incidents and antithrombotic therapy-related data were collected. At baseline, three groups were constituted, an aPL- group with 107 aPL negative patients, an aPL+ group with 81 aPL positive patients without clinical thrombosis and a secondary antiphospholipid syndrome (APS) group with 84 aPL+ patients who met the Sapporo criteria. ⋯ In the aPL+ group, thrombotic events reoccurred in 1.9% of those receiving antithrombotic prophylaxis and 6.9% of those without primary prophylaxis. Despite anticoagulant therapy, thrombotic events reoccurred in 8.3% of the APS group. These findings indicate that LAC, constant and cumulative presence of aPL and previous thrombosis are positive predictors for the development of thrombotic complication in lupus patients.
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Pancreatitis is a relatively rare complication in systemic lupus erythematosus (SLE). Here we present a case of SLE associated with autoimmune pancreatitis. A 37-year old woman was admitted to our hospital because of fever, skin rash, proteinuria and abdominal pain. ⋯ Systemic administration of glucocorticoid successfully induced remission of pancreatitis and nephritis along with the reduction of IgG4. Autoimmune pancreatitis is a newly recognized type of pancreatitis, in which IgG4 immune response is thought to participate pathophysiologically. Although the disease has been observed to develop in patients having various connective tissue diseases, our report is the first to describe IgG4-related autoimmune pancreatitis in a patient with SLE.
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Review Case Reports
Development of hairy cell leukemia in a patient with antiphospholipid syndrome.
We present a young woman who was diagnosed as primary antiphospholipid syndrome (deep vein thrombosis and pulmonary embolism in 1999; moderate thrombocytopenia with high-positive anticardiolipin ELISA tests in 2002, and cerebral thrombosis in 2003), and then developed hairy cell leukemia (massive splenomegaly, neutropenia, hairy cells in blood smear and bone marrow trephine biopsy in 2004). A partial remission was achieved with interferon-alpha 2a therapy. After the initiation of 2-chloro-deoxyadenosine therapy, splenomegaly disappeared, the percentage of hairy cells on the bone marrow reduced below 1%, platelet count returned to normal levels. ⋯ In our literature search, we found only six cases that had both antiphospholipid antibodies and hairy cell leukemia. Our case is the first case of antiphospholipid syndrome before the development of hairy cell leukemia. Both hairy cell leukemia and antiphospholipid syndrome responded to lymphocytotoxic treatment with 2-chloro-deoxyadenosine.