1/46. Cryosupernatant plasma exchange in the treatment of antiphospholipid antibody syndrome with lupus nephritis.We report a case of a 22-year-old female with antiphospholipid antibody syndrome (APS) associated with systemic lupus erythematosus in whom cryosupernatant plasma exchange was effective and improved both the refractory venous thrombosis in her legs and relapsing thrombocytopenia. A renal biopsy specimen showed not only features of active lupus nephritis but also renal arteriolar thrombosis which is considered to be a type of thrombotic microangiopathy (TMA). Because a pathological role of unusually large von willebrand factor (vWF) multimers has been reported in patients with TMA including thrombotic thrombocytopenic purpura, plasma exchange using replacement with cryosupernatant, which is free of unusually large vWF multimers, is likely to be an option of treatment modality for patients with refractory and chronic relapsing APS manifesting TMA.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus (Clic here for more details about this article) |
2/46. Death due to thrombotic thrombocytopenic purpura in pregnancy: case report with review of thrombotic microangiopathies of pregnancy.maternal death during pregnancy, although uncommon, may result from a broad range of conditions. In this paper, a case of thrombotic thrombocytopenic purpura diagnosed by postmortem examination is presented. Thrombotic thrombocytopenic purpura is one of a subset of diseases that result in the formation of microthrombi within the vasculature, either as a primary or secondary manifestation. Other conditions included in the differential diagnosis during pregnancy are hemolytic uremic syndrome, systemic lupus erythematosus, preeclampsia-eclampsia and the hellp syndrome, acute fatty liver of pregnancy, antiphospholipid antibody syndrome, and disseminated intravascular coagulation. The histologic manifestations of these diseases can be similar and in most cases do not provide adequate information to accurately differentiate these diseases in the postmortem period. This paper addresses the need for clinical history (i.e., symptomatology, trimester of onset) and antemortem laboratory testing in addition to a thorough autopsy to accurately differentiate among the conditions named previously. In the absence of an adequate clinical history and antemortem laboratory testing, the more general diagnosis of "thrombotic microangiopathy of pregnancy" is acceptable.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus (Clic here for more details about this article) |
3/46. Use of cyclophosphamide in the treatment of thrombotic thrombocytopenic purpura complicating systemic lupus erythematosus: report of two cases.Thrombotic thrombocytopenic purpura (TTP) is an unusual complication of systemic lupus erythematosus (SLE). There are no randomized, prospective studies of its treatment. The association of plasma infusions and (or) plasmapheresis with steroids improves survival when compared with steroid treatment alone, but these patients still have a higher mortality than those with "classic" TTP. The role of immunosuppressive drugs in the management of this disorder remains uncertain. We report two cases of TTP in SLE which presumably benefited from the addition of cyclophosphamide to the treatment with plasmapheresis and steroids.- - - - - - - - - - ranking = 4.7804856924658keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus (Clic here for more details about this article) |
4/46. Close relationship between systemic lupus erythematosus and thrombotic thrombocytopenic purpura in childhood.OBJECTIVE: To determine the association between childhood-onset thrombotic thrombocytopenic purpura (TTP) and systemic lupus erythematosus (SLE). methods: The charts of all 5 patients diagnosed with idiopathic TTP at the Hospital for Sick Children (HSC) in Toronto from 1975 to 1998, and all cases of childhood-onset TTP (ages 6-20 years) reported in the literature over the same period were reviewed. Fourteen of the 44 patients identified in the literature were excluded from the analysis for lack of clinical and laboratory information. The remaining 35 patients were grouped into either an SLE/TTP group or a TTP only group, according to the presence or absence of the American College of rheumatology (ACR) classification criteria for SLE. The groups were compared for differences in clinical or laboratory features. RESULTS: The clinical presentation and initial disease course of pediatric patients with TTP were similar to those observed in adults. Of the 35 patients with childhood-onset TTP included in this review, 9 (26%) fulfilled > or = 4 ACR criteria for SLE and 8 (23%) were found to have incipient SLE. Of the 5 patients initially diagnosed with idiopathic TTP at the HSC, 3 were diagnosed with SLE within 3 years, and the other 2 patients fulfilled 3 ACR classification criteria for SLE within 4 years of disease onset. The clinical syndrome of pediatric TTP presenting with proteinuria, especially with high-grade proteinuria, was significantly associated with the development or coexistence of childhood-onset SLE. CONCLUSION: TTP in childhood is a rare, but life-threatening, disease. Unlike in adults, TTP in childhood is commonly associated with SLE. High-grade proteinuria at diagnosis of TTP is the best predictor for the presence or subsequent development of SLE.- - - - - - - - - - ranking = 5keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus (Clic here for more details about this article) |
5/46. Small vessel thrombosis without major thrombotic events in systemic lupus erythematosus patients with antiphospholipid syndrome.antiphospholipid syndrome has been defined by the presence of antiphospholipid antibodies or lupus anticoagulant in association with certain clinical events, including recurrent arterial or venous thromboses and recurrent fetal loss. It comprises two separate clinical entities: simple, characterized by large vessel occlusions, and catastrophic, with multiple occlusive events predominantly affecting small vessels. Three patients with systemic lupus erythematosus and permanently increased IgG anticardiolipin antibody levels are being described. Only postmortem histopathological examination revealed microangiopathic thrombotic changes in different organs, which were clinically silent in early stages of the disease and misinterpreted later in its course because of a peculiar clinical picture. All patients presented features of catastrophic antiphospholipid syndrome in the final stage of the disease.- - - - - - - - - - ranking = 5keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus (Clic here for more details about this article) |
6/46. Progressive appearance of overlap syndrome together with autoantibodies in a patient with fatal thrombotic microangiopathy.We describe an extraordinary patient with overlap syndrome (systemic lupus erythematosus, systemic sclerosis, and rheumatoid arthritis) having positive autoantibodies against Sm, double stranded dna, dna topoisomerase I, and centromere, together with rheumatoid factor. The patient had multiple organ involvement resulting from thrombotic microangiopathy that mimicked so-called normotensive scleroderma renal crisis, and died mainly of massive pulmonary hemorrhage caused by thrombotic thrombocytopenic purpura. The clinical presentations of the case support the concept of strong associations between disease-specific autoantibodies and clinical features.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus (Clic here for more details about this article) |
7/46. Thrombotic thrombocytopenic purpura in autoimmune hepatitis.A 19-year-old woman presented with clinical manifestations of sudden, fulminant thrombotic thrombocytopenic purpura associated with autoimmune hepatitis and autoimmune thrombocytopenic purpura. Although thrombotic thrombocytopenic purpura may, rarely, be associated with systemic lupus erythematosus and other autoimmune diseases, to our knowledge, the syndrome has never been described in association with autoimmune hepatitis. In this patient, too, the etiology of thrombotic thrombocytopenic purpura associated with autoimmune disease remains elusive. The patient was treated with corticosteroid, which brought about no improvement in her condition, and she died of multiorgan failure. diagnosis is challenging, but prompt diagnosis is necessary because thrombotic thrombocytopenic purpura is a life-threatening syndrome whose prognosis has been improved significantly by early plasmapheresis treatment.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus (Clic here for more details about this article) |
8/46. Systemic lupus erythematosus and thrombotic thrombocytopenic purpura: a case and review.Thrombotic thrombocytopenic purpura (TTP) is rare in children with systemic lupus erythematosus (SLE). We report a 15-year-old female who simultaneously presented with TTP and SLE. kidney biopsy showed membranous nephropathy. Her condition improved with plasmapheresis, intravenous cyclophosphamide, and prednisone pulse therapy. We also reviewed the literature for this association in pediatric patients comparing presenting sequence and renal pathology with cases documented in the adult literature.- - - - - - - - - - ranking = 4.1219427698632keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus (Clic here for more details about this article) |
9/46. Thrombotic thrombocytopenic purpura and systemic lupus erythematosus.Thrombotic thrombocytopenic purpura (TTP) is a rarely seen complicating systemic lupus erythematosus (SLE). The diagnosis of TTP in a setting of SLE is challenging since both share common features including thrombotic microangiopathy. We report two cases of SLE with TTP one with a good response when cyclophosphamide was given early with plasmapheresis and steroids; the other with a poor outcome in a patient given cyclophosphamide late in the course of the disease.- - - - - - - - - - ranking = 4.7804856924658keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus (Clic here for more details about this article) |
10/46. Surge of anti-SS-A antibody associated with fulminant thrombotic thrombocytopenic purpura in pregnancy.Thrombotic thrombocytopenic purpura (TTP) is an uncommon clinical syndrome and is rarely associated with systemic lupus erythematosus (SLE). diagnosis of TTP in patients with SLE, especially those who are pregnant, is challenging. We report the case of a pregnant woman with a high level of anti-SS-A antibody (162,143 U/mL) and fulminant TTP. The patient responded to plasma exchange treatment. Recent studies indicate that patients with SLE and another serologic abnormality, such as the presence of antiphospholipid antibody, may be at high risk for TTP. We explore the possible pathogenesis of acute TTP in patients with SLE and summarize the risk factors for acute TTP in patients with SLE and the current treatments for SLE-associated TTP.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus (Clic here for more details about this article) |
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