Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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.
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PMID:Death due to thrombotic thrombocytopenic purpura in pregnancy: case report with review of thrombotic microangiopathies of pregnancy. 1041 63

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.
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PMID:Use of cyclophosphamide in the treatment of thrombotic thrombocytopenic purpura complicating systemic lupus erythematosus: report of two cases. 1042 32

A 20-year-old woman visited a nearby hospital because of sudden, severe, and unusual genital bleeding. She also exhibited severe anemia and thrombocytopenia. In transit to our hospital, the patient suddenly suffered cardiac arrest and died soon thereafter despite immediate blood transfusion and therapeutic intubation. Thrombotic thrombocytopenic purpura (TTP) was initially diagnosed at autopsy due to the observation of numerous fragmented erythrocytes in peripheral blood, evidence of hemolysis, and thrombotic microangiopathy in multiple organs. In addition, histopathologic and serologic findings disclosed an association with systemic lupus erythematosus (SLE). Test for anticardiolipin antibody was positive, and hemophagocytic findings were detected in lymph node specimens. Reports of TTP in association with SLE have been increasing in recent years. However, the mechanisms correlating these two illnesses have not been identified. We speculated that the rapid clinical course in this case was attributable to TTP that had been provoked by endothelial microangiopathy due to SLE, and moreover, the fact that the patient's general condition had been seriously complicated by excessive menstrual bleeding.
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PMID:[Systemic lupus erythematosus presenting as fulminant thrombotic thrombocytopenic purpura]. 1049 44

Thrombotic thrombocytopenic purpura (TTP) is a clinical syndrome characterized by microangiopathic hemolytic anemia, thrombocytopenia, fever, neurological symptoms, and renal involvement. The relationship of TTP to systemic lupus erythematosus (SLE) has been recognized in the medical literature since 1939. The differential diagnosis is difficult because both diseases have similar clinical features. The mainstay for recognizing TTP in the context of active SLE is the presence of helmet red cells, marked reticulocytosis, and negative direct Coomb's test. We report three female patients with simultaneous presentation of TTP and SLE. We suggest combined treatment with immunosuppressive therapy and plasma exchange using fresh frozen plasma.
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PMID:[Thrombotic thrombocytopenic purpura and systemic lupus erythematous. Three cases presenting simultaneously]. 1075 18

The aim of our work is the focusing on some aspects about both the etiopathogenesis of thrombotic thrombocytopenic purpura and the therapeutic choices required to strongly reduce the mortality. Moreover the article reviews the association between thrombotic thrombocytopenic purpura and systemic lupus erythematosus. Thrombotic thrombocytopenic purpura is a rare and severe hematologic syndrome, first described in 1924, characterized by a clinical pentade: fever, microangiopathic anemia, thrombocytopenia, neurologic abnormalities and renal involvement. It is unknown if the endothelial damage represents the first lesion or if the platelet hyperaggregability precedes the vascular injury. Some data suggest a possible role of immune mechanisms in the development of the disease, that may be associated in some cases with autoimmune disorders. To our knowledge 31 cases of association between thrombotic thrombocytopenic purpura and systemic lupus erythematosus are reported in the English literature from 1970 today; the link between the two diseases is unclear. The authors review these cases with particular care to the diagnosis, that may be very difficult, and the therapy.
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PMID:[Thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus. Clinical diagnosis and therapeutic strategies]. 1107 42

PP is a safe, expensive, labor-intensive procedure. Its absolute SLE indications include hyperviscosity, cryoglobulinemia, pulmonary hemorrhage and TTP. PP may be useful in cyclophosphamide-resistant, serious, organ-threatening disease. It may be potentially useful in the antiphospholipid syndrome or mothers or children at risk for congenital heart block. Refinements in apheresis technology may expand the indications for PP.
Lupus 2001
PMID:Apheresis for lupus erythematosus: state of the art. 1131 51

Autologous stem cell transplantation (HSCT) has been shown to be effective in curing a large spectrum of autoimmune disorders. Case reports are being collected in the EBMT/EULAR Autoimmune Disease Stem Cell Project registry, which reports transplant-related mortality (TRM) of 6%. In order to reduce TRM and preserve the anti-autoimmune effect we evaluated a more immunoablative as opposed to myeloablative conditioning regimen for the autotransplant of severe immunomediated diseases. We enrolled patients affected by systemic lupus erythematosus (SLE: 3 patients), by autoimmune thrombocytopenic purpura (AITP: one patient), by thrombotic thrombocytopenic purpura (TTP: one patient), by pure red cell aplasia (PRCA: one patient), and by a severe cryoglobulinemia (one patient). All patients were mobilized with cyclophosphamide (Cy) 4 g/m2 + G-csf. Conditioning regimen consisted of Cy 50 mg/kg/day (days -6 and -5); anti-T-globulin (ATG) 10 mg/kg/day and 6-methylprednisolone (PDN) 1 g/day (days -4, -3, and -2). Immunomagnetically selected CD34+ cells were re-infused on day 0. In three patients neutrophil count fell below 0.5 x 10(9)/l, while a PLT count below 20 x 10(9)/l was registered in two patients. Extrahematological toxicity was very low. Four patients (2 SLE, 1 TTP, 1 cryoglobulinemia) are in complete corticosteroid-free remission with a median follow up of 335 days. The third SLE patient improved considerably; however, he still needs low-dose corticosteroid maintenance. The AITP and PRCA patients achieved a CR but soon relapsed; nevertheless, the procedure restored a steroid-sensitive status. The use of this immunoablative conditioning regimen in auto-HSCT transplant was shown to be effective in controlling disease progression and could be a valuable strategy in reducing TRM.
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PMID:Intense immunosuppressive therapy followed by autologous peripheral blood selected progenitor cell reinfusion for severe autoimmune disease. 1142 2

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.
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PMID:Systemic lupus erythematosus and thrombotic thrombocytopenic purpura: a case and review. 1151

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.
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PMID:Thrombotic thrombocytopenic purpura and systemic lupus erythematosus. 1172 47

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.
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PMID:Surge of anti-SS-A antibody associated with fulminant thrombotic thrombocytopenic purpura in pregnancy. 1181 65


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