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)

Arterial thrombotic events, thrombocytopenia, and hemolytic anemia with schistocytes may be encountered in the setting of both thrombotic thrombocytopenic purpura (TTP) and primary antiphospholipid syndrome (APS). We report 2 cases of TTP occurring in patients with definite primary APS. We also describe the results of tests for ADAMTS-13 activity in 20 consecutive patients with primary APS, as well as tests for antiphospholipid antibodies in 26 patients who had TTP, severe ADAMTS-13 deficiency, and ADAMTS-13-inhibiting antibodies. In both of the patients with primary APS and TTP, ADAMTS-13 activity was undetectable, and ADAMTS-13-inhibiting antibodies were present. None of the 26 patients with TTP and severe ADAMTS-13 deficiency was positive for the lupus anticoagulant. One of these patients had a low level of anticardiolipin antibodies (22 IgG phospholipid units). In the 20 patients with primary APS, mean ADAMTS-13 activity was 116% (range 44-250%), and no severe deficiency (< 5%) was observed. Our findings suggest that primary APS must be added to the list of autoimmune disorders that can be complicated by TTP.
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PMID:Thrombotic thrombocytopenic purpura with severe ADAMTS-13 deficiency in two patients with primary antiphospholipid syndrome. 1547 41

A 41-year-old woman with known systemic lupus erythematosus (SLE) was diagnosed with thrombotic thrombocytopaenic purpura (TTP). At the time of admission she was suffering from petechia, purpura and had neurological symptoms. At first a relapse of the SLE was suspected. Additional laboratory findings demonstrated haemolytic anaemia, thrombocytopaenia and high levels of fragmentocytes. After multiple plasmapheresis treatments and immunosuppressive therapy she recovered. TTP can be differentiated from other thrombotic microangiopathic syndromes by its normal levels of prothrombin time, partially activated thromboplastin time (APTT), fibrinogen and direct Coombs-test. Further investigation is needed to confirm the diagnosis by determination of the activity of Von Willebrand factor cleaving protease ADAMTS-13. In this patient, the ADAMTS-13 activity returned after plasmapheresis. This case demonstrates the importance of fast and appropriate laboratory testing in order to diagnose TTP quickly.
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PMID:[A woman suffering from systemic lupus erythematosus and acquired thrombotic thrombocytopenic purpura]. 1552 34

Thrombotic microangiopathy (TMA) is a rare disorder characterized by microvascular thrombosis. TMA has been reported in patients with antiphospholipid antibodies and/or antiphospholipid syndrome but its pathogenesis is not clarified. We present two patients with TMA associated with IgG phosphatidylserine dependent antiprothrombin antibodies (aPS/PT). CASE 1: A 44-year-old Japanese female with systemic lupus erythematosus (SLE) and positive lupus anticoagulant (LA) was started on ticlopidine after having stroke. Four weeks later she developed TMA. IgG/M/A anticardiolipin antibodies (aCL) were negative, but strong positive IgG aPS/PT were detected. CASE 2: A 32-year-old Russian female with SLE was admitted because of hypertension, renal insufficiency and proteinuria at 14 weeks of pregnancy. She developed TMA after surgical abortion. IgG aPS/PT and LA were strongly positive but IgG/M/A aCL were negative. Neither case had von Willebrand factor cleaving protease (ADAMTS-13), suggesting that TMA in those patients was associated with thrombophilia rather than insufficient ADAMTS-13. Both patients were successfully treated with a series of plasma exchange.
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PMID:Thrombotic microangiopathy in patients with phosphatidylserine dependent antiprothrombin antibodies and antiphospholipid syndrome. 1832 60

A 4-year-old boy with an atypical course of haemolytic uremic syndrome (HUS), who developed microangiopathic antiphospholipid-associated syndrome (MAPS) with signs of multiple organ failure during the course of his disease, is reported. Early and aggressive treatment with intravenous gammaglobulin, pulse methylprednisolone and plasmapheresis resulted in an excellent clinical recovery. Our patient showed a concomitant presence of multiple factors that could precipitate atypical HUS, including positive antiphospholipid antibodies, decreased level of factor H and positive anti-ADAMTS-13 antibodies. We suggest that, along with infections, autoimmune conditions or defined genetic abnormalities of complement regulatory genes, MAPS should be considered among the pathogenic mechanisms in patients with atypical HUS.
Lupus 2008 Sep
PMID:Atypical haemolytic uremic syndrome complicated by microangiopathic antiphospholipid-associated syndrome. 1875 67

A 51-year-old woman was admitted to our hospital because of systemic jaundice, general fatigue in August 24, 2007. She was diagnosed with systemic lupus erythematosus (SLE) as a result of a discoid rash, photosensitivity, lymphocytopenia, elevated serum anti ds-DNA antibody and a positive test for antinuclear antibody. Her laboratory data revealed severe liver dysfunction, suggesting autoimmune hepatitis (AIH). She was also diagnosed with thrombotic thrombocytic purpura (TTP) because of thrombocytopenia, hemolytic anemia, renal dysfunction and decreased ADAM-TS13 activity. The patient was treated by methylprednisolone pulse therapy, fresh frozen plasma infusion and ursodeoxycholic acid. Her symptoms and laboratory data rapidly improved and a liver biopsy was carried out. Interface hepatitis and lymphocyte infiltration were observed in the specimen. A diagnosis of definite AIH was made from her International AIH group score of 20 points. AIH and TTP are rare complications of SLE. The prevalence of the complication of SLE and AIH has been reported as 1.7 approximately 2.7%, and that of SLE and TTP as 1 approximately 4%. We reported here a rare case of SLE complicated with AIH and TTP.
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PMID:[A case of systemic lupus erythematosus complicated with autoimmune hepatitis and thrombotic thrombocytic purpura]. 1940 9

We report the case of a patient with systemic lupus erythematosus (SLE) who first revealed hemophagocytic syndrome (HPS), which was treated successfully with glucocorticoid and intravenous cyclophosphamide. The patient then demonstrated refractory thrombotic thrombocytopenic purpura (TTP) with normal a disintegrin and metalloprotease with thrombospondin motifs (ADAMTS)-13 activity that responded well to rituximab. After rituximab treatment, the patient showed a flare of HPS that was controlled by additional intravenous cyclophosphamide treatment. This case showed that TTP with normal ADAMTS-13 activity is B-cell dependent and indicated that B-cell depletion might exacerbate some autoimmune conditions in SLE.
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PMID:Rituximab was effective on refractory thrombotic thrombocytopenic purpura but induced a flare of hemophagocytic syndrome in a patient with systemic lupus erythematosus. 1978 42

Hemolytic uremic syndrome (HUS) is related to a renal thrombotic microangiopathy, inducing hypertension and acute renal failure (ARF). Its pathogenesis involves an activation/lesion of microvascular endothelial cells, mainly in the renal vasculature, secondary to bacterial toxins, drugs, or autoantibodies. An overactivation of the complement alternate pathway secondary to a heterozygote deficiency of regulatory proteins (factor H, factor I or MCP) or to an activating mutation of factor B or C3 can also result in HUS. Less frequently, renal microthrombi are due to an acquired or a constitutional deficiency in ADAMTS-13, the protease cleaving von Wilebrand factor. Hemolytic anemia with schistocytes, thrombocytopenia without evidence of disseminated intravascular coagulation, and renal failure are consistently found. In typical HUS, a prodromal diarrhea, with blood in the stools, is observed, related to pathogenic enterobacteria, most frequently E. Coli O157:H7. HUS may also occur in the post partum period, and is then related to a factor H or factor I deficiency. HUS may also occur after various treatments such as mitomycin C, gemcitabine, ciclosporin A, or tacrolimus, and as reported more recently bevacizumab, an anti VEGF antibody. Atypical HUS are not associated with diarrhea, may be sporadic or familial, and can be related to an overactivation of the complement alternate pathway. More recently, some of them have been related to a mutation of thrombomodulin, which also regulates the alternate pathway of complement. In adults, several HUS are encountered in the course of chronic nephropathies: nephroangiosclerosis, chronic glomerulonephritis, post irradiation nephropathy, scleroderma, disseminated lupus erythematosus, antiphospholipid syndrome. Overall the prognosis of HUS has improved, with a patient survival greater than 85% at 1 year. Chronic renal failure is observed as a sequella in 20 to 65% of the cases. Plasma infusions and plasma exchanges are effective in most of the cases to treat hemolysis and thrombocytopenia. Steroid therapy is debated, as well as immunosuppressive drugs, including rituximab, in autoimmune forms. A new monoclonal anti-C5 antibody is tested, and seems to be effective in atypical HUS with abnormal complement alternate pathway activation. If terminal renal failure occurs, renal transplantation can be performed but the risk of recurrence, which very low in post infectious forms of HUS, is about 70 to 80% in genetic forms of complement regulatory protein deficiency.
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PMID:[Hemolytic uremic syndrome in adults]. 2039 68

We report a case of acquired thrombotic thrombocytopenic purpura (TTP) in a 34-year old patient with a prior diagnosis of systemic lupus erythematosis (SLE) who was recently started on hydroxychloroquine. Presenting symptoms included fevers, sore throat and productive cough with progressive weakness, dyspnea on exertion, hemoptysis and dark urine. Initial laboratory abnormalities were consistent with an acute microangiopathic hemolytic anemia and severe thrombocytopenia. At the time of admission, the patient's lupus was highly active as evident by his high SLE Disease Activity Index (SLEDAI) score. He was later also found to have severely reduced ADAMTS-13 levels and a positive antibody assay. This case highlights the occasional difficulty in pinpointing the exact etiology of TTP as well as establishes a possible novel drug association between hydroxychloroquine and TTP development.
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PMID:Acquired thrombotic thrombocytopenic purpura: puzzles, curiosities and conundrums. 2081 10

Thrombotic thrombocytopenia purpura (TTP) caused by a deficiency in ADAMTS-13 activity is considered to involve a subset of thrombotic microangiopathy (TMA). Although concept of TTP is included under the umbrella of TMA, discrimination of TTP from TMA is occasionally difficult in an autoimmune disorder. Herein, we report a case with TTP associated with systemic lupus erythematosus (SLE). In this case, it was difficult to discriminate TTP from TMA and the measurement of ADAMTS-13 activity was useful for obtaining an accurate diagnosis. SLE patients having thrombocytopenia in complication with anemia should be considered a monitoring of ADAMTS-13 activity even though the patients lacked symptoms of TTP related to the microvascular coagulation.
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PMID:A Case of Thrombotic Thrombocytopenia Purpura Associated with Systemic Lupus Erythematosus: Diagnostic Utility of ADAMTS-13 Activity. 2177 77

Thrombotic thrombocytopenic purpura (TTP) may be seen in association with autoimmune disorders such as immune hemolytic anemia and systemic lupus erythematosus, but rarely has it been associated with Graves' disease. We report one such case of a woman with new-onset thyrotoxicosis caused by Graves' disease, who abruptly developed TTP, confirmed by low serum ADAMTS-13 value. She had a dramatic response to plasma exchange, with remission of TTP. Definitive treatment with radioactive iodine resulted in euthyroidism and has prevented recurrence of TTP.
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PMID:Thrombotic thrombocytopenic purpura precipitated by thyrotoxicosis. 2264 92


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