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)

Thrombotic microangiopathy, which includes thrombotic thrombocytopenic purpura (TTP), shiga-toxin-associated hemolytic uremic syndrome (Stx-HUS) and atypical HUS, is characterized by the development of hyaline thrombi in the microvasculature resulting in thrombocytopenia, microangiopathic hemolysis, and organ dysfunction. Renal failure is a predominant complication of both Stx-HUS and atypical HUS, whereas neurological complications are more prominent in TTP. Other disorders such as lupus or bone marrow transplantations may occasionally present with features of thrombotic microangiopathy. Recent studies have found autoimmune inhibitors or genetic mutations of a von Willebrand factor (VWF) cleaving metalloprotease ADAMTS13 in patients with TTP. In approximately 30-50% of patients with atypical HUS, mutations have been detected in complement factor H, membrane cofactor protein (CD46), or factor I. All three proteins are involved in the regulation of complement activation. Additionally, autoantibodies of factor H have been described in patients without genetic mutations. These advances illustrate that dysregulation of VWF homeostasis or complement activation owing to genetic or autoimmune mechanisms may lead to the syndrome of thrombotic microangiopathy.
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PMID:The molecular biology of thrombotic microangiopathy. 1676 Sep 11

Thrombotic thrombocytopenic purpura (TTP) is a rare but potentially fatal disease in childhood. The association of microangiopathic hemolytic anemia, schistocytes, and thrombocytopenia without fever, neurologic, and renal involvement is sufficient to suspect TTP at an early stage for prompt plasma infusion or exchange therapy. TTP has been increasingly described especially in association with systemic lupus erythematosus (SLE). We report the youngest Chinese boy who presented his SLE with TTP and subsequently experienced 9 relapses of TTP in a 2-year period. SLE disease activity index was low during his TTP relapses and therefore alertness of TTP relapse is required even in a relatively inactive period of SLE. TTP should be recognized even without renal or neurologic features and can respond to plasma therapy.
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PMID:Recurrent thrombotic thrombocytopenic purpura in a young boy with systemic lupus erythematosus. 1776 61

Thrombotic thrombocytopenic purpura (TTP) is a rare dynamic life-threatening disease with systemic formation of platelet thrombi in the microvasculature within all the organs. Until recently the mortality in TTP has exceeded 90%. The progress of medical research in the last two decades has significantly improved our understanding of the pathogenesis of TTP and allowed a reduction in mortality. This paper describes a case of a 36-year-old female patient with systemic lupus erythematosus (SLE) who was admitted to our hospital for jaundice and speech disturbances. Laboratory tests revealed hemolytic anemia with a negative Coombs test. Abundant schistocytes were present in peripheral blood smear. During the first day of hospitalization progression of neurological signs was observed -the patient received high-dose corticosteroids (1000 mg of methylprednisolone daily for 5 consecutive days) and underwent plasma exchange therapy. We observed significant improvement of the patient's condition, as well as a complete resolution of clinical and laboratory abnormalities. In the following 24 months there have been no signs of the relapse of TTP. The article contains also a brief update of this unusual disease.
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PMID:[Thrombotic thrombocytopenic purpura in a patient with systemic lupus erythematosus successfully treated with plasma exchange and corticosteroids]. 1801 86

Small vessel occlusions may occur as part of the vascular manifestations of the Antiphospholipid Syndrome (APS) and may affect glomerular, skin, retinal, bowel, hepatic or pulmonary vessels. These thrombotic lesions are proven (usually by biopsy, surgical procedures, at autopsy or by specialized techniques e.g. in the case of retinal vascular occlusions). Another group of small vessel occlusions remain unproven and include osteonecrosis, hearing loss and a variety of brain syndromes. All these constitute the microvascular manifestations of the APS. Another separate group exists viz. thrombotic microangiopathic antiphospholipid-associated syndromes including Thrombotic Thrombocytopenic Purpura (TTP), HELLP syndrome and the thrombotic microangiopathies (primary or secondary e.g. to SLE itself or lupus-like disease). There is an accompanying haemolytic anaemia, often thrombocytopenia and presence of schistocytes. There are no large vessel occlusions and the antiphospholipid antibodies (aPL) may be generated by endothelial damage. It is possible that some of these "non-pathogenic" aPL may be rendered pathogenic by factor(s) unknown at this time causing a disturbance of the haemostatic equilibrium with resultant large vessel occlusions. This may be occurring in patients with the catastrophic antiphospholipid syndrome (CAPS/Asherson's syndrome). The term "MAPS" is suggested for these two groups of conditions.
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PMID:Microvascular and microangiopathic antiphospholipid-associated syndromes ("MAPS"): semantic or antisemantic? 1819 Aug 72

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

Thrombotic thrombocytopenic purpura (TTP) rarely occurs in the setting of systemic lupus erythematosus (SLE); when it does occur it has a high mortality rate and is refractory to conventional treatment. No clear treatment guidelines exist when refractory TTP occurs in the setting of SLE. A 24-year-old male patient presented with TTP in the setting of SLE that was refractory to conventional treatment but responded to rituximab.
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PMID:Rituximab therapy for refractory thrombotic thrombocytopenic purpura and autoimmune-mediated thrombocytopenia in systemic lupus erythematosus. 1870 78

To clarify the pathogenic processes of thrombotic microangiopathies (TMAs) in patients with connective tissue disease (CTD), we analysed clinical characteristics and plasma ADAMTS13 levels in 127 patients with CTD-TMAs, including patients with systemic lupus erythematosus (SLE), systemic sclerosis, polymyositis/dermatomyositis, and rheumatoid arthritis (RA), and 64 patients with acquired idiopathic thrombotic thrombocytopenic purpura (ai-TTP). Plasma levels of ADAMTS13 activity, antigen, and inhibitors were determined by enzyme immunoassays. IgG type anti-ADAMTS13 antibodies were also detected by immunoblots using purified ADAMTS13. ADAMTS13 activity was significantly decreased in CTD-TMAs, regardless of the underlying disease, but the frequency of severe deficiency (defined as <0.5% of normal) was lower in CTD-TMA patients than in ai-TTP patients (16.5% vs. 70.3%, p < 0.01). Severe deficiency of ADAMTS13 activity was predominantly detected in patients with RA- and SLE-TMAs, and was closely associated with the presence of anti-ADAMTS13 IgG antibodies. CTD-TMA patients with severe deficiency of ADAMTS13 activity appeared to have lower platelet counts and better therapeutic outcomes. At least two phenotypic TMAs occur in patients with CTDs: a minor population with deficient ADAMTS13 activity caused by neutralising autoantibodies, and a major population with normal or moderately reduced activity. Classifying CTD-TMAs by ADAMTS13 activity may be useful in predicting the clinical course and therapeutic outcomes, as patients with moderately reduced activity are likely to have more prominent renal impairment and poor prognoses.
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PMID:Heterogeneous pathogenic processes of thrombotic microangiopathies in patients with connective tissue diseases. 1965 89

Thrombotic thrombocytopenic purpura (TTP) and systemic lupus erythematosus (SLE) very rarely present simultaneously and pose a diagnostic and therapeutic dilemma to the critical care team. Prompt diagnosis and management with plasma exchange and immunosuppression is life-saving. A patient critically ill with TTP and SLE, successfully managed in the acute period of illness with plasma exchange, steroids and mycophenolate mofetil is described.
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PMID:Thrombotic thrombocytopenic purpura and systemic lupus erythematosus: successful management of a rare presentation. 1974 52

Thrombotic thrombocytopenic purpura (TTP) is a serious disorder with a significant morbidity and mortality. Majority of cases of TTP are idiopathic, but some cases may be secondary to connective tissue diseases. TTP has been rarely associated with systemic lupus erythematosus (SLE) and may be refractory to treatment with plasma exchange, requiring immunosuppressive therapy. We describe a patient with TTP and SLE who was refractory to plasma exchange and corticosteroids but responded to anti-CD20 antibody rituximab with continued remission after eight months of follow-up. Rituximab appears to be an effective treatment in refractory cases of TTP associated with SLE.
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PMID:Response to rituximab in a refractory case of thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus. 2006 3

The antiphospholipid syndrome (APS) comprises the association between vascular thrombosis, including microthrombosis, pregnancy morbidity and the coexistence of anti-phospholipid antibodies. Thrombotic microangiopathy (TMA) can be one of the manifestations of the APS and may involve any organ. This feature of the APS is probably less recognized by clinical doctors than venous thrombosis and recurrent abortions. This case report presents a patient who developed a widespread TMA with renal failure, gastric mucosa ulceration, urinary bladder ulcerations and a finger necrosis as part of the APS.
Lupus 2010 Nov
PMID:Thrombotic microangiopathy and the antiphospholipid syndrome. 2064 51


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