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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pulmonary hemorrhage is a rare, but serious manifestation of systemic lupus erythematosus (SLE). Herein, we report 13 cases of severe pulmonary hemorrhage in SLE. Hemoptysis was present in 11 patients. All thirteen patients had active nephritis and were in the stage of nephrotic syndrome. A majority of the patients had neuropsychiatric manifestations and coagulopathy including thrombocytopenia or lupus anticoagulant. All episodes of pulmonary hemorrhage occurred after large dose of corticosteroid had been administered in treating nephritis. Recurrent pulmonary hemorrhage was noted in four patients. Ten (77%) of the 13 patients finally died. Respiratory failure was the main cause of death. Our observation suggests that active nephritis with hypoalbuminemia is a major risk factor for severe pulmonary hemorrhage in SLE patients and that high dose corticosteroid use can not prevent the occurrence of severe pulmonary hemorrhage in SLE.
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PMID:Clinical experience of 13 cases with severe pulmonary hemorrhage in systemic lupus erythematosus with active nephritis. 975 70

With the use of low-dose heparin, fetal survival of aPL pregnancies is 70-80%, but prematurity and intrauterine growth restriction are common. It is likely, but not proven, that dysregulated placental coagulation and resultant vasculopathy are the cause of fetal loss. Details of dysregulated coagulation remain to be described. Opportunities remain to determine the role of coagulopathy in repeated pregnancy loss, identify a critical event or window to which intervention might be directed, identify maternal (and fetal) characteristics other than aPL that determine fetal loss, describe toxicity profiles of current treatments, develop more specific, less toxic therapies, and describe long-term fetal and maternal outcomes.
Lupus 1998
PMID:Pregnancy loss and antiphospholipid antibodies. 981 80

A potentially fatal hemophagocytic syndrome (HPS) has been noted in patients with reactive HPS. We describe 2 patients with reactive HPS treated with a regimen of therapeutic plasmapheresis and evaluate the efficacy of plasmapheresis for fatal HPS. Case 1 was a 31 year-old woman who had been treated for systemic lupus erythematosus (SLE) with corticosteroid hormones and immunosuppressants. She presented with persistent leukopenia and thrombocytopenia with spiking fever. She had an elevated level of serum ferritin, liver dysfunction, coagulopathy, and plasma inflammatory cytokines. Her bone marrow smear disclosed numerous hemophagocytosis of histiocytes. She was administered therapeutic plasmapheresis with total plasma exchange by fresh frozen plasma. There was an immediate and prominent decrease of cytokines, and she completely recovered. Case 2 was a 34 year-old woman who had been receiving high doses of corticosteroids and plasmapheresis for severe Stevens-Johnson's syndrome. After 18 months, she presented with physical and laboratory findings resembling lupus-like conditions and was administered high doses of corticosteroids and immunosuppressants. Human parvovirus B19 infection was detected by IgM and IgG antibodies and viral DNA from a bone marrow sample; moreover, a bone marrow smear disclosed findings of HPS. Repeated therapeutic plasmapheresis was effective for improving her symptoms and laboratory abnormalities; however, she suffered from septic methicilline resistant staphylococcus aureus infection and finally died of a brain hemorrhage resulting from disseminated intravascular coagulation (DIC).
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PMID:The efficacy of therapeutic plasmapheresis for the treatment of fatal hemophagocytic syndrome: two case reports. 1022 60

We describe two patients whose initial presentation of systemic lupus erythematosus (SLE) was accompanied by haemorrhagic episodes and significant coagulopathy. Further investigation demonstrated positive lupus anticoagulant and decreased Factor II (prothrombin) activity. Both patients were diagnosed with lupus anticoagulant-hypoprothrombinemia syndrome (LAC-HPS) as a result of non-neutralizing antibodies directed against Factor II. LAC-HPS is a rare clinical entity that can occur in association with SLE, transient viral infections, drug reactions or even in healthy individuals. Mixing studies, which can be affected by other coagulation factor inhibitors, play an important role in the diagnosis of LAC-HPS. Factor VII level was decreased in the second patient, a finding that has not previously been reported in association with SLE. In both patients, bleeding stopped promptly and coagulation studies improved significantly with high dose corticosteroids. We discuss the pathogenesis, diagnosis and management of LAC-HPS in patients with SLE.
Lupus 1999
PMID:Lupus anticoagulant-hypoprothrombinemia syndrome associated with systemic lupus erythematosus: report of 2 cases and review of literature. 1048 36

A 14-year-old man was admitted with sudden onset of paralysis in his lower extremities paralysis and of sensory loss below Th10 level. On admission, linear high intensity signals was seen in the spinal cord from Th 8 to Th 12 level on thoracic MRI T2 weighted image. Laboratory data on admission indicated existence of lupus anticoagrant in the patient's serum. Systemic lupus erythematodes was negative in his past history. The diagnosis of transverse myelitis caused by primary antiphospholipid syndrome was made accordingly. Plasmapheresis was performed to remove the lupus anticoagrant. After plasma pheresis coagulopathy was normalized with disappearance of the lupus anticoagrant. The lesion of the spinal cord is too extensive to be caused by single obstruction of any one blood vessel branch of the spinal cord. The transverse myelitis may be aggravated by the direct invasion of the lupus anticoagrant into nervous tissue from the vasculature whose blood brain barrier had been compromised by intravascular coagulation of this substance.
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PMID:[A case of transverse myelitis caused by primary antiphospholipid antibody syndrome]. 1061 66

We described an 11 year-old boy with systemic lupus erythematosus (SLE) and various coagulopathy. He had purpura on the legs, pancytopenia, positive anti-DNA antibodies and hypocomplementia. Hematological examination also showed that platelet counts were 80 x 10(3)/microliter, lupus anticoagulant and anticardiolipin antibodies were positive. The aPTT was remarkably prolonged. Those laboratory findings fulfilled the criteria of antiphospholipid syndrome. Following treatment with predonisolone and heparin, thrombocytopenia improved. When heparin discontinued and renal biopsy was performed, severe thrombocytopenia recureded. FDP and FDP-DD became high, but the aPTT was not prolonged. Thrombocytopenia didn't improved by the therapy with heparin, high dose of methylpredonisolone, FOY and gamma-globulin. However by the therapy with both warfarin and cyclophosphamide, remarkable improvement of coagulopathy was absorbed. Probably anticardiolipin antibodies and disseminated intravascular coagulation (DIC) participate in the various coagulopathy in this case.
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PMID:[A case of SLE with positive antiphospholipid antibody and various coagulopathy]. 1061 89

We reported an autopsy case of cerebral infarction with primary lung cancer. The patient was a 50-year-old man. Despite having been treated with warfarin potassium and ticlopidine hydrochloride, he relapsed cerebral infarction. His laboratory data on admission showed that lupus anticoagulant was positive, together with a high value of beta-thromboglobulin, thrombin-antithrombin III complex, markers of platelet and coagulation activation, CEA and CA 19-9. The autopsy finding revealed a primary papillary adenocarcinoma in the right lower lung, multiple cerebral infarction, renal infarction, pulmonary infarction and splenic infarction. The atherosclerotic changes were mild in the whole tissues and findings of vasculitis were not observed. Recurrence of cerebral infarction was effectively suppressed with the addition of steroid therapy to antithrombotic therapy. This case was considered as catastrophic antiphospholipid syndrome. It is necessary to differentiate antiphospholipid syndrome in case of the abnormal coagulation and fibrinolytic factors with recurrent cerebral infarction. Moreover, systemic examinations are important, because malignant tumor may exist on the background of the case.
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PMID:[An autopsy case of catastrophic antiphospholipid syndrome presenting with recurrent multiple cerebral infarction associated with lung cancer]. 1068 94

A sixty-five year old white male presented with an acquired Factor V inhibitor after an episode of cholecystitis and cefotaxime therapy. Plasma Factor V activity was less than 1%. He developed lower gastrointestinal bleeding a week after onset of coagulopathy, and was treated with plasmapheresis, fresh frozen plasma, oral cyclophosphamide, and prednisone. The coagulopathy resolved within four days of treatment, and within two weeks of presentation. Laboratory studies revealed an IgG inhibitor to Factor V that closely mimicked the more commonly encountered lupus anticoagulant. We would like to alert clinicians to this entity because, in contrast to a lupus anticoagulant, the acquired Factor V inhibitor can be associated with clinical bleeding as in our patient, and requires therapy prior to any surgical procedures.
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PMID:An acquired factor V inhibitor: clinical and laboratory features. 1085 68

Anemia, thrombocytopenia, and neutropenia are common manifestations in patients with human immunodeficiency virus infection that become more frequent and severe with progression from the asymptomatic state to acquired immunodeficiency syndrome (AIDS). Causes of anemia in AIDS include nutritional deficiencies, infection, and marrow suppression by antiretroviral drugs and by the disease itself. Autoimmune hemolysis and blood loss from gastrointestinal lymphoma or Kaposi sarcoma may also contribute. Granulocytopenia may be due to infection, autoimmunity, or bone marrow suppression by drugs or the immunodeficiency virus. Lymphopenia, the classic hallmark of the disease, typically affects T-helper cells first and worsens as the disease advances. Lymphopenia is a result of the direct cytopathic effects of the virus. Thrombocytopenia can occur from antibodies causing an idiopathic thrombocytopenic purpura-like state from bone marrow suppression or from thrombotic thrombocytopenic purpura. A prolonged partial thromboplastin time due to a coagulopathy caused by lupus anticoagulant causing has been described. A variety of malignancies occurs.
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PMID:Hematological Effects of Human Immunodeficiency Virus Infection. 1088 19

The term "antiphospholipids" (aPLs) refers to an heterogeneous family of antibodies diagnosed either by clotting tests: the lupus anticoagulants or by Elisa: anticardiolipin (aCL) and anti-beta2-glycoprotein I (anti-beta2GP1) especially. aPLS recognize phospholipids, alone or bound to plasma protein cofactor(s), or the cofactors themselves. aPLs have long been described in autoimmune diseases such as SLE, but may also be found in other clinical settings including infections, malignancies and drug administration. Their persistent presence can be associated with venous and/or arterial thrombotic complications and/or recurrent miscarriage, thus defining the "antiphospholipid syndrome" (APS). The heterogeneity of aPLs makes a comprehensive approach to laboratory investigation essential. Detection of lupus anticoagulants relies on increased clotting times in phospholipid-dependent tests. Their 4 step diagnosis includes: 1) screening (by at least two different tests); 2) demonstration of an inhibitory activity; 3) evidence of its phospholipid dependence; 4) exclusion of an associated coagulopathy. Among the aPLs detected by Elisa, IgG aCL are the most frequently investigated. However, other antibodies may represent useful biological tools. Among them, anti-beta2GP1 are thought to be more closely associated with a history of thrombosis than aCL and testing for anti-beta2 GP1 should now be systematically included in the biological diagnosis of APS. The Elisa used for aCL and anti-beta2GP1 are not fully standardized, and a number of methodological parameters may account for the interlaboratory discrepancies often observed. The clinical importance of other antibodies such as antiphosphatidylethanolamine, antiprothrombin or antiannexin V is being evaluated. An appropriate laboratory investigation of APS should, in all cases, combine the use of clotting and immunological assays, and assess the persistence of autoantibodies over time.
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PMID:[Antiphospholipid antibodies: clinical significance and biological diagnosis]. 1102 99


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