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)

Pancreatitis in SLE remains an enigma and probably results from multiple pathogenetic mechanisms. However, cases of pancreatic thrombus formation remain few and far between, suggesting that APS is not the major cause of pancreatitis. Whether aPL contribute to pancreatic thrombus formation in rare patients needs to be demonstrated by antemortum arteriographic studies showing thrombus or vasculopathy. The postmortem pancreatic examination of patients who have died of the disseminated vasculopathy-coagulopathy syndrome remains problematic, because if thrombi are found, they may simply represent the endstage of multiple mechanisms, including hypotension, hypoxia, DIC, or TTP, rather than an aPL specific mechanism.
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PMID:Pancreatitis in systemic lupus erythematosus: still in search of a mechanism. 151 54

The case of a patient admitted with thrombotic thrombocytopenic purpura nine years after developing systemic lupus erythematosus (SLE) is reported. Thrombotic thrombocytopenic purpura associated with SLE has been described on other occasions, but in most patients the diagnosis of SLE precedes that of thrombotic thrombocytopenic purpura. The unusual sequence and the chronological separation of the two diseases is emphasised.
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PMID:Thrombotic thrombocytopenic purpura preceding systemic lupus erythematosus. 157 91

The frequency of renal vascular lesions (RVL) and their relevance in the progression of renal damage were evaluated by the Pathology Group of the "Gruppo Italiano per lo Studio della Nefrite Lupica" (GISNEL). Of 285 patients with lupus nephritis collected from 20 nephrology centers in Italy and classified according to World Health Organization (WHO) criteria, 79 cases (27.7%) with RVL were identified and classified as follows: (1) lupus vasculopathy (n = 27); (2) hemolytic-uremic syndrome/thrombotic thrombocytopenic purpura (HUS/TTP) malignant hypertension-like lesions (n = 24); (3) vasculitis (n = 8); (4) arterio-arteriosclerosis (n = 20). At the time of renal biopsy, patients with RVL had mean serum creatinine levels significantly higher than patients without RVL (201.8 +/- 195.9 mumol/L [2.2 +/- 2.2 mg/dL] v 108.1 +/- 108.0 mumol/L [1.2 +/- 1.2 mg/dL]; P less than 0.01). Hypertension was more frequent in patients with RVL than in those without (68.4% v 30.5%; P less than 0.01). The probability of kidney survival assessed according to the Kaplan-Meier method at 5 and 10 years was, respectively, 74.3% +/- 5.9% and 58.0% +/- 8.9% in patients with RVL, compared with 89.6% +/- 2.7% and 85.9% +/- 3.7% in patients without RVL. However, the two groups did not differ significantly as regards overall survival, the probability of survival at 5 and 10 years being 86.5% +/- 4.5% and 78.8% +/- 6.6% in patients with RVL and 92.2% +/- 2.2% and 83.3% +/- 4.4% in patients without RVL.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Renal vascular lesions as a marker of poor prognosis in patients with lupus nephritis. Gruppo Italiano per lo Studio della Nefrite Lupica (GISNEL). 186 81

Thrombotic thrombocytopenic purpura (TTP) is a clinical syndrome of unknown etiology and has a high mortality rate due to disseminated platelet thrombi. However, the mechanism of platelet agglutination is not understood. Although an immunological mechanism has been suggested as the basis for the pathogenesis of TTP, any possible immune-mediated etiology remains unclear. The association of TTP with systemic lupus erythematosus (SLE) affords a unique opportunity to study such possibilities, because SLE is a prototype of autoimmune disease. This report describes two patients with SLE who developed TTP. The development of anti-platelet antibodies is one possible immunological mechanism for platelet agglutination in patients with SLE complicated by TTP. More importantly, patient J.Y., who had anti-platelet antibodies, responded dramatically to high doses of prednisolone.
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PMID:Thrombotic thrombocytopenic purpura in two patients with systemic lupus erythematosus: clinical significance of anti-platelet antibodies. 239 32

Human immunodeficiency virus (HIV) infection is associated to different clinical and laboratory hemostatic alterations. The most important is thrombocytopenia, which very often leads to a picture indistinguishable from idiopathic thrombocytopenic purpura with poor clinical relevance but with possible important problems of differential diagnosis. Thrombotic thrombocytopenic purpura is sometimes associated to HIV infections, although only few reports there exist. Finally, the high incidence of lupus anticoagulant in some phases of HIV infection has not a clinical relevance, but is essentially a laboratory finding which is to be known because it can cause a prolonged partial thromboplastin time.
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PMID:Hemostatic alterations during human immunodeficiency virus infection: a review. 267 75

Three groups of kidney specimens from patients with systemic lupus erythematosus (SLE) were examined for histologic evidence of vascular lesions in small arteries and arterioles. Group 1 consisted of 24 autopsy kidneys from patients who died before the advent of steroid therapy, and Group 2, of 26 more recent autopsy specimens from patients treated with steroids and/or immunosuppressive drugs. Group 3 comprised 276 renal biopsies. Group 1 showed characteristic subendothelial eosinophilic deposits in small arteries and arterioles of 8 cases; Group 2 showed similar lesions in 5 specimens, while 3 others revealed evidence of resorption of deposits. Deposits were characterized by clumping and were delimited toward the media by a thick basement membrane. Only one case showed necrotizing arteritis resembling polyarteritis nodosa. Group 3 presented vascular deposits in 19 cases and thrombotic microangiopathy in 2. Electron microscopic appearance of some of the deposits is described. Immunofluorescence microscopy showed a mixture of IgG, IgA, and IgM in 7 cases, a finding that was not seen in a group of non-lupus patients with various vascular lesions. Vascular deposits are generally rare in systemic lupus erythematosus, although in autopsies widely scattered involvement of arteries and arterioles was seen in nearly 1/3 of the cases. The deposits were more common in male patients. The evolution of the lesions could be followed through various stages to eventual sclerosis, particularly in patients treated with steroids or immunosuppressants. Some deposits appeared to resolve after treatment. Patients with vascular deposits had more severe glomerular disease and a more serious clinical course. Thrombotic microangiopathy appears to be a secondary phenomenon whose pathogenesis is unknown.
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PMID:Vascular lesions in lupus nephritis. 323 4

Possible relationships between Moschcowitz's disease and acute systemic lupus erythematosus are analyzed based on findings in one case. Initial exclusively buccal symptoms suggested a diagnosis of lupus, and then of Moschcowitz's disease leading to a fatal outcome. The main points discussed relate to diagnostic difficulties to enable distinction between the two diseases. A thrombotic thrombopenic purpura during the second phase of the disease showed a characteristic microangiopathy associated with a non-autoimmune hemolytic anemia and a schizocytosis: two forms of the affection are recognized, one with a chronic and one with an acute course. The latter, which has a fulminating progression leading to death, was that described in the case reported.
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PMID:[Thrombopenia as the presenting symptom of acute disseminated lupus erythematosus: Moschcowitz's disease]. 657 93

Thrombotic thrombocytopenic purpura (TTP) and systemic lupus erythematosus (SLE) are both multisystem diseases and the latter can manifest hemopoietic abnormalities that may mimic TTP. This has led to diagnostic confusion and reports of the 2 diseases occurring in a single patient. We describe a 15-yr-old girl who presented at age 12 with purpura, fever, headaches, changes in conscious state, thrombocytopenia and microangiopathic hemolytic anemia and who was diagnosed despite the absence of renal disease, as having TTP. ANA and LE cells were negative then, and again 1 yr later. Three yr later she presented with the nephrotic syndrome with a positive ANA (1:100), elevated DNA antibody (79 U/ml, normal less than 25) and a circulating anticoagulant. Renal biopsy confirmed the presence of lupus nephritis. The association is discussed.
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PMID:Systemic lupus erythematosus and thrombotic thrombocytopenic purpura. A case report and review of relationship. 702 83

Thrombotic thrombocytopenic purpura (TTP) is a rare syndrome of unknown etiology and has a high mortality rate. TTP is characterized by a pentad of clinical findings, including microangiopathic hemolytic anemia, thrombocytopenia, renal abnormalities, neurologic signs and fever. The pathological feature of TTP consists of disseminated microvascular platelet thrombi. We describe a case of TTP with primary anti-phospholipid syndrome. A 27-year-old woman developed TTP in her second trimester of pregnancy. She presented with classical symptoms of TTP with compatible renal biopsy findings. Although four articles of SLE criteria (1982 ARA) were fulfilled, three of them were considered to be derived from multiple thrombosis except for a positive antinuclear antibody. Positive antiphospholipid antibodies (lupus anticoagulant and anticardiolipin antibody) with SLE-like findings suggested the diagnosis of antiphospholipid antibody syndrome rather than SLE. Although TTP has been described in patients with SLE and they can share common clinical and pathological features, the relationship between these two diseases is controversial. Many theories have been proposed to explain the nature and cause of intravascular platelet aggregation in TTP. But the pathogenesis of TTP is still unclear. This case suggests an important causal relationship between antiphospholipid antibodies and TTP during pregnancy. In TTP patients who also have SLE or SLE-like features, the antiphospholipid antibodies may have a role in the development of multiple microthrombosis.
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PMID:[Thrombotic thrombocytopenic purpura (TTP) observed in a patient with primary antiphospholipid antibody syndrome]. 786 89

A review of 50 patients who manifest features of the catastrophic antiphospholipid syndrome (CAPS) is presented. The clinical features comprise mainly organ involvement as opposed to large-vessel venous or arterial occlusions as is seen in patients with 'simple' antiphospholipid syndrome (APS), which makes the pathogenesis of this unusually rare complication perhaps somewhat different from that of patients with the APS. The mortality of the condition is 50%, most patients dying as a result of a combination of cardiac and respiratory failure. Fifteen patients (28%) suffered from disseminated intravascular coagulation (DIC) as well, which may have contributed to the multiorgan thrombotic microangiopathy characteristic of the CAPS. Although most patients were treated with high-dose i.v. steroids, heparin, cyclophosphamide and other modalities of therapy (such as i.v. globulin), plasmapheresis (advocated for TTP, a similar microangiopathic condition) seemed to offer some benefit (68% recovery). The systemic inflammatory response syndrome (SIRS) was responsible for some of the clinical manifestations such as adult respiratory distress syndrome (ARDS) seen in 15 patients. Pathogenesis of the CAPS seems dependent on a 'two-hit' or even 'three-hit' hypothesis in patients already suffering from a hypercoagulable state. Precipitating factors include infections, trauma (surgical), drug administration and warfarin withdrawal. A recent view that the multiple thrombotic lesions themselves may contribute to further thrombosis ('thrombotic storm') is also discussed.
Lupus 1998
PMID:The catastrophic antiphospholipid syndrome, 1998. A review of the clinical features, possible pathogenesis and treatment. 981 75


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