Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A poor prognosis was observed in patients who had end-stage renal disease (ESRD) as a result of systemic lupus erythematosus (SLE). This was true even in patients in whom SLE disease activity was transiently quiescent during the period of hemodialysis. Six of 9 patients with ESRD and SLE died with active SLE and/or sepsis 1-28 months following the onset of dialysis. In 5 of the 6 patients, acute inflammatory activity of SLE flared within 1 month of the patient's death. Four patients died with superimposed sepsis, but only 2 of the 4 were receiving high-dose concomitant immunosuppressives for more than 1 week prior to death. Infected hemodialysis vascular access sites were implicated as the source of septicemia in 3 of 4 infectious deaths. The 3 surviving patients had minimal lupus activity prior to the development of ESRD, a possible marker for stability in SLE patients who require hemodialysis. Our results suggest that hemodialyzed lupus patients with nonautologous vascular access sites may be at continued increased risk for life-threatening inflammatory and septic complications.
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PMID:Poor prognosis in end-stage lupus nephritis due to nonautologous vascular access site associated septicemia and lupus flares. 281 17

A circulating lupus anticoagulant factor was detected in a 38-year-old man with end-stage renal disease and a 'lupus-like' syndrome with a diffuse proliferative glomerulonephritis. When treated with steroids, the 'lupus' complications were controlled and the anticoagulant factor disappeared; however, renal function did not recover and the patient commenced regular haemodialysis. Four months later the patient received a cadaver kidney transplant. At transplantation and during follow-up there was neither clinical nor laboratory evidence of lupus activity, but 19 months after transplantation, when steroids were tapered to a low dose, the lupus anticoagulant factor was detected, and renal-vein thrombosis complicated by sepsis led to the patient's death. A membranous glomerulonephritis was found on autopsy. This is the first time in which a (probably 'de novo') membranous glomerulonephritis has been detected in the allograft of a patient with circulating lupus anticoagulant factor.
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PMID:Allograft membranous glomerulonephritis and renal-vein thrombosis in a patient with a lupus anticoagulant factor. 314 30

A 56-year-old woman with a 12-year history of systemic lupus erythematosus presented with severe optic-disc swelling and blepharitis. At the same time, she developed acute pancreatitis and ultimately died of gram-negative sepsis. Although it appeared that the ocular and systemic disorders were manifestations of lupus, her serum antinuclear antibody and complement levels remained normal throughout her hospital course. Optic neuritis may be secondary to lupus, but the diagnosis is difficult to make when the serology is negative.
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PMID:Lupus optic neuritis with negative serology. 320 10

Critical conditions had been established in 21 (23.1%) of 91 patients with systemic connective tissue diseases for a 12 year period: renal failure (most often), sepsis, pericarditis with cardiac tamponade, hemorrhagic diathesis, terminal arteritis with gangrene, gastrointestinal perforations with peritonitis, etc. The corticosteroids applied in high doses and predominantly parenterally and the immunosuppressors are the main drugs used in the treatment of these conditions. Plasmapheresis when possible is a useful supplement. The prognosis of the acute critical conditions depends mainly on the affected organ (more favorable in pericarditis with tamponade and unfavorable in renal failure and gastrointestinal perforations with peritonitis (and on the basic disease) more optimistic in systemic lupus erythematodes and very pessimistic in nodal periarteritis and other allergic vasculitis).
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PMID:[The problems of treating acute critical states in diffuse connective tissue diseases]. 321 40

In 18 of 20 patients with psychosis secondary to systemic lupus erythematosus (SLE), autoantibodies to ribosomal P proteins were detected by immunoblotting and measured with a new radioimmunoassay using a synthetic peptide as antigen. The frequency of anti-P was not increased in patients with other central nervous system manifestations of SLE (3 of 20, by radioimmunoassay), in patients with transient behavioral abnormalities due to SLE (none of 8), in patients with psychosis who did not have SLE (none of 13), or in normal controls (none of 20). In four of five paired serum samples, anti-P-peptide antibody levels increased 5-fold to 30-fold during the active phase of lupus psychosis. Longitudinal studies of anti-P activity in two patients with psychosis revealed that anti-P levels increased before and during the active phases of psychosis but not during sepsis or other exacerbations of SLE, and that the elevations were selective for anti-P antibodies, as opposed to anti-DNA antibodies. Longitudinal studies of anti-P activity in two patients with anti-P but without psychosis showed less than threefold changes in anti-P levels despite exacerbations of disease. We conclude that anti-P is associated with lupus psychosis and that synthetic peptide antigens may be useful for the detection and measurement of autoantibodies to intracellular proteins.
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PMID:Association between lupus psychosis and anti-ribosomal P protein antibodies. 349 38

The causes of death were examined in patients with systemic lupus erythematosus (SLE) who were cared for at the University of California, San Francisco and who died after 1969. Of the 44 deaths analyzed, 33 patients had autopsies. Infections were common and often determined to be the cause of death. Overall, infections were present in 55 percent (22/44), and judged to be a cause of death in 30 percent (13/44) of all deaths. The infections could be divided into 2 groups: those due to common bacterial organisms and those due to opportunistic infections. These two types of infections occurred with similar frequency. When compared to common bacterial infections, however, the opportunistic infections were more likely to be first diagnosed at autopsy (p = .001). In only 3 of the 15 patients with an opportunistic infection was the diagnosis made antemortem. Failure to diagnose an opportunistic infection early occurred when the infection simulated active SLE, and when the possibility of an opportunistic infection was not aggressively investigated. The most common opportunistic infections were Candida albicans and Pneumocystis carinii. The most common site of opportunistic infection was the lung. Seventeen patients had 27 common bacterial infections, chiefly sepsis from Staphylococcus aureus and aerobic gram-negative organisms. Eight patients had both a common bacterial and an opportunistic infection. Stepwise linear regression analysis showed that death from infection correlated most strongly with prednisone and cytotoxic drug use in the 3 months before final admission. No measure of lupus activity was found to correlate with death from infection, except that hypocomplementemia correlated with death from bacterial infections.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Fatal infections in systemic lupus erythematosus: the role of opportunistic organisms. 362 46

Three patients from two families with complete hereditary deficiency of the fourth component of complement (C4) and systemic lupus erythematosus are described. The syndrome presented by these patients is characterized by early onset in life; exquisite sensitivity to sunlight and to cold exposure, the latter resulting Raynaud's phenomenon; and skin lesions involving not only exposed areas of the body but also palms and soles and presenting as butterfly rashes, maculopapular eruptions, and lesions similar to those of chronic discoid lupus erythematosus, with marked scaling, atrophy, and scarring. Lupus erythematosus (LE) cell tests were negative and antinuclear antibody (ANA) titers low or negative. The male patient of our series died at the age of 31/2 years from septicemia, whereas the two girls, aged 18 and 11 years, respectively, were alive at the time of writing. The C4-deficient gene is associated with HLA-Aw32, Bw38, and Bf S in one family and with HLA-A30, B18, DR7, and Bf S1 in the other family; the latter is the second family in which this HLA haplotype has been found to be associated with hereditary C4 deficiency.
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PMID:Systemic lupus erythematosus in hereditary deficiency of the fourth component of complement. 617 71

We have previously described (Medicine 56:493, 1977) 12 patients with diffuse lupus glomerulonephritis who had no clinical or laboratory evidence of renal involvement at the time of the initial biopsy. In this article we report the course of 10 of these patients followed for 5-11 yr (mean 83 mo). One patient died in renal failure and two others of related causes (septicemia and subarachnoid hemorrhage). Seven patients (Group I) had a benign course from a renal standpoint, with stable renal function and mild or no urinary abnormalities. Repeat biopsy in four patients in this group revealed near complete resolution of the original lesion in two and considerable improvement in two others, who now have primarily mesangial hypercellularity and a focal lesion, respectively. Renal function deteriorated in three patients (Group II), resulting in loss of congruent to 50% of GFR in two and renal death in the third. Repeat biopsy in one of these patients showed a more severe, albeit focal, glomerulonephritis. Prognosis for renal function appears better in patients with silent nephropathy, but larger numbers are required to substantiate this impression. Until definitive answers become available, we believe it prudent to biopsy SLE patients even in the absence of overt renal involvement and to treat those with diffuse proliferative glomerulonephritis.
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PMID:Silent diffuse lupus nephritis: long-term follow-up. 704 6

A case of excessive heparin requirement during cardiopulmonary bypass is reported. A patient with sepsis secondary to a myocardial abscess require 13.5 mg x kg-1 of heparin to increase his activated coagulation time to a therapeutic level. This phenomenon might be due to individual variability, lupus vasculitis, septicaemia, repeated thromboembolic phenomenon with hypercoagulable state, or chronic disseminated intravascular coagulation with partial antithrombin deficiency.
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PMID:Excessive requirement for heparin during cardiac surgery. 723 24

Non-resolved chronic pulmonary thromboembolism is a frequent cause of pulmonary hypertension. In long-standing disease hypertension is progressive due to intimal and medial changes in the perfused vessels. Non-resolution of thromboemboli is often associated with underlying coagulopathies; the presence of a lupus anticoagulant may pose a significant problem in the peri-operative management of these patients. Pulmonary thrombendarterectomy presents an efficient option of treatment which is feasible in the majority of patients. By means of pulmonary angiography and computed tomography operability is verified by the often difficult recognition of thromboembolic changes in the central pulmonary arteries. Patients with solely peripheral thromboembolic changes or primary pulmonary hypertension must be excluded. In presence of significant exertional dyspnea and/or pulmonary pressure elevation surgery is indicated. Mortality is high and mainly related to unrelieved pulmonary hypertension or pulmonary complications; pulmonary reperfusion edema, respiratory failure or pneumonia and sepsis. In all survivors the reduction of pulmonary hypertension is highly significant and persistent. Thromboembolic pulmonary hypertension may be treated curatively in most patients by thrombendarterectomy. Correct selection of surgical candidates is mandatory, and the patients should preferably be diagnosed and undergo surgery in an early stage of their disease.
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PMID:[Surgical treatment of thromboembolism-induced pulmonary hypertension]. 786 94


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