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)

The incidence of hepatic involvement was followed up in a retrospective study carried out in a group of 177 patients with SLE. The liver damage was estimated by clinical examination, the titer of hepatocytic enzymes and, in some cases by hepatic biopsy. The proportion of liver involvement was found reduced but that did not exclude severe hepatic inflammation in certain cases. A high incidence of hepatomegaly was noted in spite of the fact that the histopathologic examination often showed normal values. The entity "lupoid hepatitis" is discussed as well as its real relationship with SLE.
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PMID:Liver disease in systemic lupus erythematosus. 147 94

A 40-year-old woman was admitted because of increasing exertional dyspnea. Right heart failure was suggested by the presence of hepatomegaly, pretibial edema and also echocardiographic findings. Physical examination and echocardiography showed no evidence of valvular disease or congenital heart disease except for right ventricular dilatation and tricuspid regurgitation. The ventricular septum deviated toward the left ventricle throughout the cardiac cycle, but left ventricular function was preserved. Severe pulmonary hypertension averaging 44 mmHg was revealed by cardiac catheterization. Digital subtraction angiography and pulmonary blood flow scintigraphy showed no evidence of pulmonary artery embolism, and no interstitial pulmonary lesions that might have caused pulmonary hypertension were recognized. Hypergammaglobulinemia suggested an autoimmune disorder, and signs of systemic lupus erythematosus (SLE), such as pleural effusion, proteinuria, lymphocytopenia, LE cell phenomenon and antinuclear antibodies were present. Several autoimmune diseases are known to be causative factors of pulmonary hypertension. However, only ten cases of SLE complicated by pulmonary hypertension have been reported the present one. These cases were characterized by a high incidence of Raynaud's phenomenon and positivity for anti-RNP antibody. In our present case, SLE activity was suppressed using prednisolone, but pulmonary hypertension persisted and the patient eventually died due to right cardiac failure. Judging from the clinical course of the ten reported cases of SLE-pulmonary hypertension, there seems to be no hope of improving the pulmonary hypertension once it has become established. Therefore it is important to detect and cure pulmonary hypertension as early as possible.
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PMID:[A case of lupus erythematosus preceded by right heart failure due to pulmonary hypertension]. 174 69

The Budd-Chiari syndrome (BCS) was diagnosed in a 30-year-old male hospitalized with hepatomegaly, abdominal collateral vessels and hepatic veins and inferior vena cava thrombosis (IVC) in 1988. The presence of circulating lupus anticoagulant (LAC) was suspected and demonstrated on this occasion in view of an earlier diagnosis of systemic lupus erythematosus (SLE) and recurrent vein thrombosis dating from 1981. There are sporadic reports of an association of BCS with SLE and other autoimmune diseases. The recent literature also describes associations with hypercoagulability due to LAC. These are reviewed together with the personal case to provide the rationale for correct diagnosis and therapy.
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PMID:Association of the Budd-Chiari syndrome with lupus anticoagulant. Case report and critical review. 174 29

Systemic lupus erythematosus (SLE) is poorly described among black children in Africa despite being more frequent among some black adult populations than their white counterparts. The first black South African child with SLE is documented. The patient was a 10-year-old girl who had fever, facial rash (with complement (C4) deposited at the dermo-epidermal junction of normal skin), weight loss, central nervous system involvement (depression, withdrawal, retinal exudates), renal involvement (glomerular filtration rate 54 ml/min/1.73 m2; membranous nephropathy with mild mesangial proliferation; World Health Organisation classification Vb), alopecia, lymphadenopathy, hepatomegaly, positive Coombs test, hypeocomplementaemia, anti-DNA antibodies and positive anti-nuclear factor.
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PMID:Systemic lupus erythematosus in a black South African child. First documented case report. 198 78

We report the case of a woman with lupus anticoagulant-positive systemic lupus erythematosus who developed small hepatic vein occlusion. Since the age of 34, she had been known to have hepatomegaly. A definitive diagnosis of systematic lupus erythematosus was made eight years later. Histological evaluation of the liver biopsy specimen was not fully diagnostic of prominent hepatomegaly during this period. Occlusion of the small hepatic veins was confirmed by hepatic venography, but the lumen of the large hepatic veins showed a smooth appearance. The lupus anticoagulant and anti-cardiolipin antibody were both positive. Since a high incidence of thromboembolic diseases in patients with the lupus anticoagulant or anti-cardiolipin antibody has been reported, the presence of this type of anticoagulant may provide an explanation for hypercoagulability and subsequent development of hepatic vein thrombosis in this patient. This is the first report of a patient with systemic lupus erythematosus who developed an occlusion of small hepatic veins attributable to the lupus anticoagulant and anticardiolipin antibody. This case suggested that a systematic search for hepatic vein occlusion should be made in patients with systemic lupus erythematosus who have developed inexplicable hepatomegaly, especially in those with positive tests for the lupus anticoagulant and/or anti-cardiolipin antibody.
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PMID:Occlusion of small hepatic veins associated with systemic lupus erythematosus with the lupus anticoagulant and anti-cardiolipin antibody. 251 8

A case of systemic lupus erythematosus (SLE) with mononucleosis-like hepatic injury was described. An emergent cesarean section was performed in a 25 yr-old house wife at 34 weeks gestation, followed by administration of several antibiotics. After the surgery she complained of high fever, hepatomegaly and dull right hypochondralgia, and mild liver dysfunction was also found. The liver biopsy showed prominent mononuclear cell infiltration in the sinusoids with minimum hepatocellular necrosis and mild triaditis, resembling hepatic lesion in infectious mononucleosis (mononucleosis-like injury). There were no clinical and serological features suggestive of infectious mononucleosis. This hepatic lesion was thought to be a manifestation of allergic reaction to drugs to which the lymphocyte stimulation test was found to be positive. Immunological abnormalities inherent in SLE might be related to occurrence of such allergic drug reaction.
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PMID:Drug-induced mononucleosis-like hepatic injury in a patient with systemic lupus erythematosus. 270 54

The clinical features of early cases of systemic lupus erythematosus (SLE) in 67 (55 female and 12 male) patients are reported in a prospective study and its prevalence rate is calculated. The disease accounted for 0.67% of all medical admissions and it was the third most frequent inflammatory rheumatic disease. By comparison with rheumatoid arthritis and extrapolation of the data, the prevalence of SLE was one case per 1867 of the population, one per 1127 of the total female population and for women aged between 10 and 49 years it was one per 616. Multisystem involvement was noted in all patients. Our results were presented and compared with other studies. Significant differences between our study and others were noted with respect to alopoecia, mouth ulcers, pericarditis and pleurisy. Subclinical involvement of the liver was noted in 26% and of the lungs in 19%. Hepatomegaly was noted twice as often in younger compared with older patients. SLE is a disease with an apparently increasing prevalence in Iraq. There is a need for a greater awareness on the part of practising physicians and more widespread availability of sensitive laboratory tests for diagnosis of the disease.
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PMID:Clinical features of early cases of systemic lupus erythematosus in Iraqui patients. 687 84

Systemic lupus erythematosus is an uncommon disease in men. The clinical and pathological features and prognosis in 50 male patients with lupus nephritis (LN) and 50 age-matched female patients with LN were analysed. The age at which the disease began and the diagnosis was made was generally older in the male. The incidence of LN was higher in the male than in female (P < 0.01). The incidence of types IV and V LN was more common in the male than in female. Type II LN was not found in male patients. Proteinuria over 3.5g/24h and renal failure were more common in the male than in female (P < 0.05). The three most common clinical manifestations in male patients were irregular fever, skin rashes and painful joints. Rashes, hepatomegaly and neuropsychiatric dysfunction were more common in the male than in female. The patients were followed up one to twelve years. The rate of recovery and improvement was lower, but that of relapse and mortality higher in the male than in female.
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PMID:[Lupus nephritis in male adults, an analysis of the clinical and pathological features]. 873 62

Lymphadenopathy (LAP) is a frequent sign in systemic lupus erythematosus (SLE). Yet, data concerning its relation to the various disease manifestations are scarce or absent. LAP was present in 23/90 (26%) SLE patients. Patients with LAP had significantly more constitutional symptoms of fatigue, fever and weight loss, more cutaneous symptoms and signs, a higher rate of hepatomegaly and splenomegaly, increased anti-dsDNA antibodies and decreased complement levels. Disease activity index was higher among patients with LAP, as was the intake of steroids and antimalaria medications. There was no difference in renal or central nervous system (CNS) involvement between patients with LAP and those without LAP.
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PMID:Lymphadenopathy in systemic lupus erythematosus. Prevalence and relation to disease manifestations. 885 65

Hepatic venous outflow obstruction also called the Budd-Chiari syndrome is increasingly being recognized as a cause of portal hypertension. In western countries the obstruction is usually in the hepatic veins while in reports from South Africa, Japan and India the predominant cause is a block in the IVC at the level of the diaphragm above the entry of the hepatic veins. A hypercoagulable state caused by myeloproliferative haematological disorders, clonal defects in haemopoietic stem cells, lupus anticoagulant, contraceptive pills and postpartum state are some of the aetiological conditions described. However in 25% to 75% cases no cause can be identified. The predominant presenting features in patients with hepatic vein obstruction are hepatomegaly and ascites while those with IVC obstruction show prominent veins on the trunk and back. Ultrasound examination should be the first investigative step. However a liver biopsy is the gold standard of diagnosis. To confirm the site of obstruction inferior vena cavography or functional hepatography may be required. In the acute phase thrombolytic therapy may be useful but for established cases either surgical intervention in the form of shunts or recently balloon angioplasty may be helpful. For patients with established cirrhosis and end-stage liver failure the only alternative is liver transplantation. All these patients however should be put on long term anticoagulants to prevent rethrombosis. Some series have reported that upto 45% of patients may develop hepatocellular carcinoma on long term followup. With proper management a larger proportion of patients can be returned to a useful productive life.
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PMID:Hepatic venous outflow obstruction. 982 3


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