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)

A patient with clinical and serological manifestations of systemic lupus erythematosus (SLE) without renal or neurological manifestations was managed conservatively for 4 1/2 years. At this time she developed ascites and abnormal liver function tests, and was found to have severe chronic active hepatitis (CAH) with cirrhosis and portal hypertension. No clinical or biochemical evidence of liver disease was documented over the first 3 1/2 years of her illness, though no tests were performed in the 12 months before diagnosis. This case emphasizes the value of monitoring liver function tests over extended periods in such patients, since appropriate immunosuppressive therapy may benefit CAH, and as in this case, systemic manifestations of CAH may simulate SLE and precede clinical liver disease.
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PMID:Chronic active hepatitis in a patient presenting with clinical and serological evidence of SLE. 43 9

A middle-aged women with hypothyroidism, idiopathic portal hypertension and nephrotic syndrome is presented. This unusual clinical appearance could not be explained as SLE by serological examinations. Pathohistological examinations showed "Banti's liver", Hashimoto's thyroiditis and diffuse proliferative glomerulonephritis with severe tubulo-interstitial nephritis. Immunohistochemical studies revealed IgA deposits in glomeruli. Electron microscopic study disclosed peculiar lucent areas of rarefaction with osmiophilic particles in tubular basement membranes. This tubulointerstitial nephritis was considered to be related to the immunological mechanism involving thyroid gland, liver and kidney disorders. This case thus had a clinically rare combination of these three.
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PMID:Interstitial nephritis associated with glomerulonephritis in a patient with Hashimoto's disease and idiopathic portal hypertension. 150 28

We describe a 26-year-old patient with systemic lupus erythematosus and pulmonary and portal hypertension associated with the presence of anticardiolipin antibodies. Postmortem histological examination revealed the presence of recent thromboses of pulmonary and portal vessels. Our findings further support the concept of pulmonary hypertension being part of the antiphospholipid syndrome and suggest that portal hypertension due to intrahepatic portal vein thrombosis might be another manifestation of this syndrome.
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PMID:Portal and pulmonary vessel thrombosis associated with systemic lupus erythematosus and anticardiolipin antibodies. 179 32

Nodular regenerative hyperplasia (NRH) of the liver is an uncommon pathologic finding associated, in most cases, with rheumatic and hematologic diseases. Although its pathogenesis remains unclear, NRH probably results from liver regeneration to maintain its functional capacity after ischemia-induced injury. An intrahepatic microvascular occlusive mechanism has been considered most likely pathogenetically. NRH may lead to portal hypertension. Thus, the diagnosis of Felty's syndrome must be considered with caution in patients with rheumatoid arthritis (RA) and NRH of the liver. We report seven additional cases of NRH in patients with rheumatic disorders and review the literature to determine the patterns of clinical presentation and natural history of this condition. We also report four patients (three systemic lupus erythematosus [SLE] and one primary antiphospholipid syndrome [PAPS]) in whom antiphospholipid antibodies may have played a role in the genesis of NRH.
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PMID:Nodular regenerative hyperplasia of the liver in rheumatic diseases: report of seven cases and review of the literature. 194 1

We performed a prospective study including cytochemical, bacteriologic and pathologic observations in 25 patients with ascites of different causes. Activity of adenosine deaminase in ascitic fluid was higher in tuberculous (103 +/- 61 mu/l) than in neoplastic (16 +/- 8), inflammatory (16 +/- 13) and portal hypertension (15 +/- 6) etiologies (p less than 0.05). Inflammatory cases included patients with lupus and spontaneous peritonitis. Activity of adenosine deaminase was higher in every patient with tuberculosis than in any other patient. Thus, a high sensitivity and specificity of this test in the diagnosis of tuberculous peritonitis is confirmed.
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PMID:[Adenosine deaminase activity in peritoneal tuberculosis]. 251 74

A case of idiopathic portal hypertension associated with connective disease resembling systemic lupus erythematosus is described. The patient was a 50-year-old woman with splenomegaly, ascites, esophageal varices, and pancytopenia, but without extrahepatic portal obstruction or cirrhosis of the liver. Electron microscopy of the liver showed perisinusoidal fibrosis. High titers of autoantibodies against proliferating cell nuclear antigen (PCNA) were found in the sera as well as in ascites; anti-DNA antibodies appeared after anti-PCNA antibodies and remained thereafter at a moderate titer. The possibility of an immunological process in the pathogenesis of idiopathic portal hypertension is discussed.
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PMID:[Idiopathic portal hypertension associated with connective tissue disease similar to systemic lupus erythematosus]. 259 83

Systemic lupus erythematosus (SLE) is a well-known acute and/or chronic multisystem disease of complex autoimmune nature, having predilection for cardiovascular system. While its cardiac manifestations have been adequately studied, there is paucity of information on its vascular manifestations. Accordingly, we studied the incidence of vascular manifestations in 50 consecutive SLE patients seen at our institutions and in private practice during the past 12 years. Systemic hypertension (44%) was the most common vascular manifestation followed by vasculitis (30%), Raynaud's phenomenon (26%), telangiectasis (20%), premature coronary atherosclerosis (6%), digital ulceration (6%), thrombophlebitis (6%), pulmonary hypertension (4%) and portal hypertension (4%). Diffuse systemic vasculitis similar to polyarteritis nodosa was rare (2%). Often more than one lesion was found in the same patient. The clinical diagnosis of these vascular manifestations in the context of the primary disease (SLE) usually does not pose any difficulty except when they antedate it. We also studied the pathology and pathogenesis of some of these vascular lesions in both autopsy and biopsy specimens by both light microscopy and immunofluorescent techniques. Our results as well as those of others who also studied these lesions indicate that immune complex deposition and subsequent complement activation play an important role in the pathogenesis of vasculitis, coronary arteritis and premature coronary atherosclerosis. Corticosteroids and vasodilators remain the drugs of choice for the management of the majority of the symptoms arising from the vascular lesions of SLE.
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PMID:Vascular manifestations of systemic lupus erythematosus. 372 68

Of 860 patients with systemic lupus erythematosus (SLE) who were evaluated during a 25-year-period, 16 (1.9%) underwent splenectomy. Twelve of these patients had steroid resistant thrombocytopenia. An excellent long-term outcome occurred in eight (67%), significant improvement occurred in three (25%), and one patient died who also had chronic active hepatitis and portal hypertension. In two of three patients (67%) with autoimmune hemolytic anemia, the condition was corrected by splenectomy; in the third patient there was some improvement, but reduced doses of corticosteroids were required. One patient with severe neutropenia and recurrent bacterial infection obtained lasting benefit following splenectomy. Histologic examination of the removed spleen was not helpful in corroborating the diagnosis of SLE in these well established cases. Splenectomy had no adverse affect upon other aspects of SLE, in particular upon renal function. The authors conclude that the indications for splenectomy have proven to be of value in selected SLE patients with autoimmune or hypersplenic cytopenia.
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PMID:Splenectomy in systemic lupus erythematosis. 372 70

A young woman with systemic lupus erythematosus developed portal hypertension and pulmonary hypertension. This is the first report of such a case. We suggest that the presence of the lupus anticoagulant may have been related to the development of these two unusual features of her illness.
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PMID:Portal and pulmonary hypertension in a case of systematic lupus erythematosus: possible relationship with a clotting abnormality. 644 58

Three autopsy cases of systemic lupus erythematosus with unique association of multiple nodular hyperplasia (MNH) of the liver, portal hypertension, and hypertensive pulmonary vascular disease are reported. None of the patients had received oral contraceptive or androgenic steroid, but they were treated with glucocorticoids for 2 to 11 years. Raynaud's phenomenon, sclerodactyly, and mild impairment of the kidney were the common clinical features. Macroscopically, MNH is characterized by many nodules scattered throughout the non-cirrhotic liver, and histologically, each nodule is made up of normal-appearing hepatocytes and not encapsulated. Portal tracts are scanty in the nodules. MNH seems to be a regenerative-hyperplastic process, but its true nature still remains unclear. Relationships between MNH and portal hypertension, MNH and pulmonary hypertension, and collagen disease and pulmonary hypertension are discussed. A brief review of the literature concerning multiple benign hepatocellular tumors similar to MNH is also presented.
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PMID:Systemic lupus erythematosus associated with multiple nodular hyperplasia of the liver. 710 12


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