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)

150 cases of chronic inflammatory lung diseases of unknown aetiology and assumed hyperergic (immuno-reactive) pathogenesis were examined for hypertensive pulmonary arterial lesions and for chronic cor pulmonale. Hypertensive lesions of the small pulmonary arteries were found in more than half of the cases with chronic disorders of long duration, but were inconspicuous in diseases of acute progressive character. Hypertensive lesions were found regularly in chronic interstitial pneumonia, frequently in scleroderma and rheumatoid arthritis and occasionally in dermatomyositis and disseminated lupus erythematosus. Chronic Cor pulmonale occurred in 16% of the cases with hypertensive arterial lesions of grade I (hypertrophy of media) and in 50% of grade II/III (hypertrophy of media and intimal fibrosis). Interstitial lung fibrosis plays an important role in the pathogenesis of cor pulmonale: two thirds of the cases with interstitial lung fibrosis had developed cor pulmonale and all the cases with cor pulmonale also had interstitial lung fibrosis. Hypertensive arterial lesions of grade IV-VI according to Heath and Edwards (angiitis, plexogenic and angiomatoid lesions) have been described in severe cases of pulmonary hypertension (congenital cardiac shunts, primary pulmonary hypertension). In secondary forms of pulmonary hypertension, as represented by our material, these changes are of little importance.
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PMID:[Hypertensive lesions of pulmonary arteries in chronic inflammatory lung diseases (author's transl)]. 15 72

A 19 year old girl with Systemic Lupus Erythematosus (SLE), in chronic renal failure and on haemodialysis, underwent tricuspid valve replacement with a Carpentier-Edwards xenograft because of a large calcified mass invading the right side of the heart. The mass caused no symptoms and had been discovered accidentally on a routine chest X-ray. The bioprosthetic valve had subsequently to be removed, having developed functional stenosis, and was not replaced. Despite the absence of a tricuspid valve, the patient remains well five years after the operation.
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PMID:Bioprosthetic valve excision without replacement in the tricuspid position in a patient with Libman-Sacks endocarditis. 337 99

This paper describes a 47-years old patient with the history of lupus valvulitis for six and a half years, who had implantation of a Starr-Edwards prosthesis for a mitral insufficiency four years ago. This case represents the longest survival reported so far after mitral valve replacement for lupus valvulitis.
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PMID:Mitral valve replacement for lupus valvulitis. 382 24

A 39-year-old woman with the diagnosis of mitral regurgitation was admitted to our hospital for surgical treatment. Cerebral thromboembolism and spontaneous abortion had been repeated in her past history. She was suffered from chronic renal failure associated with systemic lupus erythematosus (SLE) and anti-phospholipid syndrome (APS). An operative procedure was recommended because of the progressive heart failure due to mitral regurgitation. For renal failure, continuous ambulatory peritoneal dialysis (CAPD) was introduced at one month before operation. As the operative procedure, valve replacement using Carpentier-Edwards bioprosthesis (27 mm) was done rather than valve reconstruction, because chordal rupture of posterior leaflet and severe hypertrophy of both leaflets were recognized. During operation, uncontrollable bleeding was not observed. However, platelet transfusion was needed. We use warfarin and antiplatelet agents jointly as postoperative anticoagulant therapy. Thromboembolic episodes have not been observed 4 years postoperatively. Relationship between SLE with APS and cardiac valvular lesions has been focused. The present case was considered to be the interesting case which various devices were necessary to operative management including chronic renal failure, cardiopulmonary bypass, selection of prosthetic heart valve, and postoperative anticoagulant therapy for preventing thrombus formation.
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PMID:[Successful surgical treatment of mitral regurgitation associated with anti-phospholipid syndrome and systemic lupus erythematosus]. 759 55

A 37-year-old man with systemic lupus erythematosus, who underwent an aortic valve replacement with a Carpentier-Edwards porcine valve for severe aortic insufficiency, was admitted to the hospital with pulmonary edema. Transesophageal echocardiography revealed severe aortic insufficiency arising from partial dehiscence of the valve sewing ring, as well as centrally from the valve cusp. In addition, marked thickening of the mitral valve was observed with severe eccentric regurgitation. At surgery, valvulitis of the native mitral and bioprosthetic aortic valves was demonstrated, with a perforation of the porcine valve cusp. After replacement of both valves, the patient had a stormy postoperative course with recurrent communications between the left ventricle and atrium requiring multiple surgeries and eventually died. This case illustrates the severity of valvulopathy and ensuing complications that can affect patients with systemic lupus erythematosus and demonstrates that the valvulopathy can affect bioprosthetic valves, a finding that has significant implications as to the type of valve replacement in these patients.
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PMID:Valvulitis involving a bioprosthetic valve in a patient with systemic lupus erythematosus. 867 31

A 40-year-old female had a history of fever, arthralgia, proteinuria, and dyspnea on effort twenty years ago, and was diagnosed as SLE, renal failure, and aortic regurgitation. She also suffered from pyelonephritis and sepsis due to the infection of E. coli. Preoperative examination revealed non-active phase of SLE. Echocardiography and aortography showed massive aortic regurgitation and operation was recommended. Operative findings showed fresh vegetation on the aortic leaflets, and aortic valve replacement (Tekna-Edwards 19 mm) was performed. Histological findings of the vegetation showed Libman-Sacks endocarditis and infectious endocarditis. Predonisolone was infused intravenously to prevent the acute deterioration of SLE after the operation. She was discharged from the hospital three weeks after the operation.
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PMID:[Aortic valve replacement due to Libman-Sacks endocarditis combined with infectious endocarditis]. 882 83

A 48-year-old woman admitted with progressive dyspnea had previously been diagnosed with systemic lupus erythematosus, antiphospholipid syndrome, and chronic renal failure, and had undergone mitral valve replacement with a Carpentier-Edwards pericardial bioprosthesis for mitral insufficiency 9 years before. She suffered a cerebral infarction 5 years earlier, despite appropriate anticoagulant therapy. On admission, echocardiography showed severe bioprosthetic stenosis. Repeat mitral valve replacement was conducted using a Mosaic bioprosthesis. On postoperative day 2, when heparinization was commenced, she suddenly had an epileptic fit. She also developed ischemic necrosis of the fingers and toes, considered secondary to microthrombosis. Aspirin was administered and heparin replaced by warfarin sodium. Necrosis gradually disappeared, and she was discharged 3 months after surgery. The original bioprosthesis showed degenerative changes with significant thrombus formation on cusps, thought to be mainly due to her hypercoagulable state. Considering the thrombophilic tendency in patients with antiphospholipid syndrome, strict management of anticoagulant therapy is required.
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PMID:Secondary mitral valve replacement in antiphospholipid syndrome and chronic renal failure. 1180 97

We investigated six new cases of primary thymic extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) to further characterize the clinicopathological and genetic features. The male to female ratio was 1:1. One female patient had suffered from systemic lupus erythematosus. Another female patient was diagnosed with rheumatoid arthritis after thymectomy. One patient had a concurrent tumor in the eyelid. Radiologically, all tumors were located in the prevascular space and presented as asymmetric heterogeneously enhanced cystic and solid masses. MALT1, BCL10 or IGH translocations and trisomy 18 were not observed in any cases by fluorescence in situ hybridization (FISH) analysis. Trisomy 3 was detected in one patient and another showed a TNFAIP3/A20 deletion. Meta-analysis (n = 51) including the present and previously reported cases revealed that the prevalence of autoimmunity was much lower in males with thymic MALT lymphoma compared to females (33% vs. 87%, p = 0.001). Additionally, the average age of females or patients with autoimmunity was about 10 years younger than that of males or patients without autoimmunity (p = 0.003 and p = 0.008, respectively). In summary, thymic MALT lymphoma arising without underlying autoimmunity frequently involves males or older patients. Trisomy 3 and an A20 deletion might play a role in the pathogenesis of thymic MALT lymphoma.
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PMID:Thymic extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue: a clinicopathological and genetic analysis of six cases. 2174 65

Chondrodysplasia punctate (CDP) is a rare group of disorders with both genetic and non-genetic underlying aetiologies. The genetic causes associated with CDP include peroxisomal disorders, type two mucolipidosis, type 3 mucopolysaccharidosis, GM1 gangliosidosis and chromosomal disorders. Peroxisomal disorders include deficiency of dihydroxyacetone phosphate acyltransferase, encoded by GNPAT, deficiency of the peroxisomal enzyme alkyl-dihydroxyacetone phosphate synthase, encoded by AGPS and Zellweger syndrome. The chromosomal disorders include Turner syndrome, trisomy 21 (Down syndrome), trisomy 18 (Edwards syndrome) and trisomy 9. Among non-genetic causes, teratogen exposure like warfarin and acenocoumarol is well known but for the past few years cases have been reported with maternal autoimmune disease mainly systemic lupus erythematosus and rarely with mixed connective tissue disorder (MCTD). However, the exact mechanism for the occurrence of CDP in MCTD is still unknown. We present here a 35-week appropriate for gestational age baby born to a second gravid mother, a known case of MCTD on treatment with hydroxychloroquine. The baby had mid-facial hypoplasia and bilateral talar region punctuate calcification suggestive of chondrodysplasia punctata. Global data on such cases are very scant. Further research work is needed to explore the association of specific antibody titre with the occurrence of such condition in maternal autoimmune disease.
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PMID:Neonatal punctate calcifications associated with maternal mixed connective tissue disorder (MCTD). 3031 86