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)

Transformation of diffuse proliferative glomerulonephritis to membranous nephropathy 10 years later, in a patient with systemic lupus erythematosus, is reported. The original biopsy showed diffuse proliferative glomerulonephritis with epithelial crescent formation, 'wire loop' thickening of the capillary walls and moderately severe interstitial inflammation. Electron microscopy showed large subendothelial electron-dense deposits. Following treatment with a combination of prednisone and azathioprine for 2 years the 24-hour urine protein decreased from 8.8 g to 300 mg. In September, 1979, she again developed facial and pedal edema. With the exception of proteinuria of 6.0 g/24 h, the renal function remained normal. Repeat renal biopsy showed membranous glomerulopathy. On electron microscopy, electron-dense deposits were predominantly in a subepithelial position. The significance of the original biopsy as a predictor of eventual outcome and of sequential biopsies to the clinical management of patients with systemic lupus erythematosus is discussed.
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PMID:Transformation of diffuse proliferative glomerulonephritis to membranous nephritis in a patient with systemic lupus erythematosus. 726 28

We retrospectively evaluated the clinico-laboratory features of 11 children and adolescents with Systemic Lupus Erythematosus between the period of 2001 and 2006. All of them (100.0%) had renal involvement at the first visit in the hospital. Female to male ratio was 10:1. Skin and or mucosal involvement (90.9%), periorbital puffiness and or pedal edema (81.8%), fever (72.7%), hypertension (72.7%), and reticuloendothelial involvement (72.7%), were the commonest presentations. All patients had anemia (8.6 +/- 1.5 gm/dl), raised ESR (46.7 +/- 9.4 mm in first hour), proteinuria, and in disease activity as evident by raised ESR and positive anti-dsDNA antibody at the first visit. The mean duration of disease was 7.6 months and the average duration of disease activity was 63.18 days. Renal biopsy was performed in 8 patients: class IV lupus nephritis in 4 patients (50.0%), class II in 2 patients (25.0%), class III and V in patient (12.5%) each. Nephrotic range proteinuria and hypertension was observed in all patients of class IV and V of lupus nephritis. Class II and III lupus nephritis patients' were normotensive and had non-nephrotic range proteinuria. Three out of 11 patients (27.2%) expired. The commonest primary determinant of mortality was uncontrolled disease activity in 2 patients (66.6%). The third one had infection and developed disseminated intravascular coagulation. The mean duration of disease activity in patients who died (mean 30 days) was statistically lower than the survival group (75.6 days) (p < 0.01). Renal involvement during first visit and mortality could be attributed by late referrals and diagnosis at hospital.
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PMID:Systemic lupus erythematosus in childhood--a review of 11 patients at a single center in eastern Nepal. 1809 38

Antiphospholipid syndrome is an autoimmune disease characterized pathophysiologically by the presence of antiphospholipid antibodies and > or =1 clinical manifestation, the most common being venous or arterial thrombosis. We describe the case of a 40-year-old male with unexplained severe pulmonary arterial hypertension with a seven-day history of progressive shortness of breath, hemoptysis, chest discomfort and bilateral pedal edema. Electrocardiographic, echocardiographic and imaging studies showed changes consistent with chronic thromboembolic pulmonary hypertension. Further work-up showed positive anticardiolipin antibodies and lupus anticoagulant with negative features for lupus with negative primary thrombophilic studies as well. The patient was managed adequately with oral anticoagulation with improvement of his clinical status and referred to a tertiary care center to be screened for pulmonary thromboendarterectomy. For patients meeting surgical selection criteria, pulmonary thromboendarterectomy has demonstrated positive outcomes with respect to survival, functionality and quality of life. We discuss the pathophysiology and treatment as well as novel therapies in nonsurgical candidates with chronic thromboembolic pulmonary hypertension in the setting of primary antiphospholipid syndrome.
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PMID:Chronic thromboembolic pulmonary hypertension as an uncommon presentation of primary antiphospholipid syndrome. 1859 78

Membranous nephropathy (MN) associated with malignancies is a well-known entity. However, its association with benign neoplasm is not broadly recognized. A 69-year-old man with recurrent nephrotic syndrome presented with pedal edema and proteinuria of 5 months' duration. Laboratory results showed hypoalbuminemia and hyperlipidemia. Proteinuria was estimated to be protein excretion of 3.5g/d. Studies were negative for viral hepatitis, syphilis, human immunodeficiency virus, autoimmune diseases, and paraproteinemia. Kidney biopsy disclosed MN with negative phospholipase A2 receptor (PLA2R) staining, favoring a secondary form of MN. Computed tomography detected a 7.6-cm duodenal schwannoma. Elective surgical resection was performed. Pathologic study showed that THSD7A (thrombospondin type 1 domain-containing 7A) was positive in both glomeruli and schwannoma. Commonly, secondary MN is related to underlying conditions, including lupus, hepatitis, and neoplasm, and can be medication induced. The risk for developing a concomitant neoplasm among patients with PLA2R-negative MN is up to 12 times higher than in the general population. Most of these neoplasms are malignancies, and the presence of autoantibodies directed at similar tissue targets is hypothesized as the potential mechanism. In our case, THSD7A may be the autoantibody that has linked the schwannoma and the development of MN. Although benign tumors rarely produce renal manifestations, effective treatment may lead to resolution of nephrotic syndrome.
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PMID:Duodenal Schwannoma as a Rare Association With Membranous Nephropathy: A Case Report. 3045 84

Lupus nephritis (LN) is an immune-complex glomerulonephritis that is usually manifested by proteinuria, active urinary sediment, hypertension, and renal failure. The objective of this study is to study the clinical and histopathological profile of LN and the response to treatment with cyclophosphamide. This was a retrospective study conducted in a tertiary care center in Assam, India, where 176 LN patients who underwent renal biopsy were included. The presenting features, laboratory parameters such as proteinuria, hematuria, and the histopathological class of the patients were studied. Among the 176 patients, 89.8% were female and 10.2% were male and maximum patients (61.3%) were in the age group of 20-40 years. Pedal edema was present in 100% of the patients, decreased urine output in 43.7%, malar rash in 38%, joint pain in 42%, hair loss in 63%, hypertension in 41.4%, oral ulcers in 31.8%, seizures in 17%, psychosis in 13%, hematuriain 78.4%, anemia in 72.1%, thrombocytopenia in 51.1%, and leukopenia in 31.7% of patients. The anti-nuclear antibody was positive in all patients and anti-dsDNA was positive in 70.5% of the patients. The most common histopathological type was class IV (50%), followed by class III (17.6%). One hundred and two patients received intravenous cyclophosphamide as initial treatment of whom, 40 received the Eurolupus regimen and 62 received the NIH regimen. The number of patients who underwent remission in both the regimen was compared. The response rate of initial treatment with cyclophosphamide in the Eurolupus group was 62.5% and in the NIH group was 64.5% (P >0.05). Majority of the patients had proliferative LN in this study, of which class IV was the most common. Proliferative LN, if not detected and treated early, leads to poor outcome in terms of patient and renal survival. Hence, patients with systemic lupus erythematosus should be evaluated for kidney involvement and treated accordingly for better outcome.
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PMID:Clinical and histopathological profile of lupus nephritis and response to treatment with cyclophosphamide: A single center study. 3103 86

A 38-year-old woman, diagnosed as Person Living with Human Immunodeficiency Virus (HIV) on Highly Active Antiretroviral Therapy (HAART) for three years, presented with features of fever, rashes, joint pain, dyspnea and pedal edema. On evaluation, a diagnosis of Systemic Lupus Erythematosus with Lupus Nephritis (LN) triggered by HIV infection was made based on clinical and serological evidence. She was continued on HAART, and immuno-suppressive therapy was co-administered resulting in the resolution of her symptoms. Lupus-like histopathological findings have been reported in patients with HIV-related kidney diseases. This case report is to highlight the co-existence of LN in a patient with HIV infection.
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PMID:Lupus nephritis in a patient with retroviral infection. 3103 90