Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Reported is the case of a 24-year-old Finnish woman who developed malignant hypertension while taking an oral contraceptive (OC) that contained 30 mcg of ethinyl estradiol. She presented with blurred vision, but reported no other remarkable signs or symptoms during the 5 months in which she had been using OCs. Laboratory tests at admission revealed incomplete systemic lupus erythematosus (SLE) with DNA antibodies and high levels of antiphospholipid antibodies. Her blood pressure was 220-140 mmHg. OC use was discontinued and antihypertensive treatment initiated, with good results. 2 years later, however, the patient developed epileptic seizures and an area of local atrophy in the cerebellum was identified through computerized tomography. In the 4-6th years after initial presentation, the patient experienced 3 miscarriages, all at 7-8 weeks of gestation. 1 year after presentation, the patient satisfied 4 of the criteria for SLE (positive DNA and antiphospholipid antibodies, thrombocytopenia, leukopenia, and proteinuria). At present, the patient's symptoms are being controlled with carbamazepine and metroprolol. The patient's older sister, who had never used OCs, had SLE. It appears that high levels of antiphospholipid antibodies are an additional risk factor for the development of vascular complications in OC users but are not induced by OCs. Similarly, while OCs are not believed to cause SLE, they can exacerbate the disease or unmask a lupus diathesis.
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PMID:Malignant hypertension and antiphospholipid antibodies as presenting features of SLE in a young woman using oral contraceptives. 174 6

The frequency of renal vascular lesions (RVL) and their relevance in the progression of renal damage were evaluated by the Pathology Group of the "Gruppo Italiano per lo Studio della Nefrite Lupica" (GISNEL). Of 285 patients with lupus nephritis collected from 20 nephrology centers in Italy and classified according to World Health Organization (WHO) criteria, 79 cases (27.7%) with RVL were identified and classified as follows: (1) lupus vasculopathy (n = 27); (2) hemolytic-uremic syndrome/thrombotic thrombocytopenic purpura (HUS/TTP) malignant hypertension-like lesions (n = 24); (3) vasculitis (n = 8); (4) arterio-arteriosclerosis (n = 20). At the time of renal biopsy, patients with RVL had mean serum creatinine levels significantly higher than patients without RVL (201.8 +/- 195.9 mumol/L [2.2 +/- 2.2 mg/dL] v 108.1 +/- 108.0 mumol/L [1.2 +/- 1.2 mg/dL]; P less than 0.01). Hypertension was more frequent in patients with RVL than in those without (68.4% v 30.5%; P less than 0.01). The probability of kidney survival assessed according to the Kaplan-Meier method at 5 and 10 years was, respectively, 74.3% +/- 5.9% and 58.0% +/- 8.9% in patients with RVL, compared with 89.6% +/- 2.7% and 85.9% +/- 3.7% in patients without RVL. However, the two groups did not differ significantly as regards overall survival, the probability of survival at 5 and 10 years being 86.5% +/- 4.5% and 78.8% +/- 6.6% in patients with RVL and 92.2% +/- 2.2% and 83.3% +/- 4.4% in patients without RVL.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Renal vascular lesions as a marker of poor prognosis in patients with lupus nephritis. Gruppo Italiano per lo Studio della Nefrite Lupica (GISNEL). 186 81

The patient, a Caucasian woman of 22 years, developed malignant hypertension at the age of 16 years. An abdominal bruit was found on routine examination. She had two spontaneous abortions and systemic lupus erythematosus was diagnosed at age 21 years. She was found to have a persistently low positive VDRL and antibodies to mitochondria were also present on repeated examinations, to a titre of 1/160. Because of angiographic findings, demonstrating an occlusion of the aorta, an endarterectomy of the descending thoracic and upper abdominal aorta was performed. This showed mainly intimal thickening and the presence of thrombus. She then had four further spontaneous abortions (with good blood pressure control). The lupus anticoagulant and antibodies to cardiolipin were found to be positive at this time.
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PMID:Aortic occlusion in systemic lupus erythematosus associated with antiphospholipid antibodies. 311 55

The authors report the association of a clinically florid form of lupus with malignant hypertension and renal failure. The renal histology revealed lesions of nephroangiosclerosis, with virtually no signs of lupus proliferative glomerulonephritis. With a follow-up period of 42 months, the renal function has returned to normal, although steroid treatment has been interrupted for 33 months. However, intensive antihypertensive treatment is still required. This unusual case is discussed in the light of previous reports in the literature of the association of disseminated lupus erythematosus and malignant hypertension, which is nevertheless a rare entity.
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PMID:[Pure hypertensive nephropathy associated with malignant arterial hypertension in disseminated lupus erythematosus. Case with a favorable outcome]. 398 Oct 75

The antiphospholipid syndrome is usually defined by the association of a clinical manifestation (recurrent venous and/or arterial thrombosis, recurrent spontaneous miscarriages) and a biological abnormality (anticardiolipin antibody, lupus anticoagulant). We retrospectively analyzed the records of 5 patients (4 females, 1 male, aged 30 +/- 12 years) with antiphospholipid syndrome, primary (n = 1) or secondary to systemic lupus erythematosus (n = 4), who developed malignant systemic hypertension with renal insufficiency, in the absence of lupus nephritis. Before the episode of malignant hypertension, all patients had normal systemic blood pressure and renal function. During malignant hypertension the systolic pressure was 206 +/- 39 mmHg and the diastolic pressure 130 +/- 25 mmHg, peak serum creatinine was 204 +/- 95 mumol/l, daily proteinuria was 1.1 +/- 0.8 gr, and complement serum levels were normal in all patients. Renal angiography found normal proximal renal arteries. Renal biopsy showed ischaemic glomeruli without proliferative lesions (n = 5), focal intimal fibrosis either isolated (n = 3) or associated with thrombosis (n = 2) of the intrarenal vessels, and the absence of vasculitis. Immunofluorescence study did not reveal typical lupus deposits. Patients were treated with antihypertensive agents, increasing doses of prednisone (n = 3), and anticoagulant (n = 2) or anti-aggregant therapy (n = 1). After a mean follow-up of 6.8 +/- 5.2 years, 4 patients were still alive with normal blood pressure and renal function, whereas 1 patient died of a probable catastrophic antiphospholipid syndrome. Patients with antiphospholipid syndrome, primary or secondary to systemic lupus erythematosus, may develop malignant hypertension with renal insufficiency and intrarenal vascular lesions, in the absence of lupus nephritis.
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PMID:Malignant hypertension in antiphospholipid syndrome without overt lupus nephritis. 827 82

Acute renal insufficiency in the setting of hemolysis and thrombocytopenia, a triad that constitutes adult or pediatric hemolytic uremic syndrome, can be associated with or triggered by diverse conditions such as verocytotoxin-producing Escherichia coli, viral infections, pregnancy, malignant hypertension, scleroderma, renal radiation, allograft rejection, lupus erythematosus, and assorted medications such as mitomycin C, cyclosporine, and oral contraceptives. Recurrent and de novo hemolytic uremic syndrome occur after renal transplantation. Relapses are also common and probably reflect incomplete resolution of the initial episode. The major differential diagnoses of hemolytic uremic syndrome in the renal allograft include acute vascular rejection, cyclosporine, FK506 or antilymphocyte antibody nephrotoxicity, and malignant hypertension, all of which may display overlapping clinical and histologic features with primary hemolytic uremic syndrome; in such instances, the exact diagnosis may be quite difficult. It is possible that the risk of recurrence may be reduced by proper timing of transplantation and suitable choice of immunosuppressive agents. Intensive plasmapheresis in conjunction with fresh frozen plasma and supportive management of renal failure may lessen mortality and morbidity even in recurrent hemolytic uremic syndrome after transplantation.
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PMID:Recurrent hemolytic uremic syndrome in an adult renal allograft recipient: current concepts and management. 858 82

Myelopathy is a rare central nervous system manifestation in systemic lupus erythematosus (SLE). We present a case of SLE, who developed motor paralytic bladder and various other neurological abnormalities. A 29-year-old female with SLE was admitted to our hospital because of complete dysuria without any troubles on defecation. Accelerated hypertension had been noticed 2 weeks before the admission. Physical examinations revealed that she had muscle weakness in right brachial biceps, bilateral carpal extensor and flexor, and flexor muscles of bilateral lower extremities. Slight sensory disturbance was present on her soles. Bilateral Chaddok and Babinski's signs were positive. Electromyographic studies including nerve conduction velocities of her limbs were normal, however, neurogenic discharges were observed in anal sphincter muscles. Cystometry demonstrated atonic bladder, but any pathological findings such as lupus cystitis and interstitial cystitis were not observed in the biopsied specimens from her bladder. Antibodies to single-stranded DNA, U1 RNP, Sm and SS-A/Ro were positive in her serum, and lupus anticoagulant and anticardiolipin antibodies (IgG) were also detected. In her cerebral spinal fluid (CSF), elevated protein level and albuminocytologic dissociation were recognized, while glucose level was low. Magnetic resonance imaging (MRI) study detected high signal intensities in the inner part of medulla oblongata and in the spinal cord at second lumbar spine level. After two courses of methyl-prednisolone pulse therapy, the patient's neurological symptoms including dysuria had completely recovered and abnormal findings previously observed on MRI had also disappeared. After 7 months of the episode, she became normotensive. The proteins and glucose levels in her CSF had gradually returned to normal. Among patients with SLE, correlations of antiphospholipid antibodies with myelitis/myelopathy or accelerated hypertension have been reported. Therefore, possible roles of antiphospholipid antibodies were considered in the pathogenesis of neurologic abnormalities observed in our patient. In addition, low glucose level in CSF might be a good indicator for the diagnosis of lupus-associated myelopathy.
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PMID:[A case of systemic lupus erythematosus with various central and peripheral neurological disorders presenting with motor paralytic bladder as a major manifestation]. 859 61

Malignant hypertension and cardiac tamponade are uncommon but potentially life-threatening medical emergencies. Both conditions may be associated with collagen vascular diseases, such as systemic lupus erythematosus. We report a case of acute cardiac tamponade associated with malignant hypertension secondary to lupus nephritis. Immediately after pericardiocentesis, blood pressure declined substantially. Although malignant hypertension is seen with modest frequency in patients with systemic lupus erythematosus, true cardiac tamponade is a less common complication of lupus serositis. Acute, simultaneous presentation of both life-threatening entities and the hemodynamic course have not been described.
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PMID:Simultaneous malignant hypertension and cardiac tamponade. 1187 97

The antiphosphospholipid antibody syndrome (APS) describes a clinical entity with recurrent thrombosis, fetal loss, thrombocytopenia in the presence of lupus anticoagulant and/or antibodies to cardiolipin. These antibodies may be associated with connective tissue diseases such as systemic lupus erythematosus (secondary APS) or be found in isolation (primary APS). Renal syndromes increasingly being reported in association with these antibodies include thrombotic microangiopathy, renal vein thrombosis, renal infarction, renal artery stenosis and/or malignant hypertension, increased allograft vascular thrombosis, and reduced survival of renal allografts. Although much has been understood concerning the biology of these antibodies and the pathogenesis of thrombosis, the optimal therapy remains to be elucidated. This article presents a historical review of the renal involvement in the antiphospholipid syndrome and discusses therapeutic options. Further research is needed.
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PMID:"Black swan in the kidney": renal involvement in the antiphospholipid antibody syndrome. 1216 54

Thrombotic microangiopathy is a rare but important finding in the context of organ transplantation. Acute renal insufficiency in the setting of hemolysis and thrombocytopenia, a triad that constitutes 'hemolytic uremic syndrome', can be associated with, or triggered by, conditions such as verocytotoxin-producing Escherichia coli, viral infections, malignant hypertension, scleroderma, allograft rejection, lupus erythematosus, pregnancy, and medications including mitomycin C, calcineurin inhibitors, and oral contraceptives. After renal transplantation, it can occur, as either a de novo episode, or recurrent disease. Calcineurin inhibitors have long been associated with post-transplantation thrombotic microangiopathy. Sirolimus has been used as a primary immunosuppressant in patients transplanted with a history of earlier hemolytic-uremic syndrome, and also as rescue therapy in patients with calcineurin-inhibitor-associated thrombotic microangiopathy. We describe four cases where there was significant thrombotic microangiopathy in the context of contemporaneous or contiguous calcineurin inhibitor and sirolimus usage. As the intrarenal cyclosporin concentration is thought to be significantly elevated when cyclosporin and sirolimus are used together, this may explain these findings, and mandates caution in their co-administration.
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PMID:Thrombotic micro-angiopathy with sirolimus-based immunosuppression: potentiation of calcineurin-inhibitor-induced endothelial damage? 1261 89


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