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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 renal biopsy technique has made it possible to classify lupus nephritis into its varied forms. Utilizing light histology, immunofluorescence microscopy, and ultrastructural techniques, the following renal morphological manifestations of systemic lupus erythematosus can be identified: mesangial abnormalities; focal proliferative, diffuse proliferative and membranous glomerulonephritis; glomerular sclerosis; interstitial nephritis; vascular sclerosis and necrotizing renal vasculitis. Each of the morphological forms is associated with distinctive clinical features and prognosis. Mesangial and focal proliferative lupus nephritis may occur in the absence of clinical abnormalities, and in general have a favorable prognosis. Diffuse proliferative lupus nephritis often is manifested by the nephrotic syndrome and renal functional impairment which proves to be irreversible and progressive. Transition from the milder forms to diffuse proliferation occurs in about one-sixth of patients. Membranous lupus nephritis is characterized by the nephrotic syndrome, which often is persistent, but renal functional impairment develops slowly and is rarely severe. Necrotizing vasculitis, which supervenes on occasion during the course of diffuse proliferative lupus nephritis, produces the clinical picture of malignant hypertension and progresses rapidly to uremia. Interstitial nephritis usually occurs in combination with one of the glomerular forms, but at times may be the predominant renal lesion both morphologically and clinically. Glomerular sclerosis, often associated with hypertension and vascular sclerosis, commonly develops in the course of lupus nephritis, especially in the more severe forms, and may progress even though active disease has remitted. An awareness of clinico-pathologic correlations in lupus nephritis provides a basis for intelligent management and critical assessment of therapy.
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PMID:Clinical usefulness of the morphological classification of lupus nephritis. 710 64

Thirty of 118 cases of childhood onset systemic lupus erythematosus collected in the Parisian area had an unfavorable outcome. Thirteen patients evolved to end-stage renal failure. Seven survived with renal substitution therapy, and 6 other patients subsequently died. Most had diffuse proliferative glomerulonephritis, the pattern of glomerular disease classically responsible for end-stage renal failure. Three patients had membranous nephropathy with segmental lesions, a form of glomerulonephritis whose severe prognosis should be emphasized. In another patient, end-stage renal failure was precipitated by thrombotic microangiopathy. Seventeen other patients died and in most, the causes of death were multiple. In 7, death could be attributed to complications secondary to an infection and in 4 other cases to SLE exacerbation with severe organ involvement. Two patients died suddenly, another showed cardiac failure and another had malignant hypertension. Of the remaining 2 patients, one suffered anticoagulant therapy complications after treatment for renal artery stenosis and the second, an urothelial carcinoma. Unfavorable evolutions were high among patients coming from French departments and territories, and among North African patients. One may speculate that poor outcome is associated with ethnic characteristics or with socioeconomic factors. However, the problem of compliance with treatment is clearly an extremely important factor in the prognosis. Both end-stage renal failure and death were in some of our cases precipitated by treatment interruption, indicating an insufficient understanding of the importance of treatment in this chronic disease.
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PMID:Unfavorable outcomes (end-stage renal failure/death) in childhood onset systemic lupus erythematosus. A multicenter study in Paris and its environs. 795 30

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

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

For various ethnic and socioeconomic reasons the pattern of renal disease in the inner city displays distinctive features. Hypertension is frequent, often intractable, and generally conditioned by salt sensitivity and a high sodium intake. Chronic hypertensive nephrosclerosis, found predominantly in African Americans, comprises marked cardiomegaly, renal shrinkage, and hypertensive retinopathy. It has been overdiagnosed in the past, but actually accounts for less than 20% of end-stage renal disease (ESRD) in African Americans. Malignant hypertension, less frequent nowadays, may cause renal shutdown, which is reversible in a few cases; the heart and kidneys are often of normal size. Idiopathic focal segmental glomerulosclerosis is the most common cause of the primary nephrotic syndrome in blacks, but its incidence has also been rising in whites and Hispanics; it does not respond well to treatment, and almost one half of the patients develop ESRD within 10 years. Systemic lupus erythematosus is also more common in African Americans, in whom the severe proliferative forms of lupus nephritis pursue a more virulent course: one half of such patients develop ESRD in 5 years. Cocaine, the use of which has assumed epidemic proportions, may cause accelerated hypertension, acute renal failure from rhabdomyolysis, and progression of preexisting renal disease. Heroin nephropathy has all but disappeared and has been replaced by human immunodeficiency virus (HIV) nephropathy. The prognosis of HIV-infected patients maintained by dialysis has greatly improved. Sickle glomerulopathy, consisting of mesangial expansion, basement membrane duplication, and the absence of immune deposits, may cause the nephrotic syndrome in 4% of patients with severe sickle cell anemia, heralding death within 2 years in one half of patients and ESRD in two thirds; survival has not improved with dialysis. Diabetes is now the most common cause of ESRD. Familial aggregation of ESRD is frequently encountered. Interventions useful in the general population, such as vascular bypass procedures, should be undertaken with great caution and restraint in dialysis patients.
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PMID:Renal disease in the inner city. 1145 21

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

The antiphospholipid syndrome is defined by the presence of antiphospholipid antibodies and recurrent thrombosis, affecting the venous system more frequently than the arterial one. Renal involvement is only observed in approximately 20-25% of cases, main renal artery thrombosis has been exceptionally described. We report a 39-year-old woman with previous history of recurrent thrombosis diagnosed as primary antiphospholipid syndrome, who presented malignant hypertension in the context of a renal artery thrombosis. She had a high IgG anticardiolipin antibody titre and positive lupus anticoagulant. An isotopic renogram demonstrated asymmetrical activity (60% right vs 40% left kidney). Renal arteriography demonstrated preoclusive thrombosis in the left renal artery. Blood pressure was well controlled by the use of ACE-inhibitor and alpha blockers.
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PMID:[Primary antiphospholipid syndrome associated with malignant hypertension]. 1521 79

The diagnosis of antiphospholipid syndrome (APS) relies on clinical and laboratory criteria, which have been recently outlined in specific consensus conferences. Renal involvement in APS is not infrequent and includes different clinical patterns. For clinical purposes a distinction can be made between large vessel and microvascular involvement. Renal artery stenosis is frequent in APS. In case of microvascular involvement with an acute clinical course a differential diagnosis with other thrombotic microangiopathic diseases has to be made, taking in account thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, malignant hypertension, drug nephrotoxicity (cyclosporin) and others. The disease is often chronic, with hypertension, different degrees of renal insufficiency and mild proteinuria. In patients with systemic lupus erythematosus and antiphospholipid antibodies the prognosis of kidney disease is generally poorer than in lupus alone. Finally, the kidney is almost invariably a target in catastrophic antiphospholipid syndrome. Anticoagulation is the therapy of choice, especially in arterial stenosis and acute disease, but is probably also indicated in chronic and subacute patterns. The role of immunomodulatory therapy has to be assessed.
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PMID:[Antiphospholipid syndrome and kidney]. 1745 26

Systemic lupus erythematosus (SLE) has been described as a cause of microangiopathic haemolytic anaemia (MAHA), however there is little literature to support this assertion. We report on three patients presenting with SLE and MAHA with a clinical picture indistinguishable from thrombotic thrombocytopenic purpura (TTP), who had underlying lupus nephritis. They all had significant proteinuria and normal Von Willebrand Factor cleaving protease (vWF-CP) levels. Their MAHA fitted better for haemolytic syndrome (HUS) and their cerebral signs were explained either by malignant hypertension or cerebral lupus. Their MAHA only improved when the appropriate treatment for lupus nephritis was given. We propose that the previously described association between SLE and MAHA, in actuality relates to the underlying presence of lupus nephritis causing haemolytic uraemic syndrome, not TTP. Significant proteinuria was present in all cases of MAHA due to lupus nephritis, so may be a useful discriminatory sign. Furthermore the demonstration of a normal vWF-CP assay aided in the distinction between TTP and MAHA due to lupus nephritis. All our patients responded to mycophenolate mofetil suggesting this may be useful in other cases of lupus nephritis causing HUS.
Lupus 2007
PMID:Microangiopathic haemolytic anaemia secondary to lupus nephritis: an important differential diagnosis of thrombotic thrombocytopenic purpura. 1757 39


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