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)

The renal manifestations of systemic lupus erythematosus in childhood range from minor abnormalities detected on urinalysis to severe renal insufficiency requiring renal replacement therapy. Clinically significant renal involvement in systemic lupus erythematosus is more common in children than in adults. Effective treatment for childhood lupus nephritis is available, and the prognosis for affected children has improved over the course of the last 30 years. Corticosteroid therapy remains the cornerstone of treatment for children with lupus nephritis. The addition of cytotoxic agents to corticosteroid treatment improves both the long and short-term prognoses. Cyclosporin may improve the clinical manifestations of lupus nephritis although the disease remains active serologically. Although survival in childhood lupus has improved, complications of therapy result in significant morbidity with distressing frequency. Immunosuppression may result in mortality and morbidity due to opportunistic infections. Individuals with otherwise successful control of renal manifestations of systemic lupus erythematosus may still be left with significant morbidity due to disturbances in growth due to long-term corticosteroid treatment. Psychosocial development may be adversely affected both as a result of chronic illness as well as due to the effects of therapy. Meticulous attention to detail over decades of treatment is necessary to optimize patient outcome in childhood lupus nephritis.
Lupus 1998
PMID:Lupus nephritis in children. 988 3

Like systemic lupus erythematosus (SLE) itself, manifestations of lupus nephritis are highly varied in their clinical presentation, ranging from mild proteinuria to rapidly progressive glomerulonephritis causing renal insufficiency within weeks. The clinical variability is in keeping with the broad spectrum of histological abnormalities present in renal biopsy specimens from these patients. The therapeutic modalities currently being used in lupus nephritis include oral steroids, pulse methylprednisolone and cytotoxic drugs such as cyclophosphamide and azathioprine either singly or in combinations, depending on the World Health Organisation morphologic classification of the disease. The use of plasmapheresis for proliferative lupus nephritis (WHO class III and IV) and cyclosporin for membranous lupus nephritis (WHO class V) is based on open trials, but not supported by randomised controlled trials. This review assesses the therapeutic modalities available for the treatment of lupus nephritis, giving the available evidence from the literature and acknowledging that none of them might be perfect.
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PMID:Treatment options in lupus nephritis. 1006 98

Autoimmune phenomena are common in human immunodeficiency virus (HIV) infection, yet systemic lupus erythematosus (SLE) and HIV infection rarely are seen concurrently in the same patient. Many of the cases of combined HIV infection and SLE reported in the literature are patients with SLE before HIV infection and who did not undergo renal biopsy at a time when both processes were present. We report the clinical manifestations and renal biopsy findings in four subjects with concurrent HIV infection and SLE and compare them with the seven previously reported cases in the literature. Taken together, most patients were black (91%) and male (73%), and approximately half (55%) were children with perinatal HIV infection. These demographics differ markedly from those of idiopathic SLE, a disease that predominantly affects female adults. Renal presentations included proteinuria and hypocomplementemia, frequently with hematuria and renal insufficiency. Renal biopsy findings in 10 cases included all classes of lupus nephritis (class IIb in two cases, class III in one case, class IV in three cases, class V in three cases, class III and V in one case), two of which also displayed overlapping features of HIV-associated nephropathy (HIVAN). One case had isolated findings of HIVAN. This cohort provides a unique population in which to study interacting pathomechanisms between HIV infection and SLE.
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PMID:Renal manifestations of concurrent systemic lupus erythematosus and HIV infection. 1007 Sep 7

Even 10 yr after the identification of the antiphospholipid syndrome (APS), renal involvement in the course of APS is still relatively unrecognized, and is probably underestimated. The association of anticardiolipin antibodies and/or lupus anticoagulant with the development of a vaso-occlusive process involving numerous organs is now confirmed. In a multicenter study, 16 cases of "primary" APS (PAPS) were found and followed for 5 yr or more, all with renal biopsy. In all 16 cases of PAPS, there was a vascular nephropathy characterized by small vessel vaso-occlusive lesions associated with fibrous intimal hyperplasia of interlobular arteries (12 patients), recanalizing thrombi in arteries and arterioles (six patients), and focal cortical atrophy (10 patients). In combination, these led to progressive destruction of the kidney, accelerated by acute glomerular and arteriolar microangiopathy in five patients. Focal cortical atrophy is a distinctive lesion, present in 10 biopsies, and likely represents the histologic and functional renal analogue to the multiple cerebral infarcts detected on imaging studies. The clinical hallmark of this vascular nephropathy in PAPS is systemic hypertension, only variably associated with renal insufficiency, proteinuria, or hematuria. The ensemble of histologic renal lesions defined in this study should aid in the separation of the lesions found in cases of secondary APS, especially systemic lupus erythematosus, into those lesions related to APS and those related to the underlying disease.
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PMID:The intrarenal vascular lesions associated with primary antiphospholipid syndrome. 1007 1

A 44-year-old patient was referred with weight loss of some 6 kg in two months, weakness and diarrhoea. According to the criteria of the American College of Rheumatology (ACR), systemic lupus erythematosus (SLE) was diagnosed: photodermatosis, nephropathy, and pancytopenia with positive antinuclear antibodies and antibodies against native DNA. In addition, adrenal failure was diagnosed with hyponatraemia, relapsing fever, low baseline cortisol and impaired response to ACTH stimulation. Clinical features of SLE may obscure signs of adrenal insufficiency, and hence, diagnosis is jeopardized. SLE combined with Addition's disease is rare. In some patients with both disorders, antiphospholipid antibodies, as found in our patient, are considered responsible for the development of Addison's disease. Possible pathogenetic mechanisms such as adrenal haemorrhage or (micro)thrombosis are discussed. The patient's condition significantly improved under steroid therapy. The progression of renal insufficiency (histology: mesangioproliferative glomerulonephritis), however, required additional immunosuppression with cyclophosphamide.
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PMID:[An uncommon case of lupus]. 1042 92

Renal insufficiency is one of the most severe outcomes of systemic lupus erythematosus (SLE). The aim of this study was to identify baseline predictors of the development of renal insufficiency in a cohort of patients with SLE. 281 patients from the The Hopkins Lupus Cohort (HLC) enrolled between 1987-1994 were followed for the occurrence of renal insufficiency, defined as a serum creatinine 1.6 mg/dl for men and 1.4 mg/dl for women. Over a mean (+/-s.d.) of 3. 3+/-2.1 y of follow up, 46 (16%) of the 281 patients developed renal insufficiency. Using a multivariate Cox proportional hazard model, we found the risk of renal insufficiency associated with younger (0-19 y) or older (40 y) age at baseline (relative risk (95% CI) 5.1 (1.4, 18.8) and 4.1 (2.1, 8.2)) and longer duration of SLE before referral to the cohort (RR 1.25 [1.05, 1.5] for every five years). Additional predictive variables were borderline elevation of serum creatinine at baseline (RR 3.1 (1.4, 6.6) for a serum creatinine 1. 4-1.5 mg/dl for men and 1.2-1.3 mg/dl for women), and mean proteinuria (RR 3.6 (1.8, 7.4) for trace-3+ and 10.6 (3.8, 30.0) for 3+ (urine dipstick level)). Socioeconomic status, race, autoantibodies and complement were not significantly associated with the risk of renal insufficiency. This study supports early referral of SLE patients to rheumatologists and emphasizes the importance of early signs of renal involvement as predictors of later renal insufficiency in SLE patients.
Lupus 1999
PMID:Risk factors for hypercreatinemia in patients with systemic lupus erythematosus. 1048 31

In children, systemic lupus erythematosus (SLE) is often more severe than in adults. Renal disease is very common in SLE, with clinical symptoms of renal involvement occurring in 30%-70% of patients. In the absence of appropriate treatment the child may die from the disease or progress rapidly to renal failure. However, aggressive treatment regimens, in particular corticosteroids, carry the risk of growth retardation, accelerated atherosclerosis, and severe infectious complications. Lupus nephritis is classified into six groups depending on the severity of the histological lesions. The most-appropriate treatment for optimal efficacy with minimal side-effects depends on the disease severity. Mild lesions (class I or II) require only careful follow-up to identify any disease progression. Patients with class III nephropathy (focal and segmental glomerulonephritis) may have mild clinical symptoms, in which case no specific therapy is indicated, or more-severe symptoms of the nephrotic syndrome, hypertension, and sometimes moderate renal insufficiency. These patients require the same aggressive therapy as those with class IV disease (diffuse proliferative glomerulonephritis). Our current protocol starts with three methylprednisolone pulses followed by 1.5 mg/kg per day oral prednisone and six monthly pulses of cyclophosphamide. After a second renal biopsy the patient may be maintained on azathioprine while the prednisone dosage is slowly tapered. In children with milder disease we use lower doses of oral prednisone (1-1.5 mg/kg per day). Patients with membranous glomerulonephritis (class V) require no specific therapy if they have pure membranous nephropathy, but require aggressive therapy if they have the nephrotic syndrome. In those patients who progress to end-stage renal disease, clinical and serological remission is common and renal transplantation can be performed, as recurrence in the transplant is very rare.
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PMID:Treatment of lupus nephritis in children. 1068 69

We examined 159 patients with thrombotic thrombocytopenic purpura and hemolytic uremic syndrome in Japan. The subjects were divided in three groups; 90 patients with thrombotic thrombocytopenic purpura, 51 patients with verotoxin-induced hemolytic uremic syndrome, and 18 patients with drug-induced hemolytic uremic syndrome. Eighty-two percent of the patients with thrombotic thrombocytopenic purpura had associated neurological disorders and 78% of drug-induced hemolytic uremic syndrome associated with pulmonary edema. Renal insufficiency was noted in the 69% cases with both hemolytic uremic syndrome groups. Seventeen patients with thrombotic thrombocytopenic purpura had systemic lupus erythematosus and 6 were pregnant. Autoantibody were positive in 53% of thrombotic thrombocytopenic purpura. Seventy-seven percent of patients with thrombotic thrombocytopenic purpura received plasma exchange at 4,000 mL/day three times a week, 71% antithrombotic agents, and 78% steroid administration, respectively. However, 27% of the patients with hemolytic uremic syndrome were treated by hemodialysis in addition to antithrombotic agents. When drug-induced hemolytic uremic syndrome was diagnosed, the drug was immediately discontinued and the patients were treated with antiplatelet agents. Seventy-four percent of the patients with thrombotic thrombocytopenic purpura were alive at 26 weeks compared with 95% of those with hemolytic uremic syndrome. As thrombotic thrombocytopenic purpura had a high mortality rate in Japan, we should carry out early diagnosis and early treatment.
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PMID:Outcome of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome in Japan. 1072 90

Coronary artery disease (CAD) is a major cause of morbidity and mortality in SLE, including the Hopkins Lupus Cohort. Currently, 9% of the cohort have had clinical evidence (angina or myocardial infarction) of CAD. In our initial prospective study we found that duration of prednisone, hypertension, hyperlipidemia and obesity were risk factors for later CAD. We can now extend that list to include age, male sex, elevated homocysteine, renal insufficiency and antiphospholipid antibodies. Many of the risk factors are amenable to intervention, but the timing of intervention, and the effectiveness of intervention, must be determined.
Lupus 2000
PMID:Detection of coronary artery disease and the role of traditional risk factors in the Hopkins Lupus Cohort. 1080 83

C1q nephropathy is an immune complex glomerulonephritis defined by the presence of mesangial immunoglobulins and complement deposits, most notably C1q, and the absence of clinical and laboratory evidence of systemic lupus erythematosus. Histology in C1q nephropathy is characterized by a slight to severe increase in mesangial cellularity and matrix, with or without segmental sclerosis. C1q nephropathy usually presents with nephrotic-range proteinuria in older children and young adults, and has a poor response to steroids. Patients may have decreased creatinine clearance at presentation, but progression to end-stage renal disease (ESRD) is slow. Severe crescentic glomerulonephritis has not been reported in C1q nephropathy. We describe a 3-year-old Hispanic girl who presented with renal insufficiency. Kidney biopsy showed C1q nephropathy with severe crescentic glomerulonephritis. The clinical and serological evaluation ruled out systemic lupus erythematosus or other immunological or infectious etiologies. In spite of immunosuppressive therapy, she progressed to ESRD within 14 weeks and is currently on chronic peritoneal dialysis. The atypical features of C1q nephropathy observed in our patient, which have not been described in earlier reports, are an early age of onset, severe crescentic glomerulonephritis, and rapid progression to ESRD. C1q nephropathy should be added to the differential diagnosis of glomerulonephritis in young children and in the patient with crescentic glomerulonephritis.
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PMID:C1q nephropathy presenting as rapidly progressive crescentic glomerulonephritis. 1097 10


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