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)

A 36-year-old woman was admitted to the hospital with the diagnosis of nephrotic syndrome due to lupus nephritis. The patient had panperitonitis caused by Staphylococcus aureus as a complication, and an emergency laparotomy was performed. After the operation, the patient developed a massive lung alveolar hemorrhage. Methylprednisolone pulse therapy showed a marked effect on the lung hemorrhage. It is known that lung alveolar hemorrhages associated with systemic lupus erythematosus have a very high mortality; the present case is relatively rare because of the good response to steroid pulse therapy.
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PMID:Lupus nephritis associated with bacterial panperitonitis and lung alveolar hemorrhage. 150 36

A 33-year-old woman presented with chronic diarrhea, vomiting and anasarca due to systemic lupus erythematosus with protein-losing enteropathy, interstitial cystitis and glomerulonephritis. Methylprednisolone could not prevent aggravation of diarrhea, obstructive uropathy, and nephrotic syndrome, and prolonged intestinal ileus developed. Because of this steroid-resistance, bolus injections of cyclophosphamide (1 g i.v. monthly) were decided. Protein-losing enteropathy and ileus both disappeared rapidly following the first injection. Protein-losing enteropathy, intestinal ileus and interstitial cystitis are exceptional manifestations of systemic lupus erythematosus; steroid-resistance of the digestive manifestations has only been reported in one case and our observation is the first reporting the efficacy of cyclophosphamide.
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PMID:[Exudative enteropathy and interstitial cystitis due to systemic lupus erythematosus]. 226 25

A 43-year-old woman who had four recurrences of acute transverse myelitis (ATM) at the fifth thoracic vertebral level in 7 years is reported. Her haematological and immunological abnormalities included antibodies to DNA and nRNP and markedly reduced serum complement during the course of the disease. These findings suggest systemic lupus erythematosus although she had no symptoms other than those of ATM. Methylprednisolone and prednisolone were effective in treating the ATM.
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PMID:Recurrent transverse myelitis associated with collagen disease. 348 25

A pedigree of C1 inhibitor (C1 INH) deficiency associated with positive LE cell and an elevated titer of DNA antibodies and antinuclear factor (ANF) and nephropathy was presented. The proband of this family was diagnosed as having definite systemic lupus erythematosus (SLE) after a clinical course of several year since her first visit to our hospital and because of the lack of hemolytic activity of complement (CH50), in spite of the absence of idiopathic edema, C1 INH levels were 1.2 mg/dl (3.8% NHS) determined as antigen and 65 site forming unit (SFU) (5.8% NHS) determined by hemolytic assay in her blood. Her mother and brother had characteristic idiopathic edema of her face, larynx, hand and bowel and they had low levels of C1 INH of 1.8 mg/dl (5.8% NHS) and 4.8 mg/dl (15.5% NHS) respectively in their blood. On the basis of these findings, this family was diagnosed as having a pedigree of hereditary angioneurotic edema (HANE) which is supposedly an inherited autosomal positive trait. Actually, however, the proband's serological and hematological indices became positive and progressed year by year, which implies that SLE was absent for the first several years. It might be said that this interesting clinical course indicates that SLE appeared chronologically as a hereditary deficiency in one of the complement components in this case. In concurrence with the general observation that recurrent viral infections due to the deficiency of complement components are presumed to be responsible for SLE-like disease. Her levels of several kinds of anti-virus antibodies were high. Methylprednisolone helped normalize her level of C1 INH and ameliorate the clinical course.
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PMID:A case of hereditary angioneurotic edema associated with systemic lupus erythematosus. 377 20

This study was done to determine whether intravenous methylprednisolone therapy given concomitantly with low-dose daily, oral prednisone would be as effective as high-dose daily prednisone in the treatment of patients with active systemic lupus erythematosus (SLE) nephritis. Thirteen patients with active SLE nephritis were started on 2 mg/kg prednisone per day, considered the high prednisone phase. Therapy was continued until remission was achieved. Prednisone administration was then tapered to less than 0.5 but more than 0.2 mg/kg per day. On later relapse, these patients received three doses of methylprednisolone (20 mg/kg per dose) on alternate days and continued on the same daily dose of prednisone (less than 0.5 greater than 0.2 mg/kg per day) prior to pulse therapy; this was the methylprednisolone phase. The 13 patients were studied in both phases, serving as their own controls. After 1 month of therapy, no significant differences were observed between treatment phases as to improvement in clinical and laboratory findings. A significant increase in the serum concentration of C3 and C4 was seen both in the high-dose prednisone and methylprednisolone phases, while the serum concentration of anti-ds DNA antibody significantly decreased. Methylprednisolone therapy seems as effective as high-dose prednisone in patients with relapse of SLE nephritis. Because side effects are minimal, methylprednisolone administration may be tried as the therapy of choice for these patients.
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PMID:Pulsed methylprednisolone therapy compared to high dose prednisone in systemic lupus erythematosus nephritis. 379 82

Involvement of the central nervous system associated with SLE (CNS lupus) is not rare. Two types of CNS lupus are noted clinically; one group manifesting focal neurological symptoms and another group showing mental symptoms. Though it is well known that neurological symptoms are caused by arteritis and thrombus, pathophysiological mechanisms leading to mental symptoms remain obscure and there is no established clinical evidence responsible for these symptoms. A 41-year-old woman was confused and her attention was markedly impaired. Her mental symptoms consisted of disorientation, restlessness, euphoria and emotional incontinence. There were neither focal neurological signs nor meningeal signs. Cerebrospinal fluid (CSF) examination showed increased number of polymorphonuclear cells and permeability of the blood-brain barrier, calculated based on the CSF/plasma protein ratio, was also elevated. Repeated bacteriological examinations revealed to be negative. Gd-DTPA MRI demonstrated diffuse enhancement of the cerebral leptomeninges. Methylprednisolone pulse therapy ameliorated her mental deterioration effectively, and subsequently the leptomeningeal enhancement with Gd-DTPA MRI disappeared in parallel. These radiological and laboratory findings suggest that SLE itself causes inflammation of vessels in the leptomeninges and adjacent cerebral cortex. We consider mental symptoms associated with SLE may be caused, at least in part, by this mechanism. To our knowledge, we could not find similar reports in the literature. Gd-DTPA enhanced MRI seems to be of clinical use for making diagnosis, evaluating clinical activity and understanding of CNS lupus.
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PMID:[Leptomeningeal abnormality on Gd-DTPA enhanced MRI in a case of SLE presenting diffuse organic brain syndrome]. 833 80

Renal involvement i.e. lupus nephritis (LN) in systemic lupus erythematosus (SLE) mainly determines course and outcome of the disease. Recognition of early manifestations of LN makes adequate therapy possible, with very good therapeutic results. We report 7 patients from a group of 150 SLE patients under our permanent control, 4 female and 3 male, mean age 21 years. All of them had signs of LN: proteinuria 7/7 haematuria 4/7 without azotaemia. Renal biopsy was performed in 6 pts, and histological finding was: class II 1 pt, class IV 3 pts and class V in 2 pts. In 4 pts tubulointerstitial changes were noted, while all showed immunofluorescent deposits of immunoglobulins and complement. Methylprednisolone "pulse" therapy (1000 mg, i.v., 3 days) followed by tapering of the steroid dose was given. Reduction of proteinuria and disappearance of haematuria were observed in all patients. During follow up, kidney function remained normal.
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PMID:[Methylprednisolone pulse therapy in the early phases of lupus nephritis]. 910 36

Severe cerebral involvement may occur in patients with systemic lupus erythematosus. Methylprednisolone pulse therapy is a management choice for those with severe cerebral involvement. However, its effectiveness is uncertain. This article describes Tc-99m HMPAO cerebral perfusion single-photon emission computerized tomography to document the restoration of cerebral perfusion after methylprednisolone pulse therapy in a case of systemic lupus erythromatosus with severe cerebral manifestations.
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PMID:Quantitative cerebral perfusion SPECT in systemic lupus erythematosus with severe central nervous involvement--before and after methylprednisolone pulse therapy. 944 96

Aggressive immunosuppressive therapy should be considered for patients with proliferative lupus nephritis as the risk for progression to end stage renal disease is high. Intermittent intravenous cyclophosphamide therapy improves renal survival; longer duration of therapy is associated with fewer relapse of nephritis and decreased risk of diminished renal function. While azathioprine therapy does not differ statistically from steroids alone in prolonging renal survival, this therapy may be considered in patients with few risk factors for progression to renal insufficiency. Methylprednisolone as a single therapy does not prolong renal survival compared with regimens including cyclophosphamide. Plasmapheresis remains under study but has not shown additional benefit in treatment of severe lupus nephritis. The potential roles for cyclosporin A and mycophenylate mofetil in the therapy of proliferative lupus nephritis remain to be defined. Supportive care including rigorous control of hypertension, consideration of angiotensin receptor inhibition or blockade to reduce proteinuria and prolong renal function, control of hyperlipidemia, prevention of osteoporosis, and prevention of pregnancy remain important clinical goals. Current research efforts focus on genetic and socioeconomic factors involved in racial differences in expression of lupus nephritis, hormonal manipulation to preserve gonadal function during cyclophosphamide therapy, and the potential impact on lupus activity of estrogen-containing oral contraceptives or postmenopausal hormone replacement therapy.
Lupus 1998
PMID:Immunosuppressive therapy of lupus nephritis. 988 1

We report a case of systemic lupus erythematosus (SLE) associated with crescentic glomerulonephritis and myeloperoxidase-specific anti-neutrophil cytoplasmic antibodies (MPO-ANCA). A 34-year-old Japanese female patient diagnosed with SLE developed rapidly progressive renal failure and nephrotic syndrome. Haemodialysis was required to restore renal function. Methylprednisolone pulse therapy followed by plasmapheresis did not suppress the progression of renal failure, so she was treated with high-dose intravenous immunoglobulin (IV-IG) therapy, which was well tolerated and effectively prevented renal failure. A renal biopsy showed diffuse proliferative lupus nephritis (WHO classification IVc) with predominant crescent formation and scant subendothelial immune deposits. These findings indicate that, in addition to lupus nephritis, which usually results from the deposition of circulating or locally formed immune complexes, MPO-ANCA may be involved in the pathogenesis of crescentic glomerulonephritis. Furthermore, we propose that IV-IG is an effective therapy for MPO-ANCA-related renal crisis in lupus nephritis.
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PMID:Successful treatment of rapidly progressive lupus nephritis associated with anti-MPO antibodies by intravenous immunoglobulins. 1008 59


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