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)

Guillain-Barre syndrome (GBS) is a transient neurological disorder characterized by an inflammatory demyelination of peripheral nerves. Although the pathogenesis of GBS has not been elucidated, there is increasing evidence pointing to an autoimmune etiology. We have studied the reactivity of GBS sera with various phospholipids which are known to be important constituents of myelin, and serve as autoantigens in other autoimmune conditions. Sixteen Guillain-Barre syndrome (GBS) sera were studied for the presence of autoantibodies to ssDNA, dsDNA, cardiolipin (CL), phosphatidyl-ethanolamine (PE), phosphatidyl-choline (PC), phosphatidyl-serine (PS), and brain extract. Six of the 16 GBS sera had autoantibodies to one or more of the antigens studied. Three of the sera contained autoantibodies to brain extract (p < 0.05), two of the sera had autoantibodies to dsDNA, ssDNA, CL and PE, and one serum had autoantibodies to PC, and PS. As expected a significant proportion of the lupus sera contained autoantibodies to ssDNA and dsDNA, while the frequency of autoantibodies to different phospholipids was significantly high in sera of patients with systemic lupus erythematosus (SLE) and cerebritis. Absorption of GBS sera with cardiolipin, phosphatidyl-choline, or brain extract inhibited the binding of the sera to cardiolipin. Our results demonstrate that some GBS patients produce autoantibodies to various phospholipid and nuclear antigens. However, these autoantibodies are probably produced as a result of the myelin damage rather than cause the demyelination.
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PMID:Autoantibodies to phospholipids and brain extract in patients with the Guillain-Barre syndrome: cross-reactive or pathogenic? 813 63

Individual case reports have documented nephrotoxicity of intravenous immunoglobulin (IVIG) preparations, but the true incidence of renal dysfunction is unknown and many data sheets do not include renal impairment as a side-effect of these preparations. We determined the incidence of renal impairment in an unselected cohort of patients receiving two different preparations of IVIG over 20 months, administering 287 courses of IVIG to 119 patients for a variety of indications, including thrombocytopenia, systemic lupus erythematosis, neuropathy, Guillain-Barre syndrome and infections. Two different preparations of IVIG were used, Vigam (BPL) and Sandoglobulin (Novartis), which differ in the concentration of sucrose added as a stabilizer. Eight patients showed deterioration in renal function (6.7%), and in two, no renal recovery occurred (1. 7%). There were no significant differences in the patient characteristics or dose or preparation of IVIG administered to those patients with or without changes in serum creatinine. There was no association between the amount of sucrose in the IVIG and development of renal failure. IVIG (regardless of the sucrose content) is associated with renal impairment which may be irreversible, with a maximum incidence of 6.7%. All patients should have their renal function monitored during the use of IVIG.
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PMID:Nephrotoxicity of intravenous immunoglobulin. 1107 32

Apheresis has developed into a common therapeutic modality. The evidence for the clinical benefit of apheresis, however, is generally from uncontrolled and nonrandomized trials. Although the effectiveness of therapeutic apheresis may be established from uncontrolled trials, well-designed, randomized controlled trials provide the best evidence for a clinical benefit because this type of trial design minimizes bias. We assess the evidence for the use of apheresis, the optimal schedules for apheresis, and the replacement solutions used, based on randomized controlled trials. The databases, MEDLINE and EMBASE, and reference lists from relevant articles were searched. The literature search was restricted to articles published in English and included adult patients only. Two of the authors (NS, CK) independently reviewed the citation list to identify reports for retrieval, and the kappa statistic was used to quantify agreement between the reviewers. Articles were included if they used apheresis as an intervention and had clinical endpoints. The quality of the studies was assessed by using a validated instrument. Five hundred and ninety-two citations were identified, and a total of 85 reports were included in this review. There are only a few well-established indications for therapeutic apheresis. Randomized, controlled trials have clearly shown a benefit for patients treated with apheresis who have Guillain-Barre syndrome and patients who have thrombotic thrombocytopenic purpura. A clinical benefit has not been shown for a variety of conditions, including systematic lupus erythematosus, polyarteritis nodosa/Churg-Strauss syndrome, and for the treatment of renal allograft rejection. The effectiveness of apheresis needs to be clarified for several other diseases. As better-designed, randomized trials are performed, the role of apheresis for these indications will be further elucidated.
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PMID:A review of randomized controlled trials using therapeutic apheresis. 1207 59

Background: Systemic lupus erythematosus (SLE) is an autoimmune disease which involves multiple organs, including peripheral nervous system. Case presentation: We describe a 12-year-old boy with progressively worsening neurological symptoms as first manifestation. Legs pain, loss of balance, and lower extremity weakness were the reason for his admission in neurologic ward. The patient was started on intravenous immunoglobulin therapy due to the possibility of Guillain-Barre syndrome and acute inflammatory demyelinating polyneuropathy (AIDP). However, there was no appropriate response and he developed recurrent attacks of polyneuropathy again with diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP). Then, he received intravenous pulse of methylprednisolone for 5 consecutive days followed by oral prednisolone for 3 months. One month after withdrawal of corticosteroid he admitted again with the same manifestations. Rheumatologic workup revealed the presence of leukopenia, hemolytic anemia, hematuria, proteinuria, positive antinuclear antibodies, and ds-DNA antibodies. On the basis of the American College of Rheumatology and Systemic Lupus International Collaborating Clinics Classification Criteria for SLE, the patient had underlying diagnosis of SLE. Eventually, he was treated by the pulse of methylprednisolone and cyclophosphamide, and oral hydroxychloroquine and prednisolone. His neurological and physical symptoms improved and complete neurological recovery occurred several months later. Conclusion: SLE and AIDP/CIDP are different entities, but ADP/CIDP can be part of the neurologic manifestations of the SLE. Although the association between AIDP/CIDP and SLE is very rare especially as a first manifestation of SLE, it should be early recognized for rapid appropriate treatment.
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PMID:Guillain-Barre syndrome as the first manifestation of juvenile systemic lupus erythematosus: a case report. 3111 4