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)

Immusorba TR (IM-TR) and PH (IM-PH) were developed as adsorbents with non-biological materials as affinity ligands to remove pathogenic autoantibodies. The adsorbents of IM-TR and IM-PH are polyvinyl alcohol gel immobilized with tryptophan and phenylalanine as ligand, respectively. IM-TR is clinically applied for treatment of autoimmune neurological diseases such as myasthenia gravis and Guillain-Barre syndrome. IM-PH is used for not only neurological diseases such as GBS and multiple sclerosis but also collagen diseases such as rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). As many autoantibodies with different specificities have been found to have similar affinities to the ligand of Immusorba, it is expected that Immusorba will be applied to more diseases and contribute to the clarification of the mechanisms of the development of diseases by the identification of adsorbed unknown pathogenic substances with Immusorba.
Ther Apher Dial 2003 Feb
PMID:Immusorba TR and PH. 1292 Nov 21

Peptides as ligands for immunoadsorption exhibit several potential advantages over native proteins. Two newly developed adsorbers are based on peptides covalently coupled to sepharose CL-4B. Globaffin is capable of binding immunoglobulins independent from their antigen specificity and thus, applicable in transplant recipients and several antibody mediated autoimmune diseases. Among others, the most important disorders suitable for the treatment with Globaffin are rheumatoid arthritis, systemic lupus erythematosus, and acute renal transplant rejection. Coraffin is a specific adsorber using two linear peptide ligands mimicking epitopes of the beta1-adrenergic receptor, that bind corresponding autoantibodies from patients suffering from idiopathic dilated cardiomyopathy. Specific immunoadsorption has been shown to be beneficial for patients with dilated cardiomyopathy. Coraffin can be used as a new therapeutic option for these patients, who get only limited benefit from medical therapy. Both adsorbers may be combined with all approved apheresis control devices available.
Ther Apher Dial 2003 Feb
PMID:Peptide based adsorbers for therapeutic immunoadsorption. 1292 Nov 22

Autoantibodies against platelets are found in patients with autoimmune thrombocytopenic purpura (AITP) as well as in thrombocytopenia associated with systemic lupus erythematosus (SLE). The high titer of platelet associated immunoglobulin G (PA IgG) suggests the presence of antibodies against platelets. Platelet associated antibodies are thought to play a major role in the pathogenesis of autoimmune thrombocytopenia. There is a possibility that double filtration plasmapheresis (DFPP) is effective in removing the antibodies against platelets from the peripheral blood. It is suggested that DFPP may reinforce the efforts of the conventional treatment, especially high-dose intravenous immunoglobulin therapy (IVIg). DFPP was performed for severe thrombocytopenia on a SLE patient with high PA IgG value, refractory to the conventional therapy including corticosteroid therapy and IVIg. The patient underwent three sessions of DFPP combined with IVIg and the corticosteroid therapy immediately before the initiation of IVIg. The severe thrombocytopenia improved drastically after the combination treatment, accompanied with the decrease of the PA IgG titer. It was suggested that DFPP combined with corticosteroid and IVIg was effective for severe autoimmune thrombocytopenia on SLE patients refractory to the conventional therapies.
Ther Apher Dial 2004 Jun
PMID:A case report of the efficacy of apheresis for refractory autoimmune thrombocytopenia in a patient with systemic lupus erythematosus associated hemolytic anemia. 1515 76

Reduction of pathological autoantibodies may be useful in the treatment of systemic lupus erythematosus (SLE). On the other hand clinically manifested myocarditis in SLE, though uncommon, may be life-threatening and its pathogenesis has been ascribed to autoimmunity. The aim of this study is to present a rare case of a patient with severe lupus myocarditis, where immunoadsorption (IA) was evaluated as rescue therapy. A case of SLE with initial manifestation of myocarditis is reported in a 29-year-old male who presented with arthritis, fever, lymphadenopathy, joint swelling and morning stiffness. Laboratory evaluation revealed increased antinuclear antibody (ANA), slightly decreased complement and positive anticoagulant panel. From the above clinical and laboratory features, criteria of SLE seemed applicable. During his hospitalization, the patient developed pericardial effusion and cardiogenic shock. Although pericardiotomy was performed and was treated with immunosuppressive agents, plasmapheresis and supported with current medications, his clinical condition remained critical with an ejection fraction of 20%. At this point it was decided to receive IA onto staphylococcal protein A. After 6 sessions with IA and concomitant immunosuppression, the patient responded well, his condition improved and was dismissed with an ejection fraction of 50%. Fulminant lupus myocarditis is a severe and rare situation lacking a satisfying specific therapy available today. In our presented case, IA in addition to immunosuppressive therapy was beneficial. Considering the benefits of our case and the current knowledge, it might be useful to clarify the open question in scale pilot studies.
Ther Apher Dial 2004 Aug
PMID:Immunoadsorption in lupus myocarditis. 1527 78

We studied whether soluble CD40 ligands (sCD40L) are removed by means of double filtration plasmapheresis (DFPP), and the removal may help decrease activity of systemic lupus erythematosus (SLE). We studied 10 female patients with active SLE. Double filtration plasmapheresis was conducted one or two times per week. Plasma sCD40L levels were measured before and after each round of DFPP and throughout the treatment course. The plasma sCD40L level of SLE patients was significantly higher (14.09 +/- 18.88 ng/mL) than that of healthy individuals (0.19 +/- 0.20 ng/mL; P < 0.0001). In the SLE patients, plasma sCD40L levels were significantly lower following DFPP (P = 0.0251). The plasma waste from DFPP of an SLE patient was subjected to gel filtration, and the sCD40L concentration in each fraction was measured. We observed a peak in the fraction corresponding to > or =60 kDa. These results indicate that trimers and higher order complexes of sCD40L are removed during DFPP. Plasma sCD40L level and SLE disease activity index (SLEDAI) were decreased following the treatment course (mean 9.3 months). sCD40L exists as both a monomer and trimer in the plasma of SLE patients. The trimer as well as higher-order compounds can be removed via DFPP. It was thought that removal of sCD40L via DFPP may be useful for improving the overall condition of SLE.
Ther Apher Dial 2005 Feb
PMID:Study of plasma levels of soluble CD40 ligand in systemic lupus erythematosus patients who have undergone plasmapheresis. 1582 9

The pathogenesis of calciphylaxis, which has a rising incidence in the chronic dialysis population and a high mortality rate, is poorly understood. Abnormalities in the calcium-phosphorus-parathyroid axis are clinically related to calciphylaxis, but alone, they cannot explain this condition. Here, we present two patients who had chronic inflammatory conditions and hyperparathyroidism and who developed calciphylaxis. A 41-year-old white woman on hemodialysis following scleroderma, hepatitis C, liver transplant, and failed kidney transplant, developed progressive ulcerative lower extremity calciphylaxis lasting more than 3 years. She had evidence of severe hyperparathyroidism and elevated serum C-reactive protein (CRP). A 39-year-old white woman on continuous ambulatory peritoneal dialysis for 6 years for renal failure secondary to lupus nephritis, with sustained lupus activity during the dialysis period, developed rapidly progressing ulcerative calciphylaxis of the lower and upper extremities not responding to adequate treatment of hyperphosphatemia and hyperparathyroidism. Her condition culminated in death within 2 months of the appearance of the skin lesions. Her serum CRP was elevated on a sustained basis before the development of the calciphylaxis and rose to a very high level after appearance of the skin lesions. Inflammation may assist in the development of calciphylaxis through depression of serum levels of fetuin-A, an endogenous inhibitor of calcification that is also a negative acute-phase reactant. The interactions between inflammation-mediated changes in the levels of endogenous inhibitors of calcification and abnormalities in calcium-phosphorus metabolism merit intensive study in the future as potential mechanisms of calciphylaxis.
Adv Perit Dial 2006
PMID:Association between calciphylaxis and inflammation in two patients on chronic dialysis. 1698 64

Plasmapheresis (PP) is widely known as the standard therapy for thrombotic thrombocytopenic purpura (TTP). Citrate is used as an anticoagulant in fresh frozen plasma, and the large amount of citrate infused during PP induces metabolic alkalosis. A 29-year-old woman was diagnosed with TTP associated with systemic lupus erythematosus, and was treated by daily PP in addition to a steroid, an immunosuppressant, vincristine, and cyclophosphamide. Uncompensated alkalosis caused by a combination of metabolic and respiratory alkalosis developed after artificial ventilation was discontinued. Her metabolic status improved after controlling her respiratory status and the activity of the TTP. Metabolic alkalosis is a common complication in TTP patients treated by frequent PP, but several factors that affect metabolic status may aggravate the alkalosis and induce uncompensated alkalosis.
Ther Apher Dial 2008 Feb
PMID:A case report of uncompensated alkalosis induced by daily plasmapheresis in a patient with thrombotic thrombocytopenic purpura. 1825 19

Induction of factor VIII (FVIII) inhibitors sometimes occurs in patients with hemophilia due to frequent supplementation of FVIII. The inhibitor is rarely detected in non-hemophilic patients; however, an association has been described in patients with chronic inflammatory diseases, such as autoimmune diseases (e.g. SLE and rheumatoid arthritis), malignant tumors and drug allergies, and also to pregnant or aged individuals without underlying disease. We report on an 82-year-old patient who was transferred to our hospital after the diagnosis of acquired FVIII inhibitor. On admission the laboratory results showed no detectable FVIII activity (0%, normal range 70-100%), prolongation of coagulation time (APTT 102.4 s), and severe anemia 7.8 g/dL (normal range 12-16 g/dL). On physical examination multiple subcutaneous hematomas were detected and further bleeding in his left pectoralis muscle was observed. Despite extensive investigation no underlying disease was detected. Thirty-six courses of plasma exchange with 360 units of fresh frozen plasma replacement daily were conducted. High dose steroids and mycophenolate mofetil (MMF) were given throughout the course, and cyclophosphamide was administered once. Thirty-four units of erythrocytes were applied during this time. After 36 courses of plasma exchange in combination with high dose steroids, FVIII activity and coagulation time normalized and bleeding could be stopped. The patient was discharged in good health 48 days after admission. Hence, we present a case of severe idiopathic FVIII inhibitor-positive hemophilia successfully treated with the combination of plasma exchange, corticosteroids, cyclophosphamide and MMF.
Ther Apher Dial 2008 Oct
PMID:A case report of plasma exchange, steroids, mycophenolate mofetil and cyclophosphamide in acquired factor VIII inhibitors. 1893 26

Serological evidence of drug-induced lupus (DIL) and antiphospholipid syndrome (APS) were detected in a paediatric patient with nephropathic cystinosis during work-up for live related renal transplantation. Cysteamine was considered the most likely cause. Antinuclear (ANA) and antihistone antibodies disappeared after stopping cysteamine. ANA became positive after reintroduction of cysteamine. The patient's post-transplant course was complicated by severe thrombosis, with histological findings in her native nephrectomy consistent with APS. This is the first reported case of DIL and APS secondary to cysteamine therapy. Clinicians should exclude autoimmune abnormalities in patients with cystinosis, especially if patients report non-specific, unusual or unexplained symptoms.
Nephrol Dial Transplant 2009 Jun
PMID:Drug-induced lupus and antiphospholipid syndrome associated with cysteamine therapy. 1932 14

Conventional treatment of antiphospholipid syndrome (APS) pregnancies with aspirin and/or heparin is sometimes unable to counteract maternal and/or fetal complications. In this article we report the cases of two patients who were unresponsive to conventional treatment for APS during their first pregnancy, and who were treated in the following pregnancy with plasma exchange and immunoadsorption respectively, in addition to conventional therapy. Both patients had a history of thrombotic events, a previous pregnancy loss at the 11th week of gestation and the same antiphospholipid antibody profile (lupus anticoagulant activity and high titers of immunoglobulin G (IgG) anti-beta2 glycoprotein I and IgG anticardiolipin antibodies). Patient 1 was treated from the fourth week of her second pregnancy with weekly plasma exchange. Due to fetal growth restriction and oligohydramnios in the 26th week she delivered, by cesarean section, a healthy female infant weighing 730 g who survived. Patient 2 was treated from the seventh week of her second pregnancy with twice a week protein A immunoadsorption. The pregnancy proceeded normally until the 36th week, when, due to slight intrauterine growth restriction, she delivered a healthy baby girl weighing 2375 g by cesarean section. Anti-beta2 glycoprotein I antibody trends were similar during both types of treatment. On the basis of our findings obtained from only two cases it is impossible to define the best aphaeretic treatment of APS high risk pregnancies. Nevertheless, as a whole these data suggest better disease control using the immunoadsorption technique as compared to plasma exchange, despite their apparently similar anti-beta2 glycoprotein I antibody removal capabilities.
Ther Apher Dial 2009 Apr
PMID:Case reports of the use of immunoadsorption or plasma exchange in high-risk pregnancies of women with antiphospholipid syndrome. 1937 56


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