Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The coexistence of organ-specific and nonorgan-specific autoimmune diseases is an interesting phenomenon. A 52-year-old woman was admitted with fever, general discomfort, polyarthritis, and Raynaud's phenomenon. Physical examination revealed a goiter of stony consistency, hardening, paleness, and atrophy of the skin on the face and upper limbs, and blood hypertension (180/110 mmHg). The biological data included leukopenia, moderate anemia, and a very high sedimentation rate. The latex test was positive (+++); LE cells positive (+); hypergammaglobulinemia (3.5 g); antinuclear antibodies, 1/1280 with an immunofluorescence granular pattern; antithyroid antibodies, 1/160. There was pulmonary, renal, and gastrointestinal involvement compatible with scleroderma, which was confirmed by skin biopsy. A thyroidectomy revealed the existence of a papillary carcinoma with thyroiditis. Responde to treatment with immunosuppressive agents, hypotensive drugs, and thyroid substitution therapy was initially good. The patient was readmitted 8 months later with general discomfort and a severe hyperproteinemia (10 g/100 ml), including 65 percent gammaglobulin and requiring various sessions of plasmapheresis. The patient was discharged, but died suddenly 4 months later. The association of lupus and scleroderma in this patient is discussed and the possibility of its being a mixed connective tissue disease is discarded. The association of this condition with Hashimoto's thyroiditis, and the latter with papillary carcinoma of the thyroid are analyzed. The peculiar features of this case are pointed out. The authors postulate that the cause of the sudden death was a vascular cerebral complication induced by the extreme hyperproteinemia.
 ...
PMID:[Scleroderma with traces of disseminated lupus erythematosus associated with Hashimoto's thyroiditis and papillary carcinoma of the thyroid gland (author's transl)]. 58 90

More than 70 medications have been implicated as a cause of drug-induced lupus. The condition most commonly manifests with arthralgias and fever. The most important laboratory feature is an elevated antinuclear antibody titer, which often corresponds to the presence of antihistone antibodies. Fortunately, the course of drug-induced lupus is more benign than that of idiopathic systemic lupus erythematosus. Clinical resolution generally occurs after withdrawal of the offending agent. The patient described here exemplifies the importance of maintaining a heightened awareness of this uncommon disorder. For him, delayed diagnosis of drug-induced lupus resulted in prolonged discomfort, restriction of daily activities, and unnecessary therapeutic intervention.
 ...
PMID:Drug-induced lupus. The list of culprits grows. 879 60

A 42-year-old multiparous pregnant woman presented with swelling and pain of the left arm at 34 weeks gestation. She had no discoloration of her arm nor a loss of radial pulse. Duplex scanning demonstrated a thrombosis in the axillary vein. She was found to have a positive circulating lupus anticoagulant. Intravenous heparin was administered and resulted in resolution of discomfort and swelling on day four of therapy. The patient was maintained on therapeutic doses of subcutaneous heparin until vaginal delivery at 39 weeks. Prenatal course was complicated by a resolving infection believed to be due to cat-scratch disease which produced a five centimeter cystic lesion in the left axillae which was removed in the first trimester. Titers for cat-scratch disease were positive for mother and infant at delivery but infant titers were negative at six weeks. Axillary vein thrombosis in pregnancy can be complicated by pulmonary embolism and should be treated by heparin.
 ...
PMID:Axillary vein thrombosis during pregnancy in association with a lupus anticoagulant. 958 9

Infliximab, a chimeric monoclonal antibody targeting tumor necrosis factor alpha (TNF-alpha), is efficacious in the treatment of rheumatoid arthritis and Crohn's disease. We report in detail an unusual adverse reaction to infliximab therapy, a drug-induced lupus-like clinical syndrome. A 45-year-old woman with steroid-dependent Crohn's colitis, successfully managed with maintenance infliximab infusions and methotrexate, developed a lupus-like syndrome eight months after her initial infusion. This was characterized by inflammatory arthritis and an urticarial and papulosquamous rash and was accompanied by high titers of antinuclear, double-stranded DNA, glomerular-binding, and histone antibodies and by reduced levels of the C4 component of complement. After discontinuance of infliximab infusions and treatment of symptoms with intermittent courses of prednisone, the patient's arthritis progressively improved, with accompanying decrements in autoantibody titers. One year later, she has minimal joint discomfort and no rash or gastrointestinal symptoms despite also discontinuing prednisone and methotrexate. Infliximab therapy may cause a lupus-like syndrome that is reversible upon discontinuing this agent. These findings support recent evidence identifying TNF-alpha as an inhibitor of autoantibody formation.
 ...
PMID:A lupus-like syndrome associated with infliximab therapy. 1279 23

Headache is a common feature in patients with systemic lupus erythematosus (SLE) and represents a significant source of patient discomfort. The exact prevalence of headache in SLE is unknown. The results of different studies widely vary, most likely due to the use of different classification for headache and the lack of controls in most studies. The relationship between headache and SLE is also unclear since it is difficult to determine which degree and type of headaches can be explained on the basis of chronic illness, or as part of the disease spectrum of SLE. No pathogenic mechanism has so far been described that can fully explain headache induced by SLE. The role of circulating cytokines, vascular injury, neuronal damage or antiphospholipid antibodies (aPL) in the development of headache in SLE patients is also a matter of debate. Other concomitant causes such as infection or hypertension should be excluded before assuming that headache is a feature of SLE activity. Therapeutic approach of headache SLE-related remains empirical and based on clinical experience.
Lupus 2003
PMID:Headache and systemic lupus erythematosus. 1471 15

Dobutamine stress echocardiography (DSE) is an accurate noninvasive test used for the diagnosis and evaluation of patients with known or suspected coronary artery disease (CAD). The aim of this study was to determine the rate of positive findings in DSE, to define the echocardiographic and clinical characteristics of women with systemic lupus erythematosus (SLE) and to evaluate the safety of DSE in SLE patients. Thirty consecutive SLE patients were enrolled in the study and underwent DSE study. The mean age of patients was 44 years (range 20-76). Mean duration of SLE was 8.1 years and mean SLEDAI was 5.5. None of the DSE tests performed were positive for myocardial ischaemia. A left ventricular outflow gradient (LVOG) was found in 15/28 (54%) patients who completed the test, a result higher than the reported 20% prevalence of this finding in the literature. There were no significant differences in baseline characteristics between patients who developed a gradient and patients in whom a gradient was not found. There were no significant adverse effects during the study. In the general population, LVOG has been reported to be associated with an increased rate of chest discomfort and with a significantly lower prevalence of CAD. Whether this is true for SLE patients requires further study.
Lupus 2004
PMID:Dobutamine stress echocardiography in women with systemic lupus erythematosus: increased occurrence of left ventricular outflow gradient. 1499 2

The anti-TNF-alpha antibody infliximab (Remicade) is highly effective in the treatment of Crohn's disease. A subset of patients experience allergic reactions as a result of antibodies to infliximab (ATIs). The purpose of the current study is to describe the safety and efficacy of adalimumab (Humira) in patients previously allergic or intolerant to infliximab. Adalimumab is an anti-TNF-alpha agent containing only human peptide sequences. Seven patients have been treated with adalimumab who had experienced immediate- or delayed-hypersensitivity reactions to infliximab and one with infliximab-induced lupus. Except for injection site discomfort, adalimumab was well tolerated without signs or symptoms of allergic reactions. One patient who had previously received pooled human immunoglobulin developed a pruritic rash after each dose of adalimumab. Patients with active disease who had previously experienced a robust response to infliximab responded to adalimumab as reflected by an improvement in Harvey-Bradshaw index and inflammatory markers. Based on these preliminary data, adalimumab may be a safe and effective substitute for infliximab-allergic patients. Individuals who have been exposed to human antibodies may be sensitized to other human antibodies such as adalimumab.
 ...
PMID:A pilot study of adalimumab in infliximab-allergic patients. 1547 39

Antiphospholipid syndrome is an autoimmune disease characterized pathophysiologically by the presence of antiphospholipid antibodies and > or =1 clinical manifestation, the most common being venous or arterial thrombosis. We describe the case of a 40-year-old male with unexplained severe pulmonary arterial hypertension with a seven-day history of progressive shortness of breath, hemoptysis, chest discomfort and bilateral pedal edema. Electrocardiographic, echocardiographic and imaging studies showed changes consistent with chronic thromboembolic pulmonary hypertension. Further work-up showed positive anticardiolipin antibodies and lupus anticoagulant with negative features for lupus with negative primary thrombophilic studies as well. The patient was managed adequately with oral anticoagulation with improvement of his clinical status and referred to a tertiary care center to be screened for pulmonary thromboendarterectomy. For patients meeting surgical selection criteria, pulmonary thromboendarterectomy has demonstrated positive outcomes with respect to survival, functionality and quality of life. We discuss the pathophysiology and treatment as well as novel therapies in nonsurgical candidates with chronic thromboembolic pulmonary hypertension in the setting of primary antiphospholipid syndrome.
 ...
PMID:Chronic thromboembolic pulmonary hypertension as an uncommon presentation of primary antiphospholipid syndrome. 1859 78

Chronic thromboembolic pulmonary hypertension (CTEPH) caused by intraluminal thrombus organization and fibrous stenosis or complete obliteration of pulmonary arteries, is a not rare but life-threatening complication of acute pulmonary embolism. The prognosis of medically treated patients with CTEPH is poor and worsens as pulmonary hypertension exacerbates. We describe the case of a 43-years old with a history of progressive shortness of breath, hemoptysis, chest discomfort and syncope. Echocardiographic and imaging studies showed changes consistent with chronic thromboembolic pulmonary hypertension. Further work-up showed only moderate increase of homocysteine level with negative features for lupus and others primary thrombophilic disease. The patient was managed adequately with thrombolytic and inotropic therapy; oral anticoagulation was started with improvement of his clinical status and was screened for pulmonary thrombo endarterectomy, but he refused. The case presented despite its evolution 'temporarily' positive perhaps related to the reduction of hemodynamic overload through bronchial arteries, reiterates the importance of early surgical intervention, before it establishes the hypertensive vasculopathy. Abnormal pulmonary function at rest and after exercise stress test associated to non invasive echocardiographic measurements are an excellent tool to identify the bad prognosis patients in CTEPH. We discuss the pathophysiology and conclude that in selected cases, pulmonary thromboendarterectomy is the best therapy, but only if executed early.
 ...
PMID:Chronic thromboembolic pulmonary hypertension: take care to a "favourable" apparently evolution. A case report. 2206 57

The danger model was proposed by Polly Matzinger as complement to the traditional self-non-self- (SNS-) model to explain the immunoreactivity. The danger model proposes a central role of the tissular cells' discomfort as an element to prime the immune response processes in opposition to the traditional SNS-model where foreignness is a prerequisite. However recent insights in the proteomics of diverse tissular cells have revealed that under stressful conditions they have a significant potential to initiate, coordinate, and perpetuate autoimmune processes, in many cases, ruling over the adaptive immune response cells; this ruling potential can also be confirmed by observations in several genetically manipulated animal models. Here, we review the pathogenesis of rheumatic diseases such as systemic lupus erythematous, rheumatoid arthritis, spondyloarthritis including ankylosing spondylitis, psoriasis, and Crohn's disease and provide realistic approaches based on the logic of the danger model. We assume that tissular dysfunction is a prerequisite for chronic autoimmunity and propose two genetically conferred hypothetical roles for the tissular cells causing the disease: (A) the Impaired cell and (B) the paranoid cell. Both roles are not mutually exclusive. Some examples in human disease and in animal models are provided based on current evidence.
...
PMID:The danger model approach to the pathogenesis of the rheumatic diseases. 2597 36


1 2 Next >>