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)

In patients with rheumatic and, especially, connective tissue diseases many organ systems apart from the joints may be involved, such as the skin, muscles, salivary glands, nerves, kidneys and blood vessels. Biopsies of these tissues may help in establishing a diagnosis, in assessing the extent and severity of the disease and in monitoring the success of therapy. In this chapter, indications for biopsy, technical procedures, potential results and possible complications are described. The emphasis is on when a biopsy is indicated, how the patient is prepared, how the biopsy is performed, what results can be expected from the biopsy and what are the possible sequelae. For full details of the histology of e.g. a renal biopsy in a lupus patient, the reader is referred to the relevant textbooks.
Best Pract Res Clin Rheumatol 2005 Jun
PMID:Procedures related to connective tissue disease. 1593 67

In this short review, four different aspects of SLE are discussed. The ACR criteria for SLE were established for the differentiation of SLE from other autoimmune diseases and not for the direct diagnosis of SLE. Treatment of SLE is continuously re-evaluated thanks ongoing clinical research. Best clinical practice should be based on experienced and continuous knowledge in the field rather than on dogmatic treatment schemes. SLE is not one disease, but a series of clinical pictures associated into a syndrome. Finally, do not forget that patients with the SLE syndrome suffer almost always from debilitating fatigue.
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PMID:[Systemic lupus erythematosus--myths and dogma]. 1594 19

The assessment of disease activity in systemic lupus erythematosus (SLE) is a task faced by clinicians in every day care, but it is also required for clinical research and in randomised controlled trials. It is crucial to distinguish disease activity from infection, chronic damage and co-morbid disease. Over the past 20 years, many indices have been developed to objectively measure lupus disease activity and several of these have been validated. The most widely used indices are the British Isles Lupus Assessment Group (BILAG) index, the European Consensus Lupus Activity Measurement (ECLAM), the Systemic Lupus Activity Measure (SLAM), the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and the Lupus Activity Index (LAI). All these indices have been validated and have excellent reliability, validity and responsiveness to change. In addition to the assessment of disease activity, the evaluation of damage using the validated SLICC/ACR damage index and health-related quality of life is advised for clinical research.
Best Pract Res Clin Rheumatol 2005 Oct
PMID:Assessment of patients with systemic lupus erythematosus and the use of lupus disease activity indices. 1615 Mar 98

Although considered a prototypic autoimmune disease, the hallmark of systemic lupus erythematosus (SLE) is its heterogeneity. Accordingly, manifestations can vary widely from person to person, with the potential involvement of virtually any bodily organ. Furthermore, the genetic abnormalities underlying this condition are complicated, with diverse genetic polymorphisms described in different ethnic groups, strongly suggesting that the actual pathology underlying the immunologic disarray might not be the same for each patient. Evolving concepts of genetics and immunity have clarified that patients can carry unique arrays of exacerbating and protective factors. These factors, in conjunction with variable environmental triggers for SLE, probably determine the sequelae that an individual experiences. Therefore, it is not surprising that the clinical manifestations are diverse, the temporal sequence of organ involvement often unpredictable, and that the flares of inflammatory activity that characterize SLE can either remit without consequence or leave permanent damage in their wake. It is widely accepted that the current standard of care for SLE patients is inadequate. Programs to develop and test new drug and/or device therapies have been ongoing since the mid-1990s but have encountered formidable obstacles. With the current burst of drug discovery and the advent of several large international trials of promising new agents, the challenge to overcome these obstacles has never been greater. A burgeoning literature in the past decades nevertheless suggests that despite the complexities of the many immunologic pathways that impact on SLE, characteristic biologic markers are emerging as potential signposts that can characterize patient subgroups, predict prognosis, mark the exacerbations and remissions of SLE flares, and serve as endpoints in the determination of the dosing and timing of immune-modulating treatments. Several of the promising biomarkers are addressed in this chapter.
Best Pract Res Clin Rheumatol 2005 Oct
PMID:The role of biomarkers in the assessment of lupus. 1615 Mar 99

The diagnosis of neuropsychiatric systemic lupus erythematosus (NPSLE) is complex not only on account of the heterogeneous nature of neurological presentation but also because of the difficulty of differentiating lupus-related pathology from other neuropsychiatric diseases. Magnetic resonance imaging (MRI) remains the gold standard for the non-invasive assessment of NPSLE but there are problems, both with sensitivity and specificity. Both T(2) quantitation and the use of gadolinium have shown promise in differentiating acute from chronic lesions. Nonetheless, the lack of sensitivity of conventional MRI has led to the exploration of other MR-based techniques. Magnetic resonance spectroscopy (MRS) allows the measurement of brain metabolites, whereas diffusion weighted imaging and diffusion tensor imaging allow assessment of white matter structure and integrity. MRS studies in NPSLE have consistently shown a reduction in N-acetyl aspartate (a neuronal marker). Diffusion weighted imaging has had only limited application in lupus and the results to date have shown abnormal diffusivity in lupus patients consistent with inflammation and loss of white matter structure. These techniques remain research tools at this early stage. Positron emission tomography (PET) and single photon emission computed tomography (SPECT) have also been explored as functional imaging tools in lupus and both appear to be more sensitive in detecting subtle brain changes in NPSLE but there are issues with specificity which deter their use in the clinical setting.
Best Pract Res Clin Rheumatol 2005 Oct
PMID:Imaging in CNS lupus. 1615 Apr

Gastrointestinal (GI) manifestations of systemic lupus erythematosus (SLE) are protean. Any part of the GI tract and the hepatobiliary system can be involved. Up to two-third of SLE patients develop GI symptoms at some stage of their illnesses. Clinical presentations of GI lupus are non-specific and can be difficult to differentiate from infective, thrombotic, therapy-related and non-SLE etiologies. Clinical acumen and appropriate endoscopic, biopsy and imaging procedures are essential for establishing the correct diagnosis. Acute abdominal pain in SLE patients can herald an intra-abdominal catastrophe and should be evaluated promptly. Surgical intervention should be instituted without delay if conservative management fails or when there is clinical or radiological suspicion of visceral perforation or intra-abdominal collections.
Best Pract Res Clin Rheumatol 2005 Oct
PMID:Investigations and management of gastrointestinal and hepatic manifestations of systemic lupus erythematosus. 1615 Apr 1

Lupus nephritis remains a strong predictor for death and the development of end-stage renal disease (ESRD) in patients with SLE. Definition of renal involvement varies but overt renal disease is found in at least one-third of SLE patients, with up to 60% of adults and 80% of children developing lupus nephritis. Clinical presentation has been found to bear little relationship to renal biopsy findings. Renal biopsy is therefore informative for all patients with SLE with abnormal urinalysis or reduced renal function, even with serum creatinine in the normal range, to guide treatment decisions. Current treatment regimens combine corticosteroids with cyclophosphamide, azathioprine or ciclosporin, although mycophenolate mofetil has received much recent attention as a potentially superior immune suppressive. The toxicity of current drug regimens contributes significantly to existing morbidity and mortality. A new era of biological therapies holds the potential for safer, more effective therapies in the future.
Best Pract Res Clin Rheumatol 2005 Oct
PMID:Diagnosis and treatment of kidney disease. 1615 Apr 3

Nervous system disease in systemic lupus erythematosus (SLE) is manifested by a wide variety of clinical manifestations. Despite the development of a universal classification for neuropsychiatric (NP) lupus in 1999, there continues to be considerable variability in the reported prevalence of NP syndromes between different lupus cohorts. Due to the lack of specificity of individual NP manifestations, non-SLE causes such as complications of therapy and co-morbidities must be considered in advance of attributing the event to one or more primary immunopathogenic mechanisms. These include intracranial microangiopathy, autoantibodies to neuronal and non-neuronal antigens, and the generation of proinflammatory cytokines and mediators. The diagnosis of NP-SLE remains largely one of exclusion and is approached in individual patients by thorough clinical evaluation, supported when necessary by autoantibody profiles, diagnostic imaging, electrophysiologic studies and objective assessment of cognitive performance. Given the diversity in clinical manifestations, the management is tailored to the specific needs of individual patients. In the absence of controlled studies, the use of symptomatic therapies, immunosuppressives, anticoagulants and non-pharmacologic interventions is supported by case series and clinical experience.
Best Pract Res Clin Rheumatol 2005 Oct
PMID:Management of neuropsychiatric lupus. 1615 Apr 4

Premature coronary heart disease (CHD) is a major cause of morbidity and mortality in patients with systemic lupus erythematosus (SLE). In certain age groups, the risk can be >50 times that of an age-matched population. This population also has an increased prevalence of several key classic risk factors that contribute to the CHD development. Chronic inflammation, anti-phospholipid antibodies and exposure to steroid therapy are also likely to have an impact. We have adopted a proactive approach to classic risk factor management with 'ideal targets' based on viewing SLE as a CHD equivalent condition. In this context, a significant proportion of SLE patients (approximately 30%) will require statins and the majority would be treated with aspirin prophylaxis. Better control of the underlying inflammatory disease is also likely to play an important role and the relative safety of anti-malarials allows their consideration as an adjunct in a large proportion of patients. Well-conducted clinical trials are now needed to advance beyond these initial recommendations.
Best Pract Res Clin Rheumatol 2005 Oct
PMID:Cardiovascular disease in lupus patients: should all patients be treated with statins and aspirin? 1615 Apr 5

High-dose immunosuppression followed by autologous hematopoietic stem cell therapy (HSCT) has the promise of long-term response after a short, intense period of immunosuppressive therapy but it is associated with an increased risk of serious short-term complications. HSCT induces major clinical responses in about 65% of patients with SLE who failed standard therapies. In some of these patients such responses are durable for at least several years, but the curative potential of this procedure in severe SLE is still unknown. Procedure-related mortality varies among studies between 5 and 12% and seems to be lower in relatively larger single center studies. Until more reliable estimates of the actual risks and long-term outcomes become available, patients with potentially life-threatening or disabling major organ involvement who are in acceptable general medical condition should be considered for autologous HSCT if they have failed a reasonable course of standard immunosuppressive therapy. To accomplish the best therapeutic and scientific results, it is necessary to treat all patients in carefully planned protocols by specialized teams of lupus specialists and transplanters. All immunoablative protocols should incorporate carefully planned studies of immune reconstitution to understand the mechanisms of cure or failure.
Best Pract Res Clin Rheumatol 2005 Oct
PMID:The role of immune ablation and stem cell transplantation in severe SLE. 1615 Apr 6


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