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PubMed2005

Assessment of patients with systemic lupus erythematosus and the use of lupus disease activity indices.

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.

Disease Progression↗
PubMed1987

Myelopathy in neonatal and infantile lupus erythematosus.

Two children with lupus erythematosus and myelopathy were studied. The first child developed the neonatal lupus erythematosus syndrome associated with transplacentally acquired anti-Ro/SSA antibodies. The cutaneous manifestations of neonatal lupus erythematosus disappeared but a residual myelopathy was confirmed at 16 months of age. The second child developed cutaneous lupus erythematosus at 3 months of age associated with a total deficiency of the Clr component of complement. A myelopathy and mesangial glomerulonephritis developed at 2 years of age which required treatment with corticosteroids. These two children with CNS lupus erythematosus, one associated with transplacentally acquired antibodies and the other associated with a complement deficiency, may suggest an immune-mediated mechanism for the pathogenesis of myelopathy in childhood lupus erythematosus.

Antibodies, Antinuclear↗
PubMed1992

Anti-Ro/SS-A and anti-La/SS-B autoantibody levels in relation to systemic lupus erythematosus disease activity and congenital heart block. A longitudinal study comprising two consecutive pregnancies in a patient with systemic lupus erythematosus.

OBJECTIVE: To analyze the anti-Ro/SS-A and anti-La/SS-B autoantibody responses, both quantitatively and qualitatively, in relation to the occurrence of congenital heart block (CHB) and disease activity in a patient with systemic lupus erythematosus. METHODS: The patient's course was carefully documented for a period of 80 months. Specific enzyme-linked immunosorbent assays, a HeLa cell immunoblotting test, and an RNA precipitation assay were used to determine autoantibody levels and fine specificities. RESULTS: Anti-Ro/SS-A and anti-La/SS-B antibody did not disappear. Levels of anti-Ro/SS-A appeared to fluctuate more frequently than levels of anti-La/SS-B. Fluctuations were unrelated to disease activity or immunosuppressive treatment. There was no characteristic relationship between levels of these antibodies and the occurrence of CHB. CONCLUSION: The occurrence of CHB cannot be predicted by frequent measurement of anti-Ro/SS-A and anti-La/SS-B levels. The immunoregulatory control of anti-Ro/SS-A and anti-La/SS-B antibodies differs from that of anti-DNA and anti-tetanus toxoid antibodies.

Adult↗
PubMed2001

The prevalence of celiac disease autoantibodies in patients with systemic lupus erythematosus.

OBJECTIVE: [corrected] Systemic lupus erythematosus has been associated with false positive autoantibodies for primary biliary cirrhosis, chronic active hepatitis, Sjogren's syndrome, rheumatoid arthritis, thyroid disorders, syphilis, and scleroderma. An increased prevalence of autoantibodies are found in celiac disease and systemic lupus erythematosus, which share the human lymphocyte HLA-B8 and HLA-DR3 histocompatibility antigens. This study examines the prevalence of celiac disease autoantibodies in systemic lupus erythematosus patients. METHODS: Patients observed in the Department of Rheumatology at our institutions in San Antonio, Texas with known systemic lupus erythematosus were offered participation in the study. One hundred three of the 130 patients contacted agreed to participate. Patients were excluded if they were pregnant or medically unable to undergo endoscopy. All volunteers were tested for the serological presence of IgA and IgM antigliadin and IgA antiendomysial antibodies. Those with positive serology underwent esophagogastroduodenoscopy with duodenal mucosal biopsy. RESULTS: Twenty-four of 103 (23.3%) systemic lupus erythematosus patients tested positive for either antigliadin antibody, whereas none of the 103 patients tested positive for antiendomysial antibody. None of the 24 antigliadin positive patients were found to have endoscopic or histological evidence of celiac disease, making the false positive rate of antigliadin antibody 23%. CONCLUSION: The presence of false positive antigliadin antibodies in patients with systemic lupus erythematosus is common. Despite shared human lymphocyte antigen loci there does not seem to be an association between celiac disease and systemic lupus erythematosus.

Autoantibodies↗
PubMed1999

Immunological and clinical differences between juvenile and adult onset of systemic lupus erythematosus.

INTRODUCTION: Systemic lupus erythematosus (SLE) in children usually follows a more severe course than in adults, but sometimes in the previous studies reported there are many confounding factors. OBJECTIVE: To analyse the immunological and clinical characteristics of SLE juvenile onset and SLE adult onset. METHODS: We studied 179 patients with SLE, 49 patients were aged 6-18 yrs at onset of disease. Anti-dsDNA antibodies were detected by radioimmunoassay and antibodies to extractable nuclear antigens (ENA): anti-nRNP, anti-Sm, anti-Ro/SS-A and anti-La/SS-B antibodies by ELISA, counterimmuno-electrophoresis and immunoblotting. RESULTS: Juvenile-onset SLE shows a higher frequency of cutaneous vasculitis (44.8% vs 27.6%; P < 0.05), seizures (18.3% vs 7.6%; P < 0.05) nephropathy (67.3% vs 48.4%; P < 0.025), and discoid lupus erythematosus (26.5% vs 13.8%; P < 0.05). The incidence of articular manifestations is lower than in adults (85.7% vs 96.1%; P < 0.025). No significant differences were found between the two groups in relation with the prevalence of antinuclear antibodies. CONCLUSIONS: Juvenile-onset SLE has more frequent neurological and renal manifestations than adult-onset SLE, but immunological markers are similar in both groups. These features suggest the most severe clinical manifestations in the juvenile-onset SLE group are not related with the presence of studied antibodies by different methods.

Adolescent↗
PubMed1999

[Endothelial response for the presence of chosen antinuclear antibodies, anti-Ro (SS-A) and anti-La (SS-B) and anti-Sm in vasculitis against the background of existing lupus erythematosus].

Systemic lupus erythematodes is a disease commonly associated with peripheric circulation disturbances. The goal of the study was the evaluation of the role of the following nuclear antibodies: anti-Ro (SS-A), anti-La (SS-B) and anti-Sm in the endothelium damage process in the SLE patients with symptoms of microcirculation disturbances. The concentration of plasma thrombomodulin was used as a marker of the endothelium destructive changes intensity. Twenty-four patients with SLE (22 women and 2 man) aged of 18-57 (the average age 40.50 +/- 9.72 years) in which occurred: the Raynauds symptom (16 patients), fingers cyanosis (5 patients) and fingers and/or toes necrosis (3 patients) were investigated. Antinuclear antibodies and thrombomodulin level was estimated with the ELISA method. In all patients, the concentration of anticardiolipin antibodies in serum was determined (ELISA-method, cardiolipin was used as an antigenes, Sigma USA). Higher titers of ANA antibodies in 83%, anti-SM antibodies in 30%, anti-Ro in 42% and anti-La in 42%, examined patients and higher titer of anti-Ro and anti-La antibodies together in 29% of examined patients were stated. Statistical significant correlation between the increased level of thrombomodulin and anti-La antibodies concentrations in serum of patients with the disease lasting over 6 years was found out. The significant statistical correlation of thrombomodulin concentration increasing and anti-La antibodies in serum was proved. The dynamism of limbs circulation disorders depends on the disease duration.

Adolescent↗
PubMed2006

[Thalidomide therapy for discoid lupus erythematosus].

INTRODUCTION: Discoid lupus erythematosus (DLE) is a chronic form of cutaneous lupus. Thalidomide has been reported as an effective treatment in at least 200 cases of severe DLE. In this article we studied the efficacy and adverse effects of thalidomide in 10 patients with disseminated DLE. METHODS: This report presents the retrospective evaluation of 10 patients treated with thalidomide in our department during the period 1996-2005. RESULTS: All patients treated with thalidomide had been unresponsive to at least two other systemic treatments. The patients were treated with low-dose thalidomide of 50-200 mg per day. The dose was tapered after a clinical remission. All patients had a favorable response within 6 weeks. Of 10 patients 9 had complete regression of lesions. Four patients remain free of disease without maintenance treatment and 5 patients relapsed after cessation of thalidomide. All relapses responded after the reinstitution of the drug. The side effects of thalidomide treatment were mostly transient. The most common adverse effects were sedation and weight gain. Two patients developed peripheral sensory neuropathy resolved with discontinuation of thalidomide. Two patients of premenopausal age reported amenorrhea. CONCLUSION: Low-dose thalidomide treatment is an effective treatment option for discoid lupus erythematosus in cases resistant to other treatments. It is essential to be conversant with all the precautions in order to minimize the risks of side effects.

Amenorrhea↗
PubMed1997

[Subacute cutaneous lupus erythematosus].

Subacute cutaneous lupus erythematosus (SCLE) is recognized as a variant of Systemic lupus erythematosus (SLE) and affects mainly middle-aged Caucasian women. The clinical picture is characterized by typical skin lesions, joint or muscle pain and occasionally arthritis. Serious systemic manifestations such as affection of the central nervous system and kidneys are rarely observed. Most SCLE patients are strongly photosensitive, which is associated with the presence of anti-SSA and/or anti-SSB antibodies. The disease can mimic both Sjögren's syndrome and SLE. The course is characterized by exacerbations and remissions. SCLE sometimes occurs concomitantly with other diseases of both a rheumatological or non-rheumatological nature, and it has also been discussed whether SCLE might be an example of a paraneoplastic syndrome. Treatment with hydroxychloroquine is usually effective. Five patients with SCLE are described.

Adult↗
PubMed1999

Guidelines for referral and management of systemic lupus erythematosus in adults. American College of Rheumatology Ad Hoc Committee on Systemic Lupus Erythematosus Guidelines.

SLE is a complex disorder with variable presentations, course, and prognosis. Since its prevalence is only 1/1,000, most primary care physicians and general internists will not have sufficient experience in the management of moderate-to-severe life-threatening disease. The major tasks of the primary care physician in the diagnosis and management of patients with SLE include early diagnosis, appropriate referral, monitoring patients with mild, stable disease, and collaboration with a specialist in the management of severe disease. Guidelines for the initial evaluation, reasons for referral, and management of mild and severe SLE are provided.

Adult↗
PubMed1987

Abnormal responses to ingested substances in murine systemic lupus erythematosus: apparent effect of a casein-free diet on the development of systemic lupus erythematosus in NZB/W mice.

To assess the development of oral tolerance to casein in NZB/W female mice, they must be bred and raised on a casein free diet. We examined the specific immune responses of the mice to the long term experimental feeding of casein. Twelve of fifteen casein free mice were still alive at 10 months of age, although by this age only 1/10 mice eating the normal diet was still alive. The casein free mice had markedly less anti-DNA antibody, their IgM to IgG antinative DNA switch was delayed and deposits of immunoreactants in the glomeruli were greatly decreased. The reason for this apparent effect of the removal of casein from the diet is unknown; however, immunostimulatory and endorphin-like regions have recently been reported in casein.

Animals↗
PubMed2001

A subset of lupus anti-DNA antibodies cross-reacts with the NR2 glutamate receptor in systemic lupus erythematosus.

In systemic lupus erythematosus, antibodies against double-stranded DNA are a major contributor to renal disease. We have previously demonstrated that the pentapeptide Asp/Glu-Trp-Asp/Glu-Tyr-Ser/Gly is a molecular mimic of double-stranded DNA. This sequence is also present in the extracellular domain of murine and human NMDA (N-methyl-D-aspartate) receptor subunits NR2a and NR2b. Here we show that the NR2 receptor is recognized by both murine and human anti-DNA antibodies. Moreover, anti-DNA antibodies with this cross-reactivity mediate apoptotic death of neurons in vivo and in vitro. Finally, we show that the cerebrospinal fluid of a patient with systemic lupus erythematosus contains these antibodies and also mediates neuronal death via an apoptotic pathway. These observations indicate that lupus antibodies cross-react with DNA and NMDA receptors, gain access to cerebrospinal fluid and may mediate non-thrombotic and non-vasculitic abnormalities of the central nervous system.

Animals↗
PubMed1995

Abnormalities of lymphocyte subsets in canine systemic lupus erythematosus.

Canine systemic lupus erythematosus (SLE) is a disease clinically very similar to its human counterpart. But so far, no study has reported an accurate evaluation of the lymphocyte subsets in the canine disease. Here, we present a study in which lymphocyte subsets have been evaluated in the peripheral blood of 20 dogs suffering from spontaneous systemic lupus erythematosus (SLE) in active and inactive phases, before and during treatment with prednisone and levamisole. 22 healthy dogs have been used as a control population. We show that canine SLE in active phases is associated with a several lymphopenia (1050 +/- 520 10(6) cells/l versus 2130 +/- 1 020 10(6) cells/l in controls). A striking finding is the imbalance of the CD4 and CD8 subsets (respectively 56.7 +/- 10.7% and 10.9 +/- 3.8% of CD4+ and CD8+ lymphocytes versus 40.5 +/- 11.5% and 18 +/- 4.4% in controls) and a strong activation of T-cells in active phases (64.1 +/- 16.9% of 2B3+ cells versus 46.5 +/- 16.7%). Moreover, we observed a persistence of the T subset imbalance during spontaneous evolution. In contrast, the treatment induced in dogs showing a good response the correction of CD4/CD8 ratio and no clinical manifestations, whereas in low responders no such improvements were observed. Thus, this work suggests that the main immunological imbalance seen in SLE could be associated with defective suppressor cells and provides further evidence of similarity of human and dog SLE.

Animals↗
PubMed2005

ITP as an initial manifestation of subacute cutaneous lupus erythematosus.

Subacute cutaneous lupus erythematosus (SCLE) is an entity characterized by widespread polycyclic lesions that heal without scarring. Skin lesions with marked ultraviolet sensitivity are distributed in an annular and/or psoriasiform configuration. Idiopathic thrombocytopenic purpura, which is an autoimmune disease (ITP), is mediated by a destructive immunoglobulin G antibody response to the platelets' membrane components. We report a case of subacute cutaneous lupus erythematosus initially manifested as thrombocytopenia, which was diagnosed as idiopathic thrombocytopenic purpura (ITP) and treated with splenectomy. Seven months later, development of cutaneous involvement followed the diagnosis of ITP. The clinical and histological features of the lesions were compatible with SCLE. Serological evaluations showed a negative anti-nuclear antibody test and an elevated anti-SSB/La antibody level. Symptoms for systemic involvement were negative. Although the clinical features such as photosensitivity, discoid rash, and thrombocytopenia were in favor of SLE, the patient did not fulfill the criteria of the American Rheumatism Association (ARA) for SLE.

Adult↗
PubMed2004

Toxic epidermal necrolysis-like acute cutaneous lupus erythematosus and the spectrum of the acute syndrome of apoptotic pan-epidermolysis (ASAP): a case report, concept review and proposal for new classification of lupus erythematosus vesiculobullous skin lesions.

The acute clinical syndrome of toxic epidermal necrolysis (TEN) is currently thought to be a distinct clinical-pathological entity typically resulting from drug hypersensitivity. We describe an adult woman who experienced a fulminate pattern of apoptotic epidermal cell injury following tanning bed exposure while taking naproxen that resulted in a clinical presentation having combined features of drug-induced TEN and an infrequently recognized form of bullous cutaneous lupus erythematosus (LE). This case calls attention to the fact that TEN-like injury can occasionally be seen in settings other than drug hypersensitivity (e.g., LE, acute graft versus host disease) and illustrates the need for a unifying concept in this area. We therefore propose the term 'Acute Syndrome of Apoptotic Pan-Epidermolysis (ASAP)' to designate a clinical syndrome that is characterized by life-threatening acute and massive cleavage of the epidermis resulting from hyperacute apoptotic injury of the epidermis. We also review vesiculobullous skin disorders that can be encountered in LE patients and suggest a new classification scheme for such lesions.

Acute Disease↗