[Precipitation reaction for diagnosis of disseminated lupus erythematosus].
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Humans↗
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[SYSTEMIC lupus erythematosus].
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Humans↗
Bullous Systemic Lupus Erythematosus as an Initial Manifestation of SLE.
Bullous systemic lupus erythematosus (BSLE) is a rare subset of systemic lupus erythematosus that is often associated with autoimmunity to type VII collagen. We describe a 45-year-old woman with BSLE who presented with vesiculobullous lesions as an initial manifestation of SLE. The patient first noticed a widespread urticarial, erythematous eruption associated with tense blisters, erosions, and crusting. She was diagnosed with bullous pemphigoid and underwent a one-month course of treatment with betamethazone. Because of the appearance of marked proteinuria, a subsequent renal biopsy, and serological tests, the patient was diagnosed with rapidly progressive glomerulonephritis and systemic lupus erythematosus. The patient's IgG circulating antibodies labeled the dermal floor of salt-split skin and recognized type VII collagen in immunoblot studies. Although methylprednisolone pulse therapy for glomerulonephritis did not alleviate the vesicullobullous eruption, treatment with dapsone resulted in dramatic disappearance of the lesions. Cessation of dapsone therapy due to hemolysis with Heinz-body formation did not aggravate the bullous disease. Our case illustrates that a generalized vesiculobullous eruption can be the sole presenting manifestation of SLE. It also emphasizes the close temporal relationship between BSLE and lupus nephritis.
Betamethasone↗
Heart disease in systemic lupus erythematosus: diagnosis and management.
Most patients suffering from systemic lupus erythematosus develop secondary heart disease at some time during the course of the primary illness. The most common forms of this type of heart disease are acute fibrinous pericarditis and hypertension. By means of echocardiography, an increased incidence of pericardial effusion has been demonstrated. Although commonly noted at autopsy, myocarditis is often clinically silent. However, endomyocardial biopsy may confirm its presence during life. Libman-Sacks endocarditis, although encountered in 40 to 50% of hearts at autopsy, is rarely diagnosed during life. When significant valve dysfunction such as aortic insufficiency or mitral regurgitation develops during the course of systemic lupus erythematosus, then Libman-Sacks endocarditis should be strongly suspected. Cardiac arrhythmias, first degree AV block, and acquired complete heart block may develop either de novo or in association with lupus pericarditis, myocarditis, vasculitis, etc. Complete congenital heart block has been reported in newborns of mothers with systemic lupus erythematosus, particularly those who have an antibody to a soluble tissue ribonucleoprotein antigen called RO(SS-A). Coronary arteritis and premature coronary atherosclerosis manifesting in either angina pectoris or myocardial infarction in young adults, particularly women suffering from systemic lupus erythematosus, have received attention recently. The development of hypertension and hyperlipidemia while such patients are receiving prolonged corticosteroid therapy has been incriminated as the significant risk factor in premature coronary atherosclerosis. Longstanding hypertension and congestive heart failure have unfavorable prognoses. This report is based on a cumulative review of 50 patients with acute and chronic systemic lupus erythematosus seen at our institution and in private practice during the last 10 years.
Journal Article↗
Mixed lichen planus-lupus erythematosus disease.
Two patients are reported with clinical symptoms of systemic lupus erythematosus associated with centrally ulcerated hypertrophic plaques and lip lesions. Histological findings from various sites were features of both lupus erythematosus and lichen planus. An increase in acid mucopolysaccharides and a thickened basement membrane which were present in some biopsies were interpreted as lupus erythematosus. The deposition of immunoglobulins and fibrinogen seemed to favor lupus erythematosus but these findings may also be seen in lichen planus. Four possible interpretations of the findings are discussed. The term "mixed lichen planus--lupus erythematosus disease" may be appropriate for these patients.
Basement Membrane↗
MHC class II-bound self peptides from autoimmune MRL/lpr mice reveal potential T cell epitopes for autoantibody production in murine systemic lupus erythematosus.
The systemic lupus erythematosus-like syndrome in MRL/lpr mice involves high-titered IgG autoantibodies, particularly antinuclear Abs that target histones, DNA, and RNA particles. Although T cell help is required for the generation of antinuclear Abs, the epitopes recognized by such helper T cells are unknown. To address this question, we isolated and sequenced self peptides bound by MHC class II molecules from MRL/lpr mice. We identified a number of peptides that are not seen in similar preparations from nonautoimmune C3H animals. The "abnormal" peptide donors include histone, a protein component of a small nuclear ribonucleoprotein, ribosomal proteins, and RNA processing enzymes. We postulate that the peptides from these donors are T cell epitopes required for the generation of the most frequent antinuclear Abs specificities seen in MRL/lpr mice.
Amino Acid Motifs↗
[Subacute cutaneous lupus erythematosus: clinical aspects, immunology and therapy].
Subacute cutaneous lupus erythematosus (SCLE) is a distinct subset of cutaneous lupus erythematosus which is defined by clinical and immunological characteristics. With regard to clinical expression and prognosis, SCLE assumes an intermediate position within the spectrum of LE between purely cutaneous discoid and systemic lupus erythematosus. The main clinical characteristics of SCLE are extensive papulosquamous or anular lesions and photosensitivity. The disease is frequently associated with Sjögren's syndrome and systemic symptoms, mainly arthralgia. Renal involvement is, however, rare. Circulating Ro-antibodies represent the main autoimmune phenomenon. An immunogenetic disposition to develop SCLE is evidenced by the detection of the HLA-B8, DR3-phenotype in a large proportion of patients. Drug therapy comprises primarily glucocorticosteroids and antimalarials, but retinoids have recently also proved highly effective.
Adolescent↗
Chilblain lupus erythematosus is associated with antibodies to SSA/Ro.
Chillblain Lupus Erythematosus (CL) of Hutchinson is a subtype of Lupus Erythematosus characterized by erythematous lesions symmetrically distributed on the face, nose, fingers and toes, knees and heels. The lesions are induced by cold, damp climates. A number of patients affected by CL eventually develop features of Systemic Lupus Erythematosus (SLE). We report here 7 patients, all but one affected by SLE, with chilblain cutaneous lesions on their hands, feet and face. The onset of CL preceded the diagnosis of SLE, from 1 to 10 years in 3 cases, it was concurrent in one case and was subsequent in the other 2 cases. Six out of the seven patients referred typical Raynaud's phenomenon and one had acrocyanosis. CL lesions developed and were aggravated by the cold during autumn and winter, they improved during summer. Skin biopsy performed in 5 patients from the lesions showed, on histology, a typical pattern of alterations with granular deposits at the dermo-epidermal junction on direct immunofluorescence. Laboratory findings showed: ANA and anti-SSA/Ro were detected in all the patients, anti-SSA/Ro were isolated in 4 patients and associated with anti-Sm in one case, anti-U1 RNP in one case and with anti-Sm and anti-RNP in a third case. Complement consumption was observed in 5 patients, anti-dsDNA in the six patients with SLE, hypergammaglobulinemia in 4 and rheumatoid factor in one. The fine specificity of anti-SSA/Ro as determined by immunoblotting using a human spleen extract as a substrate, showed: anti-60kD and anti-52 kD in two sera, anti-60kD isolated in 2 sera, anti-52kD isolated in one serum (from the patient without SLE) while 2 sera did not blotted. In conclusion, our study confirms the previous report of anti-SSA/Ro antibodies in association with CL. This clinical and serologic association widens the spectrum of cutaneous disease that is associated with antibodies to SSA/Ro to include conditions such as to SCLE, hypergammaglobulinemic purpura and neonatal lupus.
Adult↗
Effects of a self-management course for patients with systemic lupus erythematosus.
BACKGROUND: For people living with systemic lupus erythematosus, the disease's potential variety and severity of manifestations and unpredictable course present challenges and repercussions in all arenas of life. AIM: A quasi-experimental study was conducted to examine the effects of a systemic lupus erythematosus self-management course for Korean patients on fatigue, coping skills, self-efficacy, depression, pain and disease activity. METHODS: In a two-group pre- and post-test design, a total of 41 participants were assigned to the experimental group (21 participants) and to the control group (20 participants). The experimental group received six weekly 2-hour sessions for groups of 10-15 literate adults of all ages, while the control group did not receive any intervention. Outcome measures included fatigue, coping skills, self-efficacy, depression, pain and disease activity. RESULTS: Patients who participated in the self-management course showed significant improvement in fatigue (P = 0.049), coping skills (P = 0.007), self-efficacy (P = 0.001), and depression (P = 0.025). There were no significant changes in pain and disease activity after the intervention. CONCLUSIONS: The systemic lupus erythematosus self-management course had effects in reducing fatigue and depression and improving coping skills and self-efficacy. This course is potentially a good nursing intervention that can be offered in community settings.
Adaptation, Psychological↗
T-cell subsets in lesions of systemic and discoid lupus erythematosus.
In 6 patients with untreated systemic lupus erythematosus (SLE) in the progressive stage, and in 6 with discoid lupus erythematosus (DLE), an analysis of inflammatory infiltrates was performed in situ using the avidin-biotin-peroxidase complex (ABC) method with monoclonal antibodies. In all patients, over 75% of the infiltrates reacted with the pan T-cell antibody OKT3, but only sporadically with that of B-cell OKB7. In addition, a large number of the infiltrates were OKIal-positive, indicating that they were in an activated state. Many OKT8-positive cells were seen infiltrating the epidermis especially in the vicinity of basal keratinocytes. Staining for T-cell subsets revealed that the proportion of OKT8-positive cells (suppressor/cytotoxic) was from 2 to 3 fold higher than that of OKT4-positive cells (helper/inducer) in lesions of SLE. On the contrary, in DLE, a predominance of OKT4-positive cells (the OKT4/OKT8 ratio was from 1:1 to 3:1) was observed. Thus, our results provide further evidence that these 2 main types of LE show quite contrary findings on immunohistochemical analysis of T-cell subsets, and that besides the humoral immune mechanism, the cell-mediated immune mechanism may be involved in the pathogenesis of these disorders.
Antibodies, Monoclonal↗
Skin lesions--an indicator of disease activity in systemic lupus erythematosus?
Cutaneous manifestations have great diagnostic value for systemic lupus erythematosus (SLE). In this study we tried to establish a correlation between lupus erythematosus LE-specific and LE-nonspecific cutaneous lesions and disease activity measured by the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). Sixty-six patients with SLE were evaluated. They were divided into three groups having: (1) only LE-specific lesions (38 or 58.46%); (2) only LE-nonspecific lesions (4 or 6.15%); and (3) both types of lesions (23 or 35.38%). Results were analyzed using the Student t-test. Patients with LE-nonspecific skin manifestations had significantly increased disease activity compared to those with only LE-specific lesions. The number of different skin lesion types also correlated with disease activity. It was significantly increased in a group with three different types of lesion, either specific or nonspecific. Patients with only one type of lesion had mild disease. An intermediate disease activity was found in the group with two different lesion types. Lupus-specific skin manifestations serve primarily as an important diagnostic clue. In conclusion, patients with LE-nonspecific lesions have significantly more active SLE than those with LE-specific lesions and may therefore require more intensive therapy and disease monitoring.
Adult↗
[Subacute cutaneous lupus erythematosus based on a study of 15 cases].
15 cases of subacute cutaneous lupus erythematosus are reported. The diagnosis was based on the presence of the typical clinical features, on the histologic and immunpathologic examination of lesional skin and on the characteristic laboratory findings. 8 patients had annular type, 4 patients had papulosquamosus type of the characteristic skin signs of subacute cutaneous lupus erythematosus. In 3 patients both types of lesions existed simultaneously. 5 patients fulfilled the American Rheumatism Association criteria for systemic lupus erythematosus, however the systemic symptoms (arthritis, arthralgia, fever, myalgia, photosensitivity) were mild. 4 patients had positive ANA test, anti-Ro/SSA antibodies were determined in 5 patients, anti-RNP antibodies were detected in 8 patients. Anti-dsDNA antibodies were not detected. Subacute cutaneous lupus erythematosus is an intermediate subset in severity between discoid lupus erythematosus and severe systemic lupus erythematosus, therefore a milder form of therapy should be chosen.
Adult↗
[Porphyria cutanea tarda and lupus erythematosus].
We observed three patients with lupus erythematosus and porphyria cutanea tarda manifesting itself simultaneously or subsequently. The possible coincidence of the two diseases might be the consequence of immunological communities indicated on the assumption of a genetically coined hepatic uroporphyrinogen decarboxylase defect. The treatment of the lupus erythematosus can be performed according to corresponding observations of the course according to general acknowledged principles including the administration of cytostatic drugs, if the chloroquine phosphate therapy which is clearing up the porphyria cutanea tarda is beginning with low dosages. The fluorescence of the fresh kidney bioptate under long-wave UV-light in porphyria cutanea tarda is referred to for the first time.
Adult↗
[Prognosis of filial generation from mothers with systemic lupus erythematosus].
Seventy three pregnancies in 44 women with systemic lupus erythematosus in our hospital from June 1970 to June 1993 were studied. It included 15 spontaneous abortions (20.55%), 8 induced abortions (10.96%), 9 perinatal fetal deaths (12.33%) and 41 live births (56.16%). Therapy with steroids during pregnancy is harmless and effective. It may reduce pregnant loss rate. Of 41 live births, 35 children were followed up (85.36%). Their physical growth, routine blood test and urinalysis were normal. There were no definite evidences of mental developmental abnormality in children. Two girls had been diagnosed of systemic lupus erythematosus at about nine years old. It is possible that systemic lupus erythematosus may be hereditable to female filial generation. So we suggest that the following-up on children born to mothers with systemic lupus erythematosus have to be done till puberty.
Adolescent↗
[Association of systemic lupus erythematosus and Sjögren's syndrome].
INTRODUCTION: Systemic lupus erythematosus and Sjögren's syndrome are multisystemic autoimmune diseases which can be associated to each other. OBJECTIVE: To investigate if there are any distinct clinical, laboratory or serologic features due to the association of the two diseases that can influence the follow up of these patients. PATIENTS AND METHODS: The authors proved the association of these two autoimmune diseases in 56 patients, and these patients' clinical, laboratory and immunoserologic alterations. 50 patients with Sjögren's syndrome and 50 patients with systemic lupus erythematosus were used as control groups. RESULTS: Compared with Sjögren's syndrome alone, in the cases of the association of the diseases, rheumatoid factor was present less frequently, Ro/SS-A, La/SS-B and DNA antibodies were present more frequently, such as antiphospholipid autoantibodies and antiphospholipid syndrome. Anaemia, leukopenia and lymphopenia were detected more often and the patients were younger than in Sjögren's syndrome. Also, affection of the lung, kidney, skin, central nervous system and serous membranes are more common. The group with systemic lupus erythematosus differs in being older, having thyroiditis, Ro/SS-A, La/SS-B and DNA more frequently. CONCLUSION: Definitive clinical, laboratory and serological features make the difference between the association of the two diseases and the diseases observed alone.
Adult↗
Genetic analysis of the cytochrome P450 CYP2D6 polymorphism in patients with systemic lupus erythematosus.
Previous reports of an association between the polymorphic cytochrome P450 CYP2D6 and systemic lupus erythematosus are conflicting. Following the elucidation of the molecular basis of the CYP2D6 genetic polymorphism, we re-examined the hypothesis of an association of this gene with a susceptibility to system lupus erythematosus by analysing the complete CYP2D6 coding sequence. For this purpose, we studies the occurrence of 16 mutations in genomic DNA from 69 systemic lupus erythematosus patients and a large control group using a previously described polymerase chain reaction-single strand confirmation polymorphism analysis. In addition, we studied the occurrence of 11 alleles and 21 genotypes in the same individuals by the combined use of restriction fragment length polymorphism and allele-specific polymerase chain reaction followed by polymerase chain reaction-single strand confirmation polymorphism analysis. No significant differences in the distribution of overall genotypes and predicted phenotypes were observed between system lupus erythematosus patients and controls. The only new finding of our study is the higher frequency of one non functional allele, namely the CYP2D6*4A, in systemic lupus erythematosus versus control individuals (P = 0.007). This increased frequency was not statistically significant in multiple comparison analysis and was not related to any specific clinical features of systemic lupus erythematosus. These results suggest that CYP2D6 genotype as well as CYP2D6 phenotype are not determinant of susceptibility to systemic lupus erythematosus but the presence of the inactive CYP2D6*4A allele may be a contributory factor.
Adult↗
In situ expression of B7 and CD28 receptor families in skin lesions of patients with lupus erythematosus.
OBJECTIVE: To examine the expression of costimulatory molecules of the B7 and CD28 receptor families in active skin lesions of patients with systemic lupus erythematosus (SLE), subacute cutaneous lupus erythematosus (SCLE), and chronic discoid lupus erythematosus (CDLE). METHODS: The in situ expression of B7-1, B7-2, BB-1, and CD28 was studied by immunohistochemistry, and B7-1 and B7-2 RNA expression was examined by reverse transcription-polymerase chain reaction. RESULTS: Only in lesional skin from SLE, SCLE, and CDLE patients did dermal and epidermal antigen-presenting cells (APC) express B7-1 and B7-2, particularly when in apposition to CD28+ T cells. These B7-1+ and B7-2+ APC bound CTLA-4 fusion protein. In lesional (but not in nonlesional) skin, keratinocytes expressed BB-1. The majority of infiltrating T cells were CD28+. B7-1 and B7-2 RNA were expressed in lesional skin from SLE, SCLE, and CDLE patients; when dermis was separated from epidermis, only faint B7-1 and B7-2 RNA signals were detectable in the epidermis, indicating that dermal but not epidermal cells were the major source of B7-1 and B7-2 RNA. During treatment, both B7-1 and B7-2 protein and RNA expression were reduced. CONCLUSION: These in situ findings suggest that costimulation via the B7-CD28 pathway may be important for the generation and/or propagation of T cell activity in skin lesions of humans with lupus erythematosus. Thus, the manipulation of this pathway (e.g., by CTLA-4 fusion protein) could be an important target for the development of future therapies for LE.
Antigens, CD↗
Cimetidine-associated exacerbation of cutaneous lupus erythematosus.
A patient with systemic lupus erythematosus (SLE) had new LE skin lesions develop following the initiation of cimetidine, a drug that is known to influence a variety of cellular functions. The temporal relationship between the onset of cutaneous LE with the ingestion of cimetidine is reported to alert others to the possibility that this H2 receptor antagonist may exacerbate cutaneous disease activity in patients with LE.
Cimetidine↗