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PubMedDate not supplied

Neonatal lupus erythematosus in offspring of mothers with experimental systemic lupus erythematosus.

Neonatal lupus erythematosus (NLE) syndrome is a result of the transfer of autoantibodies produced by the mother, across the placenta, to the fetus. NLE is characterized by a transient dermatitis, a variety of systemic and hematological abnormalities, and isolated cases of congenital heart block. The latter has been reported to be due to the presence of autoantibodies specific to La (SS-B) and/or Ro (SS-A). As female mice with experimental SLE, induced by immunization with the monoclonal anti-DNA 16/6 Id, produce a variety of autoantibodies including anti-Ro and anti-La antibodies, we examined the relevance of NLE in the murine system. Offspring of SLE-afflicted BALB/c mothers possessed antibody titers to the 16/6 Id, ssDNA, and nuclear extract, which gradually declined until reduced to normal levels by day 60 after delivery. Antibody titers in the sera of the mothers remained elevated throughout this period. Electrocardiograms were recorded from groups of neonates from mothers with experimental SLE. The results indicated that a high percentage of the offspring had defects in their conduction system including first, second, and third degree heart block; significant bradycardia; and wide QRS complex. Normal patterns were observed in offspring of healthy mothers. Experiments done with mice that were exposed to SLE-related autoantibodies early in their development indicated that offspring to mothers with experimental SLE were neither protected nor more susceptible to disease induction by the 16/6 Id.

Animals↗
PubMed1998

[A case of male who was taken systemic lupus erythematosus with chilblain lupus].

A 38-year-old male was admitted to our hospital because of high grade fever, erythema of face and extremities and oral ulcer. The laboratory examination showed leukopenia, high titer (320 dil.) of antinuclear antibody, a positive reaction of anti-Sm antibody. Especially histopathology of hand erythema showed hydrophilic degeneration consist with chilblain lupus. Because his symptoms were consistent with the criteria of American College of Rheumatology (1982), he was diagnosed as systemic lupus erythematosus (SLE). After administration of 60 mg prednisolone daily, the symptoms gradually improved. Any recrudescence of serological abnormalities or clinical symptoms was not observed until now, although chilblain lupus still persisted. Originally SLE is frequent in young female and often attended with renal disease, central nerve disease and serositis. We reported a case of middle-aged male who developed SLE with chilblain lupus which was rare skin lesion of SLE without severe organic lesion and discussed the relation among chilblain lupus, discoid lupus erythematosus (DLE) and SLE.

Adult↗
PubMed1981

The histopathology of lupus erythematosus panniculitis.

Lupus erythematosus panniculitis is a clinical variant of lupus erythematosus in which the main pathologic process involves the deep corium and subcutaneous tissue. We reviewed twenty-nine cases of lupus panniculitis, as well as the cases previously reported in the literature. The histopathologic changes in lupus panniculitis are characterized by a lymphocytic panniculitis, hyaline degeneration of the fat, hyaline papillary bodies, and lymphoid nodular structures in the lower dermis and subcutaneous tissue. Direct immunofluorescence can be important in supplementing the histopathologic study of lupus panniculitis. Lesions of discoid lupus erythematosus are seen in 21% of cases. When this disorder exists in the absence of other typical cutaneous or systemic lesions, the diagnosis of lupus erythematosus has been questioned. We believe that the histopathologic findings of this entity are alone sufficient for a diagnosis of lupus panniculitis, even in the absence of cutaneous or systemic lesions.

Adult↗
PubMed1993

Preliminary, dermatologic first step criteria for lupus erythematosus and second step criteria for systemic lupus erythematosus.

BACKGROUND: Comparisons of cases of systemic lupus erythematosus (SLE) with cases of rheumatoid arthritis and other rheumatologic disorders affords the basis of the 1982 revised criteria of the American Rheumatism Association (ARA) for classifying SLE cases. We address three questions: Do comparisons of LE cases with non-LE cases that have suggestive skin lesions yield criteria for use in dermatology clinics for primary classification of cases with photo distributions of skin lesions? Do comparisons of SLE with cutaneous LE cases yield the same or similar criteria to the revised ARA criteria for SLE? How should subacute cutaneous LE cases be evaluated for signs of significant systemic involvement? METHODS: Discriminant analyses on 168 cases with skin lesions suggestive of LE were performed using data based on the ARA criteria for SLE and study factors for cutaneous LE suggested by the European Academy of Dermatology and Venereology. RESULTS: These yielded two sets of criteria: (1) The 11 preliminary, dermatologic first step criteria (10 plus 1 for discoid lesions and histology) serve to classify cases as LE or non-LE. (2) The 11 preliminary, dermatologic second step criteria classify LE cases as cutaneous LE or systemic LE. Interestingly, 5 of 11 of these second step criteria differ from the 11 ARA criteria for systemic LE. These second step criteria afford a useful means of distinguishing between subacute cutaneous LE cases with or without significant systemic involvement. CONCLUSIONS: The study factors included in both the first and the second step criteria fall into three groups, notably clinical criteria, laboratory criteria, and "added study factors." The latter factors distinguish between the groups compared (LE vs. non-LE and cutaneous vs. systemic LE) but not as well as the study factors included as "criteria."

Humans↗
PubMed1987

Acute gastrointestinal manifestations of systemic lupus erythematosus.

Systemic lupus erythematosus is a common multisystem disorder of young women, and gastrointestinal symptoms are a frequent clinical manifestation. There is serious danger that appropriate therapy may be delayed in the patient with an acute abdomen when systemic lupus erythematosus is not considered as a cause. This paper describes four patients with acute abdomen as a manifestation of systemic lupus erythematosus. They responded dramatically and without complication to steroid therapy. The symptoms recurred in one patient but responded to plasmapheresis and intravenous administration of cyclophosphamide. The other gastrointestinal manifestations of systemic lupus erythematosus and their pathogenesis are discussed. Management requires consideration of systemic lupus erythematosus as the cause of acute abdomen, paracentesis for culture and treatment with high doses of steroids with or without broad-spectrum antibiotics. If there is no notable improvement within 24 hours laparotomy should be performed.

Abdomen↗
PubMed1994

Neonatal lupus erythematosus with cardiac involvement in offspring of mothers with experimental systemic lupus erythematosus.

Neonatal lupus erythematosus (NLE) syndrome is characterized by a transient dermatitis, a variety of systemic and hematological abnormalities, and isolated cases of congenital complete heart block. The latter has been reported to be due to the presence of autoantibodies specific to La (SS-B) and/or Ro (SS-A). As female mice with experimental systemic lupus erythematosus (SLE) induced by immunization with the human monoclonal anti-DNA antibody bearing the 16/6 Id produce variety of autoantibodies including anti-Ro and anti-La antibodies, we looked for NLE related symptoms in the murine model. Offspring of BALB/c mice with SLE possessed high levels of autoantibodies that declined gradually till reduced to normal levels at day 60 after delivery. Electrocardiograms recorded in groups of offspring from mothers with experimental SLE indicated that a high percentage of the offspring had defects in their conductive system including first-, second-, and third-degree heart block, significant bradycardia, and a wide QRS complex. In contrast, a normal pattern was observed in offspring of healthy mothers.

Animals↗
PubMed1979

Subacute cutaneous lupus erythematosus: a cutaneous marker for a distinct lupus erythematosus subset.

We have characterized the clinical and laboratory features of 27 patients who had in common a recurring, superficial, nonscarring type of cutaneous lupus erythematosus (LE) that occurred in a characteristic distribution (subacute cutaneous lupus erythematosus [SCLE]). This clinically distinct form of cutaneous LE has not previously been analyzed as a separate entity and thus, its clinical importance has not been fully appreciated. We found that these patients frequently had a mild systemic illness marked by musculoskeletal complaints and serologic abnormalities. Forty-eight percent had systemic LE by American Rheumatism Association criteria; however, none had serious CNS or renal disease. Thus, those with SCLE are a subset of patients with LE who generally have an illness intermediate in severity between discoid LE and severe systemic LE.

Adult↗
PubMed1998

T cell receptor clonotypes in skin lesions from patients with systemic lupus erythematosus.

Systemic lupus erythematosus is an autoimmune disease characterized by the presence of autoantibodies and by lymphocytic infiltration into lesions at several sites such as skin, kidney, and other organs. Immunohistologic studies have clarified that the majority of lymphocytes in the skin are CD4+ alphabeta T cells. In the present work, to clarify the pathologic role of T cells in the skin of systemic lupus erythematosus patients, we analyzed T cell receptor (TCR) clonotypes of T cells infiltrating into skin lesions. TCR Vbeta gene transcripts from T cells from discoid lesions of the skin and peripheral blood lymphocytes of four systemic lupus erythematosus patients were amplified by reverse transcriptase polymerase chain reaction. Southern blot analysis of polymerase chain reaction product demonstrated the heterogeneous TCR Vbeta repertoire of T cells in the skin of systemic lupus erythematosus. Single-strand conformation polymorphism analysis showed several distinct bands for smears of most TCR Vbeta genes from T cells infiltrating the skin, whereas smears with few bands were found for all TCR Vbeta genes from peripheral blood lymphocytes of the same patients. The number of bands encoding each TCR Vbeta gene from the skin was significantly higher compared with peripheral blood lymphocytes. Sequencing analysis showed a Leucine-X-Glycine amino acid motif at position 96-98 in the CDR3 region at the frequency of 23-24% in skin-accumulated T cells from two patients, whereas the frequency of this motif in peripheral T cells was only 0-3%, indicating limited T cell epitopes. In conclusion, T cells infiltrating into the skin of systemic lupus erythematosus patients might recognize restricted T cell epitopes on autoantigens and trigger the autoimmune reaction in skin lesions.

Amino Acid Sequence↗
PubMed1975

[Morphologic changes in the chorio-allantoic membrane and organs of chick embryos inoculated with blood of patients with systemic lupus erythematosus (concerning the viral etiology of systemic lupus erythematosus)].

Morphological studies of 14 chorionallantoic membranes and organs of chick embryos which had been infected on the 7--8th day of incubation with leucocytic mass from patients with systemic lupus erythematosus were carried out. Corresponding virological and morphological control studies were performed. The changes observed were repeatedly reproduced in multiple inoculations (from the 2nd to the 16th passage). Apart from morphological changes, which may be listed among general pathological processes (impairment of the circulation, dystrophy, proliferation), changes testifying to a possible cytopathic effect disintegration of nuclei, formation of gigantic cells and multinuclear symplasts were also noted. The most pronounced changes, both in the chorionallantoic membrane and in internal organs, were observed on the 3rd--5th passage and on the 3rd--5th day of incubation following inoculation. They correlated with the clinical activity of the process in patients from whom the blood for inoculation had been taken. The data obtained justify the assumption concerning the existence in the blood of the patients with systemic lupus erythematosus a transplantable agent producing a cytopathic action, and may be considered as a new indirect corroborative evidence in favour of the concept of the viral nature of systemic lupus erythematosus.

Animals↗
PubMed1984

Systemic lupus erythematosus with membranous lupus nephropathy in Malaysian patients.

Thirty-one patients with systemic lupus erythematosus had membranous lupus nephropathy (MLN). They were divided into two groups. Group I consisted of 13 patients who had pure MLN but the patients in Group 2 had segmental proliferation in up to 35 per cent of their glomeruli. The rest of the glomeruli had purely membranous change. The patients of Group 2 were no different from the other MLN patients in terms of age, sex and race. The extrarenal disease in both groups was extensive and severe. The renal disease was usually associated with the nephrotic syndrome or oedema but was asymptomatic throughout in one patient. Both renal and extrarenal features responded to treatment initially but relapses were frequent and often severe. Relapses often occurred as treatment was discontinued or medication reduced. Survival at six years in Group I was 62 per cent and in Group 2 was 50 per cent. Only one patient died with renal failure although five patients had impaired renal function at death. The chief causes of death were disease of the central nervous system and infection.

Adolescent↗
PubMed1998

Postmenopausal estrogen replacement therapy and the risk of developing systemic lupus erythematosus or discoid lupus.

OBJECTIVE: There is evidence that estrogens play a role in the etiology of systemic lupus erythematosus (SLE), but this has not yet been shown for discoid lupus. We examined the association of postmenopausal estrogen use with the development of SLE and discoid lupus. METHODS: We did a case-control evaluation, using the UK based General Practice Research Database. We analyzed 41 cases with SLE, 34 cases with discoid lupus, and 295 age, sex and practice matched controls, and estimated relative risk estimates (odds ratios) in relation to estrogen exposure duration as well as total cumulative dose and estrogen type (alone or combined with progestogens). RESULTS: While short term estrogen exposure was not associated with increased risk, the risk of developing SLE (adjusted OR 2.8; 95% CI 0.9-9.0) or discoid lupus (adjusted OR 2.8; 95% CI 1.0-8.3) was significantly increased among current users who were exposed for 2 or more years. The adjusted RR estimate comparing longer term estrogen users and nonusers for all cases (SLE and discoid lupus combined) was 2.8 (95% CI 1.3-5.8; p < 0.01). A difference was found between longterm users of estrogens alone (OR 5.3; 95% CI 1.5-18.6) and those who used estrogens combined with progestogens (OR 2.0; 95% CI 0.8-5.0), compared to nonusers. CONCLUSION: Our findings suggest that longer term use of postmenopausal estrogens plays a role in the etiology of both SLE and discoid lupus. There is a suggestion that progestogens may reduce the effect of estrogens on these autoimmune disorders.

Aged↗
PubMed2002

Characterization of the inflammatory infiltrate and expression of endothelial cell adhesion molecules in lupus erythematosus tumidus.

Lupus erythematosus tumidus (LET) is a disease with characteristic clinical and histopathologic features that has not always been considered a subset of cutaneous lupus erythematosus (CLE). Although LET was first mentioned in the literature in 1930, it has rarely been documented, and immunohistochemical studies have never been performed. The aim of the present study was to characterize the inflammatory infiltrate and to analyze the expression of endothelial cell adhesion molecules in skin specimens from patients with LET and to compare the results with those from patients with other variants of CLE, such as discoid lupus erythematosus (DLE) and subacute cutaneous lupus erythematosus (SCLE). Cryostat sections of lesional skin specimens from ten patients with LET demonstrated an infiltrate composed of more than 75% CD4+, CD8+, and HLA-DR+ cells. Interestingly, CD45RO+ cells, in contrast to CD45RA+ cells, were the prevailing inflammatory cell population. Compared with skin specimens from patients with DLE and SCLE, the mean expression of CD4+ and CD8+ cells was higher (but not significantly so) in LET, and no differences were observed with the other three antibodies. Furthermore, in contrast to controls, intercellular adhesion molecule-1, vascular adhesion molecule-1, E-selectin, and P-selectin showed the same expression pattern in skin specimens from patients with DLE, SCLE, and LET. In conclusion, the inflammatory infiltrate of LET primarily consists of CD4+/CD8+ lymphocytes. Furthermore, expression of endothelial cell adhesion molecules was equally upregulated in LET compared with the expression in DLE and SCLE, suggesting a similar immunopathomechanism of these subtypes of CLE.

Amine Oxidase (Copper-Containing)↗
PubMed1984

Pregnancy outcome in women with systemic lupus erythematosus.

Systemic lupus erythematosus has a predilection for women of childbearing age and may potentially complicate pregnancy. Poor pregnancy outcome in women with systemic lupus erythematosus has been principally ascribed to the presence of renal involvement. Maternal risks have been more difficult to evaluate because of the unpredictable nature of the disease and limited individual experience. The present study reports the outcome of 108 pregnancies in 39 women affected by systemic lupus erythematosus with and without renal manifestation of the disease. Prematurity, small-for-gestational-age birth weight, stillbirth, and spontaneous abortion occurred more frequently in women after the diagnosis of systemic lupus erythematosus was made. Those women with renal involvement had an even further increase in spontaneous abortions as well as a decrease in successful pregnancy outcome (P less than .05). Morbidity in this group of women was rare in the immediate postpartum period, but increased morbidity and mortality did occur in the first two years after delivery.

Abortion, Spontaneous↗
PubMed2005

Abetimus sodium: a new therapy for delaying the time to, and reducing the incidence of, renal flare and/or major systemic lupus erythematosus flares in patients with systemic lupus erythematosus who have a history of renal disease.

The safety and efficacy of abetimus sodium (abetimus) has been evaluated in 13 controlled and uncontrolled clinical trials involving > 800 patients or subjects. The two pivotal trials enrolled a total of 547 patients with systemic lupus erythematosus (SLE) who had a history of lupus nephritis. Evidence of clinical effectiveness of abetimus comes from analyses of data from patients with SLE and high-affinity antibodies to the DNA epitope of abetimus at baseline; a retrospective subgroup in the pivotal Phase II/III LJP-394-90-05 trial (90-05 trial) and the intent-to-treat population in the Phase III LJP-394-90-09 trial (90-09 trial). These studies enrolled SLE patients who had experienced prior renal manifestations of their disease and had elevated anti-dsDNA antibodies at baseline by Farr assay. Both were long-term studies, with a mean duration of treatment participation of 371 days in 90-05 and 310 days in 90-09 for the population of patients with high-affinity antibodies at baseline. The 90-05 and 90-09 studies, as well as all other clinical studies of abetimus, consistently showed that treatment with abetimus resulted in durable and persistent reductions in anti-dsDNA antibodies in SLE patients. Treatment with abetimus was associated with statistically significant decreases in anti-dsDNA antibody levels from baseline compared with placebo in both the 90-09 and 90-05 trials. Positive trends were noted for the incidence of renal flares and major SLE flares in patients treated with abetimus. Abetimus appeared to be well tolerated for treatment periods of < or = 22 months.

Clinical Trials as Topic↗
PubMed2000

[Cardiological state of offsprings of the mothers suffering from systemic lupus erythematosus].

Systemic lupus erythematosus is a disease in which inflammatory process provoked by different antibodies affects many organs and systems. The circulatory system is one of them. In patients with systemic lupus erythematosus cardiac disorders are generally known and well proved. It is known that this disease has heritage background. Thus, the offsprings of patients suffering from systemic lupus erythematosus belong to a risk group. Moreover, it is thought that maternal antibodies crossing transplancentally to the fetus cause damages of tissues including the heart. The aim of this study was to evaluate cardiological status of 38 children whose mothers suffered from systemic lupus erythematosus. In all sick mothers the diagnosis fulfilled criteria of the American Rheumatism Association. The mothers have been remaining under medical treatment while the children have been under control and simultaneously prophylaxis of lupus has been undertaken. The study was undertaken in 17 girls and 21 boys aged 3 to 18 years (average: 12 +/- 4.5 years). Physical development of presented children was satisfactory. During cardiological examination all subjects were in good general condition, without any clinical evidence of collagen disease and infection. Obtained results were compared with the ones found in control group of 38 children of healthy mothers, being at the same age. Study methods were: physical examination, arterial blood pressure measurement, standard and 24 hours according to Holter method ECG record, echocardiographic and Doppler examination, and physical performance test according to Bruce's protocol. In children of sick mothers examined laboratory parameters were within the normal limits excluding the presence of antinuclear antibodies (controlled by indirect immunofluorescence test), result of which was positive in 15 studied children (39%). In the group of the children of sick mothers the abnormalities of sinus node function were detected in 12 cases (32%), significantly more often than in the control. There were found abnormalities of atrio-ventricular and intraventricular conduction in 15 subjects (40%). The premature beats of ventricular origin were noticed in 3 cases (8%). These disturbances were significantly different from the control group. In addition, correlation between the presence of antinuclear antibodies and the cardiac abnormalities was taken into consideration. So, significant correlation between antinuclear antibodies and heart rhythm disorders was proved. During echocardiographic examination structural and functional abnormalities were found. They were: ventricular septal defect (muscular part) (1), pericarditis effusion (1), prolapse of the mitral valve anterior leaflet (2), mitral valve regurgitation of the second degree (2) and increased diameter of left atrium (8). One girl from the studied group, suffering from atrio-ventricular block of III* was operated on because of persistent ductus arteriosus still in the newborn's period. At the same time the permanent pace-maker was implanted. After 1 year of age this girl was operated on because of atrial septal defect (ASD II). In studied group of children echocardiographic global indices of left ventricular systolic function were normal. The subclinical impairment of diastolic left ventricle function was found in 8 children with increased left atrium-aorta index. Both the theoretical knowledge and the results of the studies suggest that the offsprings of mothers suffering from SLE need a careful cardiological observation.

Adolescent↗