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PubMed1982

Natural killer augmentation in systemic lupus erythematosus via a soluble mediator derived from human lymphocytes.

Seventeen patients with diagnosed systemic lupus erythematosus were studied for natural killer activity and the ability of a human soluble immune-response factor to modify this activity. Defective natural killer activity was found in patients with systemic lupus erythematosus regardless of the stage of disease, medication, or organ system involvement (24.6% versus 47.8% cytotoxicity). After treatment with the immune-response factor, the natural killer activity increased in all patients, with a mean of 47.3% compared with 24.6% before treatment. This product has other immunologic activities and was shown to be free of interferon. The mechanism of action is not yet known, but such an immune-response factor has potential use in the treatment of systemic lupus erythematosus.

Adolescent↗
PubMed1973

Systemic lupus erythematosus. Studies of the antibodies bound to skin.

Systemic lupus erythematosus is characterized by antibodies demonstrable by immunofluorescence on the renal glomeruli and at the basement membrane area of both normal and involved skin. Acid eluates from glomeruli and from normal-appearing skin of three patients with systemic lupus erythematosus contained an antinuclear antibody. This antibody fixes complement and produces a mixed immunofluorescent pattern. Anti-deoxyribonucleic acid or antiextractable nuclear antigen antibodies may be present. This antibody is concentrated on the skin and glomerular basement membrane in proportion to the total serum IgG concentration. In two cases the skin eluate contains, in addition to the antinuclear antibody, a basement membrane antibody that fixes complement, gives a linear immunofluorescent pattern, and appears to be similar (although not identical) to the pemphigoid antibody.

Adolescent↗
PubMed1991

Prognosis of systemic lupus erythematosus and the factors that affect it.

The prognosis of systemic lupus erythematosus has improved over the past four decades. Articles published in the past year continue to demonstrate this improved survival, both overall and in patients with renal disease. Several factors may be associated with improved survival, including earlier diagnosis, better treatment for systemic lupus erythematosus, and improved medical therapy in general. Hydroxychloroquine has now clearly been shown to prevent flares, and ancrod has been shown to improve renal disease in patients with glomerular thrombosis. Treatment with dialysis and transplantation resulted not only in improved survival but also in improved renal status, and reduction in overall disease activity. Specific organ damage continues to be an issue, primarily with regard to kidney disease and neurocognitive impairment. The inclusion of health status assessment in the evaluation of patients with lupus, and as an outcome measure, is discussed.

Health Status↗
PubMed1990

Cerebral transmural angiitis and ruptured cerebral aneurysms in patients with systemic lupus erythematosus.

Two cases of cerebral transmural angiitis and ruptured aneurysm in patients with established systemic lupus erythematosus are presented. A 22-year-old woman with a 4-year history of systemic lupus erythematosus was found to have a ruptured cerebral aneurysm at the trifurcation of the middle cerebral artery. She died 10 days after admission because of the brain swelling. Necropsy showed remarkable brain edema and focal transmural angiitis at the site of the ruptured aneurysm. Another 29-year-old woman with a 3-year history of systemic lupus erythematosus had an acute subdural hematoma resulting from a ruptured aneurysm of the right anterior cerebral artery. She was surgically treated. The ruptured aneurysm was resected and examined histologically. The transmural angiitis was demonstrated. This report describes two radiographically and pathologically confirmed cases of central nervous system lupus producing focal angiitis of a cerebral artery with secondary aneurysm formation.

Adult↗
PubMed2006

Shrinking lung syndrome in a 14-year-old boy with systemic lupus erythematosus.

Pulmonary complications occur frequently in people with systemic lupus erythematosus. We report on an adolescent with an acute onset of dyspnea and pleuritic chest pain with severe restrictive lung physiology on pulmonary function testing (forced vital capacity, 20% of predicted) who had no evidence of parenchymal lung or pleural disease. He was found to have restricted diaphragmatic movement as assessed by fluoroscopy, without evidence of generalized respiratory muscle weakness. His clinical presentation and results of diagnostic tests were typical for shrinking lung syndrome. Given the rarity of shrinking lung syndrome in the pediatric age range, many clinicians are not aware of it as a clinical entity. Shrinking lung syndrome should be included in the differential diagnosis of dyspnea in both children and adults with systemic lupus erythematosus.

Adolescent↗
PubMedDate not supplied

Lupus erythematosus cells in pleural fluid cytologic diagnosis in two patients.

Lupus erythematosus (LE) cells were demonstrated inpleural fluids from two patients with systemic lupus erythematosus (SLE). They were observed in preparations stained with the Giemsa and the Papanicolaou stains. The finding of LE cells in serous fluid has rarely been reported. Our results suggest that it is imporant to search for LE cells in serous fluid in patients in whom the diagnosis of SLE has not been established.

Adult↗
PubMed1996

Giant coronary artery aneurysms and myocardial infarction in a patient with systemic lupus erythematosus.

A 26-year-old woman with systemic lupus erythematosus and end-stage renal disease presented for renal transplantation. She had no findings of cardiac disease. Radiologic evaluation, cardiac magnetic resonance imaging, and coronary angiography showed giant aneurysms of the right and left anterior descending coronary arteries. Despite therapy, acute anterior myocardial infarction developed during follow-up. Giant coronary artery aneurysms associated with systemic lupus erythematosus are unusual and their management is controversial. Antithrombotic therapy probably should be considered for giant coronary artery aneurysms.

Adult↗
PubMed1984

Bone lesions in systemic lupus erythematosus.

Routine joint radiographs of 125 patients with systemic lupus erythematosus were studied. A total of 121 patients had a clinical history of articular symptoms. Cystic bone lesions were found in 51 patients (41%). The lesions, which were typically located subchondrally in the small joints of hands and feet, appeared as well-defined radiolucent areas surrounded by either normal bone or a narrow sclerotic zone. Nutritional disturbance in the bones, caused by the vasculitis associated with systemic lupus erythematosus, is proposed as a possible pathogenetic mechanism of the cystic lesions.

Adult↗
PubMed1987

Hip arthroplasty in patients with systemic lupus erythematosus.

Forty-three prosthetic hip replacements (twenty-nine conventional total hip replacements and fourteen bipolar endoprosthetic replacements) were implanted between January 1971 and June 1982 in thirty-one patients who had systemic lupus erythematosus. All but four patients had stage-III or IV osteonecrosis of the femoral head. The median age at operation was forty-three years, and the median length of follow-up was fifty-seven months. Ratings were good or excellent for all but three total hip arthroplasties at a mean of sixty-six months of follow-up. Complications included delayed wound-healing (in approximately 15 per cent) and superficial wound infection (in approximately 10 per cent). The occurrence of these complications could not be correlated with the use of corticosteroids at the time of the operation. Twenty-five per cent of the patients, who were a mean of forty-three years old at operation, died less than five years postoperatively from complications related to systemic lupus erythematosus. Conclusions regarding the systemic effects of hip arthroplasty in patients with systemic lupus erythematosus could not be drawn on the basis of this study. Total hip arthroplasty uniformly provided a good or excellent result in patients of all ages who had systemic lupus erythematosus at a mean length of follow-up of sixty-six months. An increased incidence of local wound complications, which were unrelated to the use of corticosteroids, should be expected in patients with systemic lupus erythematosus who undergo prosthetic arthroplasty of the hip.

Adult↗
PubMed1999

Autoimmune thyroid disorders in juvenile chronic arthritis and systemic lupus erythematosus.

The appearance of autoimmune thyroiditis in the course of other autoimmune diseases, which do not affect specific organs (systemic lupus erythematosus, Sjögren syndrome, rheumatoid arthritis and others), is more frequent than is usually believed. Nevertheless, it is scarcely studied, especially in children. The purpose of this study was to look for autoimmune lesions of the thyroid gland in children suffering from juvenile chronic arthritis (JCA) and systemic lupus erythematosus (SLE). Twenty seven children having JCA and twelve children with SLE, aged 5 to 18 years, were enrolled into study. In all of them the disease was in an active phase. The serum levels of total thyroid hormones (T3, T4) and TSH, thyroid antibodies (TAB and MAB) and antinuclear antibodies (ANAB) were analyzed using respectively fluoroimmunologic, microhemagglutination and indirect immunofluorescention tests. According to our results, autoimmune thyroiditis was found in 12 out of 27 children with JCA (44.4%); 85.2% of them were euthyroid, 11.1% had a compensated hypothyroidism, and 3.7% had Hashi-toxicosis. From a clinical point of view, very interesting was the combination of JCA, autoimmune thyroiditis and pseudoxanthoma elasticum in a 13-year old girl. Positive thyroglobulin antibodies (1:80-1:5120) were found in 17 out of 27 cases of JCA (63%). The microsomal antibodies were elevated (1:100-1:1600) in 7 out of 27 (25.9%); antinuclear antibodies (1:80-1:640) were detected in 15 out of 27 cases of JCA (55.5%). A simultaneous elevation of all three kinds of antibodies was found in 14.8% of children with JCA, and of TAB and MAB--in 18.5%. Thyroid gland disorders were detected also in children suffering from SLE. Thyroglobulin antibodies were positive (1:80-1:5120) in 7 out of 12 cases. Antinuclear antibodies (1:320-1:2560) were detected in 8 out of 12 cases (66.7%). The serum levels of T3, T4 and TSH were in the reference limits in all children with SLE. The present study suggests that involvement of the thyroid gland is not uncommon in autoimmune disease in Autoimmune thyroiditis can occur in association with other autoimmune diseases, affecting some organs or systems, such as the insulin-dependent diabetes mellitus, pernicious anaemia, thrombocytopenia, vitiligo, as well as some chromosomal aberrations--Turner's syndrome, Noonan's syndrome and Down's disease [1]. The appearance of autoimmune thyroiditis together with other autoimmune diseases which do not affect specific organs (such as systemic lupus erythematosus, Sjögren syndrome) is the reason to classify them in a common subgroup of the autoimmune polyendocrine syndromes--type IIID [2]. The rheumatic diseases are--more frequently than suspected--associated with autoimmune thyroiditis, but this connection is not well studied. The literature offers very scarce information on the problem, especially for the childhood. The purpose of this study was to look for autoimmune lesions of the thyroid gland in children suffering from juvenile chronic arthritis (JCA) and systemic lupus erythematosus (SLE).

Adolescent↗
PubMed2003

Antiphospholipid antibodies in 57 children and adolescents with systemic lupus erythematosus.

OBJECTIVE: To investigate the frequencies and behavior of antiphospholipid antibodies in 57 children and adolescents with systemic lupus erythematosus. METHODS: Anticardiolipin antibodies were investigated by ELISA and lupus anticoagulant antibodies by the international tests recommended. The antiphospholipid antibodies analyses were performed in frozen samples (mean of 5.3 samples per patient obtained during a mean follow-up period of 3 years and 7 months) and on blood samples collected between January 1997 and November 1998 (mean of 2.5 samples per patient during a 2-year follow-up period). RESULTS: The frequencies of antiphospholipid antibodies (anticardiolipin and lupus anticoagulant) were similar in the samples collected prospectively and in the frozen samples (retrospective study): 63.2% and 75.4% respectively. Positivity for these antibodies fluctuated during the follow-up period and was not associated with any clinical or laboratory parameters of lupus erythematosus, including autoantibodies and also including disease activity and/or severity scores. CONCLUSIONS: The frequencies of antiphospholipid antibodies in children and adolescents with lupus erythematosus were similar to those observed in adults. The positivity fluctuated during the follow-up and was not correlated with clinical and/or laboratory disease parameters.

Adolescent↗
PubMed1987

Complete heart block as the initial manifestation of systemic lupus erythematosus.

Complete heart block is a rare complication of systemic lupus erythematosus, as well as other rheumatic diseases. We describe a patient with systemic lupus erythematosus who presented with complete heart block as the initial manifestation of the disease. Anti-Sjögren's syndrome antibodies as well as antiribonucleoprotein antibodies were present in the patient's serum.

Adult↗
PubMed1988

Lupus erythematosus in a patient with long-standing multiple sclerosis.

The occurrence of both lupus erythematosus and multiple sclerosis in several members within the same family has been previously documented. In the past, patients manifesting symptoms and findings compatible with both of these diseases have posed difficult diagnostic problems. This report concerns a patient with long-standing multiple sclerosis and in whom cutaneous and serologic lupus erythematosus developed. To our knowledge this is the first such case reported in the English literature. A daughter of this patient had developed serologic lupus erythematosus. The intensified study of patients with both of these diseases and those families with several members with one of these diseases may lead to new insights into the cause and pathogenesis of these disorders.

Adult↗
PubMed1993

Systemic sclerosis (scleroderma) associated with discoid lupus erythematosus.

Six patients with systemic sclerosis (SS) and discoid lupus erythematosus (DLE) were studied to determine whether such cases have some common clinical and laboratory findings. DLE preceded SS in all cases. Three patients had diffuse scleroderma with lung and esophagus involvements and the others limited scleroderma. Three patients had anti-topoisomerase-I and antiribonucleoprotein antibodies, 2 had either of them and the remaining anticentromere antibodies. Four had DLE located on the scalp, leading to alopecia. The other 2 had DLE on the face and extremities. No case fulfilled criteria for systemic lupus erythematosus (SLE). The present cases with SS and DLE, but without SLE, indicate that this type of systemic-cutaneous collagen disease overlap does exist and may be not so rare.

Adult↗
PubMed1987

[Pan T, T4 and T8-positive lymphocytes in the peripheral blood of patients with systemic lupus erythematosus].

In a group of 23 patients with systemic lupus erythematosus by means of monoclonal antibodies differentiation markers were investigated on peripheral blood T-lymphocytes. Individual specific differences in the percentage of T1-, T3-, T4- and T8-positive cells were found, indicating various possibilities of immunological disorders in this disease. Furthermore, in comparison with healthy donors, an increased number of T4-, T8-double stained cells was detected in the peripheral blood of patients with systemic lupus erythematosus. Normally, this cell phenotype can be detected in the thymus only. Therefore, it becomes necessary to perform parallel and simultaneous determinations, respectively, of several T cell markers in order to gain a more exact insight into the disturbances of the immune system and to exclude incorrect interpretations of the phenotypical distribution of T cell markers.

Fluorescent Antibody Technique↗
PubMed1986

Reticuloendothelial system Fc receptor function in the drug induced lupus erythematosus syndrome.

In view of reported delayed clearance of 51Cr labelled IgG sensitized autologous erythrocytes in systemic lupus erythematosus, we measured clearance half-time (t1/2) in 16 patients with the drug induced lupus erythematosus syndrome. The mean t1/2 for the patients with drug induced lupus was 52 min, and for 16 normals mean was 48 min, a difference without statistical significance. Nine of the patients with drug induced lupus had laboratory abnormalities only, and mean t1/2 in this group was not significantly different from that in the 7 symptomatic patients. T1/2 was not significantly associated with Clq or DNA binding levels in symptomatic or asymptomatic patients. Normal mean clearance of model immune complexes was thus present in patients with drug induced lupus, with and without symptoms, and may protect against renal and central nervous system disease in drug induced lupus erythematosus.

Adult↗
PubMed1977

Immunohistologic findings in the lung in systemic lupus erythematosus.

A 29-year-old woman with systemic lupus erythematosus (SLE) developed dyspnea, hemoptysis, pleuropericarditis, and azotemia shortly after an episode of arthritis and progressive hair loss. She had a high titer of radioimmune anti-DNA Antibodies, positive fluorescent anti-smooth muscle antibodies, and depressed C3 levels in her serum. Antiglomerular basement membrane antibodies were negative, and the titer of antibodies against extractable nuclear antigen was within normal limits. Cryoglobulins and lupus erythematosus cell preparations were negative. Despite steroid therapy and other supportive measures, including dialysis, she died ten days after admission. Percutaneous renal and pulmonary biopsies were performed postmortem at bedside and were processed for immunohistology. Identical granular deposits of C3 and IgG were found in both the lungs and the kidneys. This finding suggests that a common pathogenetic mechanism is operating in the development of pneumonitis and nephritis in SLE, and is in agreement with the currently held views on immune-complex diseases.

Adult↗
PubMed1998

[Prevalence of psychiatric disturbances and psychopathologic status in patients with systemic lupus erythematosus].

BACKGROUND: Psychiatric disorders are commonly associated with systemic lupus erythematosus (SLE). Some authors consider that these disorders can be associated with cognitive impairment. OBJECTIVES: The objectives of this study were to establish the particular prevalence of psychiatric disorders in these patients and to investigate the relationship between psychiatric disorders, cognitive impairment, life quality, psychological status and adjustment to illness. MATERIAL AND METHODS: A cross-sectional study is reported of 46 spanish patients with systemic lupus erythematosus (SLE) attending the outpatients clinics of the Internal Medicine and Rheumatology Departments at a University Hospital. The following instruments were used: a Structured Clinical Interview for DSM-III-R, the Nottingham Health Profile, the Symptom Check-List 90-Revised, the Psychological Adjustment to Illness Scale and the Integrated Program of Neuropsychological Assessment Test-Barcelona. RESULTS: The prevalence of psychiatric disorders at interview was 33%. The most common diagnoses were affective disorders and psycho-organic syndrome. Affective disorders were associated with a poorer life quality, number of hospitalizations and psychiatric background. Adjustment to illness accounted for the psychologic status of the patients.

Adult↗