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PubMed2000

[Subacute cutaneous lupus erythematosus].

SKIN LESIONS: The concept that patients with subacute cutaneous lupus erythematosus (SCLE) skin lesions represent a distinct lupus erythematosus subset was proposed in 1979 by Sontheimer and supported by many studies. Skin lesions are papulo-squamous, psoriasiform or annular. Photosensitivity is a common complaint and photo-reproduction is significantly frequent in these patients. They persist for weeks or months and typically heal without atrophy or scarring with hyperpigmentation or more often hypopigmentation. SYSTEMIC INVOLVEMENT: Systemic disease is generally quite mild and the prognostic is usually favourable. High-titer, precipitating antibodies to Ro/SSA and HLA DR2 and/or HLA DR3 are strongly associated with SCLE. In some observations, the relation-ship between SCLE and drugs has been recognized. TREATMENT: Antimalarial agents are first line systemic treatment. Other therapies, including Thalidomide are helpful for patients with resistant disease. The pathomechanisms of photosensitive SCLE involved antigens Ro/SSA, epidermal and dermal cytokines, intercellular adhesion molecules, mononuclear cells.

Acute Disease↗
PubMed1965

Systemic lupus erythematosus and renal transplantation. Report of two cases.

Two cases of systemic lupus erythematosus along with terminal uremia secondary to lupus nephritis are presented. Both patients were treated with renal hemodialysis and transplantation. Certain mechanical, infectious, and immunologic problems were responsible for their death. There was no evidence of resurgence of lupus erythematosus in either patient, and the transplanted kidneys did not show evidence of lupus nephritis. Renal transplantation should be considered as a last resort in treatment of patients with terminal lupus nephritis.

Adolescent↗
PubMed2003

Thalidomide for treatment of severe generalized discoid lupus lesions in two patients with systemic lupus erythematosus.

We describe 2 patients with systemic lupus erythematous whose widespread discoid lupus erythematosus was unresponsive to systemic steroids and antimalarial agents. They showed dramatic improvement to thalidomide at a dose of 300 mg/d, with maximum benefit achieved within 15 weeks of therapy. Dosages of 50 to 100 mg/d were effective in maintaining remission for 1 year. However, thalidomide-induced neuropathy was observed in both cases.

Adult↗
PubMed1978

B-lymphocyte alloantigens associated with systemic lupus erythematosus.

We examined B-lymphocyte alloantigens in 41 patients with systemic lupus erythematosus and 184 controls, using a panel of 47 pregnancy serums, and compared reaction frequencies of individual serums. One serum, la-715, reacted with B lymphocytes from 75.6 per cent of patients and 14.1 per cent of controls (Pc less than 0.005, relative risk 18.8). Twenty-eight of the patients were also typed with a panel of HLA-D-related serums from the Seventh International Histocompatibility Workshop, HLA-DRw types assigned, and compared to 490 Workshop controls. Both HLA-DRw2 (57.1 per cent vs. 26.4 per cent, Pc less than 0.004) and HLA-DRw3 (46.4 per cent vs. 22.2 per cent, Pc less than 0.03) were increased in systemic lupus erythematosus. This study demonstrates that select B-lymphocyte alloantigens, which are controlled by genes in the major histocompatibility complex, are present in increased frequency in systemic lupus erythematosus.

Adolescent↗
PubMedDate not supplied

Subacute cutaneous lupus erythematosus on the lines of Blaschko.

We describe a 42-year-old woman who had erythemateous plaques with sharply demarcated margins and fine scaling on her right trunk and leg. The lesions had a linear distribution following the lines of Blaschko. Histological findings supported the diagnosis of subacute cutaneous lupus erythematosus (SCLE) on the lines of Blaschko. Treatment with systemic corticosteroids and anti-malarial agents resulted in remission. Up to now, eighteen cases of linear lupus erythematosus have been described. Linear lupus erythematosus mostly affects children presenting with lesions on the scalp that were classified as discoid lupus erythematosus. To our knowledge, this is the first case of subacute cutaneous lupus erythematosus on the lines of Blaschko.

Adolescent↗
PubMed1980

IgA-containing circulating immune complexes in dermatitis herpetiformis, Henoch-Schönlein purpura, systemic lupus erythematosus and other diseases.

The sera of patients with dermatitis herpetiformis, Henoch-Schönlein purpura and systemic lupus erythematosus were examined for IgA-containing immune complexes using a newly described radioimmunoassay. The IgG Raji cell radioimmunoassay and the 125I-C1q binding assay were also used to detect IgG- and IgM- containing soluble immune complexes. IgA-containing immune complexes were found in the sera of twelve of forty-nine (24%) patients with dermatitis herpetiformis, four of six (67%) patients with Henoch-Schönlein purpura, and seven of ten (70%) patients with systemic lupus erythematosus. IgG- or IgM- containing immune complexes were also found in six of forty-seven patients with dermatitis herpetiformis, in one of six patients with Henoch-Schönlein purpura, and in nine of ten patients with systemic lupus erythematosus, by either the 125I-Clq binding assay or the IgG Raji cell assay. The finding of soluble IgA immune complexes in a high percentage of patients with systemic lupus erythematosus and Henoch-Schönlein purpura suggests that they may play an important role in the pathogenesis of these diseases. In contrast, their low prevalence in patients with dermatitis herpetiformis suggests that IgA-containing immune complexes may not play a major role in the pathogenesis of dermatitis herpetiformis.

Antigen-Antibody Complex↗
PubMed2002

The value of lymphocytopenia as a marker of systemic involvement in cutaneous lupus erythematosus.

BACKGROUND: Some patients suffering from cutaneous lupus erythematosus (CLE) develop extracutaneous manifestations during the course of the disease: up to 5% of patients with discoid LE (DLE) and up to 30% of subacute cutaneous LE (SCLE) patients show systemic involvement. Recent studies revealed some markers indicating systemic manifestations of CLE patients. However, the significance of diminished peripheral lymphocyte numbers as a marker of systemic involvement in CLE has not been investigated before. OBJECTIVES: To determine the value of lymphocytopenia (< 1500 cells microL(-1)) as a marker of extracutaneous manifestations in CLE patients. : Methods The records of 72 CLE patients (44 DLE; 28 SCLE) were investigated. Systemic involvement was defined in accordance with the criteria of the European Academy of Dermatology and Venereology. Analyses of peripheral lymphocyte numbers were done by fluorescence-activated cell sorter analysis. RESULTS: Five CLE patients developed extracutaneous manifestations during the course of disease. All these patients were lymphocytopenic. Differences between peripheral lymphocyte numbers of CLE patients with and without additional systemic involvement were highly significant (P < 0.01). CONCLUSIONS: Our results suggest that lymphocytopenia in patients with CLE is a high sensitive but low specific marker of systemic involvement.

Adult↗
PubMed1994

Indicators of disease activity, prognosis, and treatment of systemic lupus erythematosus.

In this article, assessment of disease activity in systemic lupus erythematosus (SLE), including available instruments and their use in patients with SLE, is discussed. Several validated measures, including the SLAM (Systemic Lupus Activity Measure), SLEDAI (Systemic Lupus Erythematosus Disease Activity Index), LAI (Lupus Activity Index), ECLAM (European Consensus Lupus Activity Measurement), and that of the BILAG (British Isles Lupus Activity Group) are now available and should be included in studies of new laboratory measures, therapeutic trials, and studies of outcome and prognosis. The prognosis of SLE has improved over the past 4 decades, including 20-year survival rates. With improved survival, other outcome measures such as specific organ function and health status need to be considered. Administration of hydroxychloroquine remains an important part of therapy for SLE. The use of cyclophosphamide should be reserved for severe manifestations of the disease. Newer forms of therapy, particularly immunotherapy, have been tested in animal models.

Humans↗
PubMed1992

[Systemic lupus erythematosus after thymectomy in myasthenia gravis].

The authors describe a case of systemic lupus erythematosus in a 33-year-old female patient who developed the disease 12 years after thymectomy on account of myasthenia gravis. The activity of systemic lupus erythematosus was controlled by pulsed treatment with methyl prednisolone followed by long-term oral glucocorticoid and immunosuppressive therapy. The authors discuss the risk of systemic lupus erythematosus after thymectomy on account of myasthenia gravis with regard to the HLA haplotype.

Adolescent↗
PubMed1992

Respiratory disease in systemic lupus erythematosus: correlation with results of laboratory tests and histological appearance of muscle biopsy specimens.

BACKGROUND: In systemic lupus erythematosus, certain laboratory tests and evidence from muscle biopsy specimens of lymphocytic vasculitis reflect disease activity. A study was designed to determine if such indices predict respiratory lesions, and in particular whether the presence of vasculitis in quadriceps muscle reflects respiratory muscle function. METHODS: Twenty seven 27 patients with systemic lupus erythematosus were studied, ten of whom were consecutive untreated patients and 17 having clinically active disease and being treated. They were prospectively evaluated on the basis of erythrocyte sedimentation rate, lymphocyte count, C3 degradation products, quadriceps muscle biopsy, spirometry, lung volumes, carbon monoxide transfer factor, and mouth pressure during a maximal sniff. RESULTS: Lung function test results were abnormal in 12 patients. Vital capacity was reduced in seven, carbon monoxide transfer factor capacity in five, and mouth pressure was low (< 70% predicted) in ten. Lymphocytic vasculitis was seen in the muscle biopsy specimens of ten patients. No correlation was found between laboratory tests and lung function or mouth pressure, or between the presence of lymphocytic vasculitis and mouth pressure. In untreated patients, those with lymphocytic vasculitis had lower spirometric values. CONCLUSIONS: In systemic lupus erythematosus, evidence from muscle biopsy specimens of lymphocytic vasculitis is not predictive of impaired inspiratory muscle function as measured by mouth pressure. In untreated patients there were relationships between some laboratory test results and respiratory function, but this was not the case for the whole group. In systemic lupus erythematosus, laboratory tests and evidence from muscle biopsy specimens of lymphocytic vasculitis are therefore unlikely to be helpful in the assessment of respiratory disease.

Adult↗
PubMed2005

[Signal transduction abnormalities in systemic lupus erythematosus].

Engagement of T cell receptors by antigen-presenting cells or stimulation by cytokines determine whether the cell will become activated, anergic or die via apoptosis or necrosis. Ca2+ is a key second messenger that delivers signal from the cell surface, reactive oxygen intermediates and nitric oxide are recently recognized as important mediators of T cell activation. Nitric oxide is a multifunctional intracellular and intercellular messenger induces mitochondrial biogenesis in many cell types, such as lymphocytes. Mitochondria produce reactive oxygen intermediates and store and release Ca2+ in response to activation and death signals. Rapid Ca2+ fluxing is increased while sustained Ca2+ signaling is decreased in lupus T cells. Lupus T cells contain increased numbers and mass of mitochondria. Serum nitric oxide levels and production of nitric oxide by monocytes is increased in patients with systemic lupus erythematosus. Lupus T cells exhibit mitochondrial hyperpolarization and increased mitochondrial mass, which confer predisposition to necrosis rather than apoptosis in response to repetitive activation and death signals. Exposure of normal T cells to nitric oxide dose-dependently increase the mitochondrial mass and mimic rapid and sustained Ca2+ signal abnormalities observed in lupus T cells. Thus increased mitochondrial biogenesis may account for altered Ca2+ handling and represents novel targets for pharmacological intervention in SLE.

Calcium↗
PubMed2004

Systemic lupus erythematosus and acute pancreatitis: a case series.

The aim of this study was to evaluate whether corticosteroid use is the etiological agent in acute pancreatitis in patients with systemic lupus erythematosus, or whether it is related to the underlying connective tissue disorder. Hospital admissions at Thomas Jefferson University Hospital between 1982 and 2002 that carried the dual diagnosis of systemic lupus erythematosus and pancreatitis were identified, and demographic data, clinical interventions and parameters of clinical progression of their disease were identified. From 2947 admissions with systemic lupus erythematosus 25 (0.85%) were diagnosed as having acute pancreatitis; 76% of cases had active systemic lupus erythematosus on presentation, with an average of 4.4 organ involvement, and a clustering of renal disease (56%), pleural effusion (48%) and arthritis (44%) in these patients. Fifteen patients with active disease and three whose disease was inactive received increased doses of corticosteroids, and four active cases and one inactive one stayed on the same doses. Two inactive patients received no corticosteroids before or after the diagnosis of pancreatitis. Eighty-two percent of patients had clinical and laboratory improvement on the higher or maintenance dose of corticosteroids. We therefore concluded that acute pancreatitis is a rare manifestation of systemic lupus erythematosus, and corticosteroids do not appear to be the etiological agent.

Acute Disease↗
PubMed1996

Lupus erythematosus profundus: a cutaneous marker for a distinct clinical subset?

Sixteen patients with histologically confirmed lupus erythematosus profundus were followed for a decade (on average). At the initial examination, two of 16 (12%) patients fulfilled the 1982 American Rheumatism Association (ARA) criteria for systemic lupus erythematosus (SLE). During the period of observation, SLE developed in another two (12%) patients, both of whom developed a malar rash. The remaining 12 (75%) patients never met the criteria for SLE. Four of the 16 (25%) had no extracutaneous manifestation. In conclusion, most patients with lupus erythematosus profundus have a relatively mild disease course, although a few develop systemic abnormalities and have abnormal laboratory findings.

Adult↗
PubMed1986

Autoimmunity, fetal losses, lupus anticoagulant: beginning of systemic lupus erythematosus or new autoimmune entity with gynaeco-obstetrical expression?

Among the various autoantibodies commonly found in women with systemic lupus erythematosus, the so-called lupus anticoagulant has been described in association with fetal losses. Recently, women with repeated spontaneous abortions and lupus anticoagulant, but no apparent systemic lupus erythematosus have been described. We have studied prospectively the possible connections between fetal losses and autoimmunity in a large control study of 130 idiopathic habitual aborters. These non-pregnant patients without any antecedent autoimmune disease were explored for the presence for autoantibodies especially lupus anticoagulant. Ten percent of the patients exhibit an antithromboplastin antibody (lupus anticoagulant) and half of this group possesses a striking association of biological manifestations of autoimmunity. In conclusion, two points appear: firstly, statistical correlation is demonstrated between antithromboplastin antibody and habitual abortion; secondly, doubt remains about the significance of the association between autoimmunity, fetal losses and antithromboplastin: beginning of systemic lupus erythematosus or new autoimmune entity with gynaeco-obstetrical expression.

Abortion, Habitual↗
PubMed2003

Noninvasive cardiac assessment in children of women with systemic lupus erythematosus.

OBJECTIVE: Noninvasive cardiac assessment of newborns and infants of women with systemic lupus erythematosus. The children had no congenital total atrioventricular block and were compared with the children of healthy women. METHODS: We prospectively assessed 13 newborns and infants aged 1 to 60 days, children of women with systemic lupus erythematosus and without congenital total atrioventricular block. These children were compared with 30 children of women who had no lupus or anti-Ro/SSA antibodies, and no risk factors for congenital heart disease either. Their age groups matched. The following examinations were performed: cardiological physical examination, electrocardiography, echocardiography, and signal-averaged electrocardiography. RESULTS: The statistical analysis showed no significant difference in ventricular function or in the cardiac conduction system between the groups. CONCLUSION: In regard to the conduction system and ventricular function in the absence of total atrioventricular block, no statistically significant difference was observed between the children of women with systemic lupus erythematosus and children of healthy women.

Antibodies, Antinuclear↗
PubMed1995

Cardiac valve involvement in systemic lupus erythematosus and primary antiphospholipid syndrome: lack of correlation with antiphospholipid antibodies.

The aim of this study was to determine the prevalence of cardiac valve disease in systemic lupus erythematosus or in patients with primary antiphospholipid syndrome and to assess the role of the antiphospholipid antibodies as risk factor for endocardial lesions. We studied 39 consecutive patients with systemic lupus erythematosus (mean age 34 +/- 12 years, 38 female and one male), 20 women with primary antiphospholipid syndrome (mean age 32 +/- 4 years) and 20 normal subjects (mean age 35 +/- 8 years, 15 female and five male). All patients with primary antiphospholipid syndrome had increased levels of serum anticardiolipin antibodies and recurrent fetal abortions; some of them also had arterial and/or venous thrombosis and/or thrombocytopenia. M-mode, two-dimensional and Doppler echocardiography were performed in all patients. IgG anticardiolipin antibodies were measured by an enzyme-linked immunosorbent assay. Valvular lesions were observed in 15 patients (38%) with systemic lupus erythematosus. These abnormalities included: mitral valve thickening or vegetation, mitral valve prolapse and aortic valve vegetation; mitral, aortic and tricuspid regurgitation; mitral stenosis. None of the patients with primary antiphospholipid syndrome and of the normal subjects was found to have valvular abnormalities. In systemic lupus erythematosus, high levels of anticardiolipin antibodies were detected in 73% of the patients with valvular lesions and in 67% of the patients without valvular lesions (P > 0.05). We conclude that valvular involvement is frequent in patients with systemic lupus erythematosus but it is apparently unrelated to antiphospholipid autoimmunization.

Abortion, Habitual↗