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[Oral lupus erythematosus. Description and analysis of 11 cases].

BACKGROUND: The aim of this study is to evaluate all the cases of lupus erythematosus with oral involvement coming to the Department of Oral Medicine in the last 15 years (1984-1998). METHODS: The files of the Oral Medicine Department of the University of Turin have been reviewed to search oral manifestations of lupus erythematosus according to international accepted diagnostic criteria. RESULTS: A total of eleven patients 7 women (64%) and 4 men (36%) with a mean age of 46 years were included. Three patients (27%) had discoid lupus erythematosus (LED) whereas 8 (73%) had systemic lupus erythematosus (LES). In four patients the diagnosis of lupus was made after oral diagnosis, whereas 7 patients (64%) had a previous diagnosis of lupus. Clinical aspect was typical in 8 cases (73%) while one patient had leukoplakia-like white patches and two patients (18%) had lichen-like oral lesion. CONCLUSIONS: This study suggest that primitive oral lesion of lupus are quite rare but frequently may have atypical features often difficult to differentiate from lichen planus.

Adult↗

Unusual eye manifestations in systemic lupus erythematosus patients.

One hundred and twelve records of patients with systemic lupus erythematosus were reviewed and four cases with unusual ocular manifestations are described. We found that anterior uveitis is not an uncommon manifestation of systemic lupus erythematosus and physicians must be aware of it during the patient's evaluation, since it can be treated without serious visual loss. Optic neuritis is uncommon in systemic lupus erythematosus and visual loss may be permanent despite therapy.

Adult↗

Systemic lupus erythematosus and pregnancy.

PURPOSE OF REVIEW: Pregnancy in patients with systemic lupus erythematosus is associated with a high risk of maternal disease exacerbation and adverse fetal outcome. This review summarizes recent published findings on lupus pregnancy. RECENT FINDINGS: The literature is in agreement that for most women with inactive and stable systemic lupus erythematosus, pregnancy is safe for both mother and fetus. The main risk factors for adverse pregnancy course and outcome are active disease, nephritis with proteinuria, hypertension, and maternal serum antibodies to SS-A/Ro, SS-B/La, cardiolipin, beta2-glycoprotein I, and lupus anticoagulant. Recent studies have broadened our understanding of the immunological mechanism underlying congenital heart block induced by anti-Ro/La antibodies. In addition, the approach to oral contraceptives has been modified on the basis of two well controlled studies suggesting that they do not cause exacerbation of inactive or mild disease. SUMMARY: Pregnancy in patients with systemic lupus erythematosus is safe and manageable provided the disease is stable. Patients should be evaluated before pregnancy for pregestational risk factors and be closely followed during pregnancy. In most cases of lupus flare during pregnancy, the disease can be safely managed.

Autoantigens↗

[Silent myocardial infarct as a main manifestation of systemic lupus erythematosus].

Myocardial infarction has rarely been reported in patients with systemic lupus erythematosus but may develop late in the disease usually as a result of severe and accelerated atherosclerosis or coronary arteritis. A 32-year-old man with untreated and unrecognized systemic lupus erythematosus, in the absence of conventional coronary risk factors (except family predisposition) and definite extracardiac manifestations of systemic lupus erythematosus had a silent myocardial infarction early in the course of the disease. A coronary arteriogram revealed multiple stenosis of the left anterior descending artery and critical stenosis of the right coronary artery. It is our belief that lupus vasculitis is a likely contributing factor in the development of obstructive coronary disease in this patient.

Adult↗

Clinical features of systemic lupus erythematosus.

Descriptive and analytic clinical research investigations dealing with systemic lupus erythematosus have focused on disease manifestations involving the heart, kidneys, and central nervous system, the complications of corticosteroid therapy, particularly cardiovascular and peripheral vascular disease, and outcomes of pregnancy. Studies exploring prognostic factors in patients with lupus nephritis as well as risk factors for the development of cardiovascular disease emphasize the importance of control of comorbid conditions, particularly hypertension and hyperlipidemia, in preventing poor outcomes in patients with systemic lupus erythematosus. The effectiveness of parenterally administered pulse cyclophosphamide has been demonstrated in patients with severe central nervous system involvement, and the effectiveness of plasmapheresis in patients with thrombotic thrombocytopenic purpura was reviewed.

Arthritis↗

[Primary central nervous system lymphoma in a patient with systemic lupus erythematosus].

A 36 year-old woman with systemic lupus erythematosus was admitted to our hospital with headache, brachiocrural hemiparesis and hemianesthesia. She had been treated with prednisone and cyclophosphamide. CT scan and MRI revealed a 15 mm nodular mass enhanced with gadolinium in left frontal convexity. CNS biopsy was performed and a diffuse large B-cell lymphoma was diagnosed. She was treated with radiation therapy without response and died. There are few reports of erythematosus systemic lupus associated with primary central nervous system lymphoma.

Adult↗

Aortic occlusion in a patient with antiphospholipid antibody syndrome in systemic lupus erythematosus.

A 41-year-old woman with systemic lupus erythematosus and lupus anticoagulant developed aortic thrombosis. The patient was receiving low dose aspirin. Aortography showed a complete obliteration of the infrarenal aorta. Computed tomography and magnetic resonance imaging showed no evidence of aortitis. The patient improved with medical treatment. Six other published cases are reviewed.

Adult↗

Strain differences in binding properties of estrogen receptors in immature and adult BALB/c and MRL/MP-lpr/lpr mice, a model of systemic lupus erythematosus.

The aim was to compare binding properties of estrogen receptors in brain, reproductive and immune tissues of immature and adult female BALB/c mice, and in the same tissues of MRL/MP-lpr/lpr mice. The latter strain spontaneously develops an autoimmune disease resembling human systemic lupus erythematosus (lupus; SLE). It is hypothesized that estradiol, through its receptors, mediates the progression of murine SLE. High-speed cytosols were prepared from hypothalamus, spleen, thymus and uterus of both strains, and incubated with the synthetic estrogen (3)H-moxestrol (NEN). Scatchard plots were derived from binding isotherms obtained after in vitro incubation. In addition, cervical lymph nodes from MRL mice could be used, but were too small in BALB/c mice. There was a significant increase in the affinity of the binding reaction i.e. a decrease in the apparent molar dissociation constant (Kd), in immune tissues and uterus with maturation in MRL but not BALB/c mice, whose tissues had, overall, a lower affinity for (3)H-moxestrol. Receptor concentrations were significantly higher in spleen and cervical lymph nodes of adult compared with immature MRL mice, but the opposite pattern was observed in BALB/c mouse spleen on maturation. These properties of estrogen receptors in MRL mice may underlie estrogen-mediated exacerbation of murine SLE.

Age Factors↗

Abnormal laboratory test results and their relationship to prognosis in discoid lupus erythematosus. A long-term follow-up study of 92 patients.

Investigation of 92 patients with discoid lupus erythematosus, manifested initially by localized cutaneous lesions only, showed abnormal laboratory test results for 57 patients (62%) on admission and for 62 patients (67.4%) on review 16 to 20 years later. Patients with discoid lesions confined to the head and neck (DLE) showed fewer laboratory abnormalities than those patients with disseminated lesions involving trunk and limbs (disseminated discoid lupus erythematosus [DDLE]). Systemic lupus erythematosus (SLE) eventually developed in six (6.5%) of the patients, and all had shown persistent multiple abnormal laboratory findings from the beginning. Complete remission occurred in 46.7%. A persistent positive antinuclear factor of either speckled or homogeneous pattern with a titer greater than 1:50, leukopenia, thrombocytopenia, or a false-positive Wassermann reaction indicated those patients who may progress to DDLE or SLE.

Antibodies, Antinuclear↗

[Cyclosporin therapy of patients with systemic lupus erythematosus].

Seven patients suffering from systemic lupus erythematosus (SLE) were treated by cyclosporin A (CSA) plus corticosteroids. The indications for treatment were: lupus nephritis refractory to treatment with corticosteroids and cyclophosphamide (one patient), nephrosis syndrome or lupus nephritis associated with severe cytopenia and refractory to corticosteroid treatment (5 patients), severe thrombocytopenia refractory to conventional treatments (one patient). The treatment for 6 months of this latter patient resulted in complete remission, lasting for at least 1.5 years. Patients with kidney involvement also responded to therapy. The response manifested within 1 month. Treatment lasted for a mean of 5.1 months (the treatment of one patient was interrupted after 1 month because of lack of cooperation). There were no side effects. CSA treatment in combination with corticosteroids, when properly indicated, may be beneficial in SLE.

Adrenal Cortex Hormones↗

Lupus erythematosus tumidus: a series of 26 cases.

OBJECTIVE: To study 26 cases of lupus erythematosus tumidus (LET), a subset of chronic cutaneous lupus erythematosus (CCLE), referred to in the literature as a rare entity. PATIENTS AND METHODS: A retrospective study was conducted of 26 patients diagnosed with LET between 1996 and 2002. The clinical characteristics, histopathologic and laboratory findings, response to treatment, association with other subsets of lupus, course, and diagnostic criteria were analyzed. RESULTS: The incidence by sex was similar. The mean age of presentation was 49.19 years. The clinical presentation usually involved erythematous, edematous plaques located on the face, chest, back, or extremities, related to sun exposure. A dermal lymphocytic infiltrate with a perivascular disposition and differing degrees of mucin deposition was observed in all cases. Minimal epidermal changes were present in 18 cases, and 11 of these also showed minimal dermal-epidermal changes. Only one case showed dermal-epidermal changes without any epidermal alteration. Direct immunofluorescence test was performed in 15 patients, and 11 were negative. All cases showed a benign course without systemic manifestations. The response to topical steroids or antimalarial treatment was excellent, but a seasonal recurrence was usually observed. Discussion No defined criteria for LET are universally accepted. The main controversies are the acceptance of LET as a separate subset of CCLE, and the histopathologic diagnostic features, mainly the presence or absence of epidermal and dermal-epidermal changes in these lesions. CONCLUSIONS: No inflexible histologic criteria should be employed for the diagnosis of LET. This subset of lupus erythematosus is characterized by intense photosensitivity, definite clinical lesions, a benign course, the absence of systemic disease, good response to antimalarial treatment, and a tendency to recur. More studies should be performed in order to establish the true incidence of LET because this subset of CCLE is probably underestimated.

Adolescent↗

Third International Conference on Systemic Lupus Erythematosus.

A Fourth International Conference on Systemic Lupus Erythematosus will be held in Jerusalem in March 1995. We look forward to a better understanding of the molecular, genetic, immunologic, and clinical aspects of the disease and trust that this knowledge will result in improved treatment.

Animals↗

The American College of Rheumatology response criteria for systemic lupus erythematosus clinical trials: measures of overall disease activity.

OBJECTIVE: Improved standards for the evaluation of therapeutic interventions in systemic lupus erythematosus (SLE) are needed. The purpose of this study by a committee of the American College of Rheumatology was to define clinically meaningful improvement, no change, or worsening in 6 existing clinical measures of SLE disease activity. This represents an important step in a disease in which some organ symptoms get better and others get worse. It is intended to help investigators develop sample size estimates based on meaningful effect sizes and to gauge the clinical relevance of any observed change in disease activity. METHODS: Medical records from 310 patients drawn from 3 sources were abstracted into a standard format. Each vignette included clinical and laboratory data obtained during 2-3 visits. Ratings on the following 6 instruments were obtained for the same patients during the visit or retrospectively: the British Isles Lupus Assessment Group (BILAG), the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), the revised Systemic Lupus Activity Measure (SLAM-R), the European Consensus Lupus Activity Measure (ECLAM), the Safety of Estrogens in Lupus Erythematosus: National Assessment (SELENA)-SLEDAI, and the Responder Index for Lupus Erythematosus (RIFLE). From this pool of vignettes, 5 common vignettes and 10 randomly selected vignettes were rated through a secure Web site by 88 international experts on SLE. The experts, who were blinded to the activity measure scores, were asked to rate each patient's clinical condition as worsened, improved, or unchanged relative to the previous visit. These ratings were transformed by statistical procedures into performance characteristic curves that related a change on a particular SLE activity measure to the physicians' agreement on whether that patient had worsened, improved, or remained the same clinically. These were discussed by the committee members, who were blinded to the actual instrument used. The committee then voted on what level of expert agreement would be used to determine clinically meaningful change. RESULTS: The physician ratings on the 5 common vignettes revealed considerable variation in their clinical appraisals. Overall, the 6 SLE activity measures showed excellent separation of clinical conditions as being worsened, improved, or the same. The committee voted to take 70% agreement by physicians as the point on the performance characteristic curves at which meaningful change in a score could be identified. For each instrument, we computed the units of change required to indicate improvement or worsening. CONCLUSION: To our knowledge, these are the first response criteria in any disease where a clinically relevant change has been determined a priori and mapped to standardized measures. This criterion should aid the clinical evaluation of new therapies, improve comparability between trials, and facilitate innovative trial designs.

Clinical Trials as Topic↗

Acebutolol-induced subacute cutaneous lupus erythematosus.

Beta-blocking medications are rarely associated with drug-induced lupus erythematosus syndrome and have never been incriminated as a cause of subacute lupus erythematosus (SCLE). We present herein the first case of SCLE induced by acebutolol. A 57-year-old woman presented with a 1-month history of a cutaneous eruption of the photo-exposed areas. One month ago, the patient had started a treatment with oral acebutolol to cure a hypertension of 1-year evolution. Physical examination revealed erythematous scaly annular plaques, involving the face, arms and trunk. Immunologic serology findings revealed a positive titer of antinuclear antibodies up to 1/1,280 with positivity of antihistone and Ro/SSA antibodies. Acebutolol was stopped, and the lesions cleared completely 4 months later. Literature data, along with our case, suggest a link between acebutolol therapy and the onset of a lupus syndrome. Although this is the first report of acebutolol-induced SCLE, we should be aware of this occurrence, and avoidance of acebutolol is recommended in patients with stigmata of lupus erythematosus.

Acebutolol↗

The contribution of direct immunofluorescence to the diagnosis of lupus erythematosus.

The contributions of direct immunofluorescence, light microscopy and serology to the investigation of 52 patients with possible lupus erythematosus were studied. Direct immunofluorescence was found to be most valuable in the investigation of possible systemic lupus erythematosus whilst only of supportive value in the investigation of possible chronic discoid lupus erythematosus. The importance of performing light microscopy before direct immunofluorescence in cases of possible chronic discoid lupus erythematosus is shown.

Biopsy↗

Acute pulmonary edema as the initial hospital presentation of systemic lupus erythematosus.

Acute pulmonary edema is an unusual initial presentation for systemic lupus erythematosus. A 46-year-old woman required intensive care for life-threatening pulmonary edema of unknown etiology, which was unresponsive to conventional treatment. Her condition improved only when pulse corticosteroid therapy was initiated, with clinical and echocardiographic improvement in cardiac function. The diagnosis of systemic lupus erythematosus was then made, based on immunologic tests and renal biopsy. The patient's condition remained stable only with continuation of appropriate therapy for systemic lupus erythematosus.

Acute Disease↗

Severe thrombocytopenic purpura due to rubella infection in a patient with systemic lupus erythematosus.

We report the case of a woman suffering from systemic lupus erythematosus who developed a severe thrombocytopenic purpura (platelet count < 1 x 10(9)/l) secondary to rubella infection. The search for antiplatelet antibodies revealed transient circulating anti-GPIIb-IIIa and anti-GPIb-IX platelet antibodies. After a few weeks, bound anti-GPIIb-IIIa antiplatelet antibodies were still detectable and they persisted several months after recovery, probably in relation to a mild autoimmune thrombocytopenia related to systemic lupus erythematosus. To our knowledge, this is the first case report of severe thrombocytopenic purpura due to rubella in an adult with systemic lupus erythematosus.

Adult↗