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PubMedDate not supplied

Subacute cutaneous lupus erythematosus--clinical, histopathological and immunophenotypical study of five cases.

Subacute cutaneous lupus erythematosus (SCLE) is a recently described distinct subset of lupus erythematosus (LE) having characteristic clinical, serological and genetic features. The clinicopathological and serological findings of 5 patients are reported. From a clinical point of view, we describe two types of cutaneous lesions: psoriasiform and annular pattern. ANA were present in 80% of the cases, anti-Ro was present in 60%, and anti-La in 20%; anti-RNP was positive in 1 patient. Circulating immune complexes were detected in 2 patients, and low levels of complement factors (C3, C4) in another 2 patients. The B- cell alloantigens HLA-A2, HLA-Bw4 or HLA-DR3 were present in 60% of the cases. The cutaneous histopathology revealed important changes in the epidermis. Our findings are similar to those described by most other authors. Also, we would like to emphasize the low disease activity and benign course of our patients.

Adult↗
PubMed1999

Haematopoietic stem cell transplantation for active systemic lupus erythematosus.

OBJECTIVE: For patients with systemic lupus erythematosus (SLE) who are at risk of disease-related mortality, we have initiated a protocol of intensive immunosuppression and haematopoietic stem cell support. The first patient enrolled in this study was in the midst of a lupus flare manifest by nephritis and rapidly declining renal function, uncontrolled hypertension, immune-mediated cytopenias, and serositis characterized by a large pericardial effusion and abdominal pain. Antinuclear antibody (ANA), anti-double-stranded (ds) DNA and complement were abnormal. This patient is now more than 1 yr post-stem cell transplant and is taking no immunosuppressive medication. Her serologies are normal, effusions have resolved, blood pressure is normal and renal function is markedly improved. The clinical and serological course of this patient is summarized here. METHODS: Autologous haematopoietic stem cells (HSC) were mobilized with cyclophosphamide (2.0 g/m2) and granulocyte colony-stimulating factor (G-CSF) (10 microg/kg/day). Stem cells were enriched ex vivo using CD34-positive immunoselection and reinfused after immunosuppression with cyclophosphamide (200 mg/kg) and antithymocyte globulin (ATG) (90 mg/kg). RESULTS: White blood cell engraftment with an absolute neutrophil count (ANC) of >500/microl (0.5 x 10(9)/l) and platelet engraftment with a non-transfused platelet count of >20000/microl (20 x 10(9)/l) occurred on day 10 and 14, respectively. Therapy was complicated by a cell lysis-like effect with hyperphosphataemia, hyperuricaemia, normal anion gap metabolic acidosis and transient exacerbation of renal insufficiency. CONCLUSION: This is the first autologous T-cell-depleted haematopoietic stem cell transplantation performed to treat lupus in an active flare. This patient has, for the first time since discase onset (13 yr ago), entered a complete clinical and serological remission which persists at >1 yr of follow-up. The durability of this remission is unknown.

Adult↗
PubMedDate not supplied

Drug-induced cutaneous lupus erythematosus after 5 years of treatment with carbamazepine.

Drug-induced lupus is a disease, inducible by several drugs, that shares symptoms and laboratory characteristics with idiopathic lupus erythematosus. We report a case of carbamazepine-induced cutaneous lupus erythematosus which developed after 5 years of treatment in a patient who was on this medication because of an epileptic crisis after cranial trauma. Carbamazepine is a medication rarely implicated in drug-induced cutaneous lupus, moreover there are very few reports of such long periods between the start of therapy and the presentation of the clinical symptoms. We describe this case to emphasize the possibility of cutaneous lupus induction by carbamazepine even after many years of therapy.

Aged↗
PubMed2005

Investigations and management of gastrointestinal and hepatic manifestations of systemic lupus erythematosus.

Gastrointestinal (GI) manifestations of systemic lupus erythematosus (SLE) are protean. Any part of the GI tract and the hepatobiliary system can be involved. Up to two-third of SLE patients develop GI symptoms at some stage of their illnesses. Clinical presentations of GI lupus are non-specific and can be difficult to differentiate from infective, thrombotic, therapy-related and non-SLE etiologies. Clinical acumen and appropriate endoscopic, biopsy and imaging procedures are essential for establishing the correct diagnosis. Acute abdominal pain in SLE patients can herald an intra-abdominal catastrophe and should be evaluated promptly. Surgical intervention should be instituted without delay if conservative management fails or when there is clinical or radiological suspicion of visceral perforation or intra-abdominal collections.

Diagnostic Imaging↗
PubMed2001

Recurrent pseudotumor cerebri in systemic lupus erythematosus: a case report.

Pseudotumor cerebri is an uncommon manifestation of neuropsychiatric systemic lupus erythematosus (SLE), and is characterized by an elevated intracranial pressure, papilledema with occasional abducens nerve paresis, absence of a space-occupying lesion or ventricular enlargement, and normal cerebrospinal fluid chemical and hematological constituents. Pseudotumor cerebri has been reported in a few sporadic cases in patients with systemic lupus erythematosus. However, the recurrent pseudotumor cerebri in patients with systemic lupus erythematosus which has been rarely reported, has not been reported in Korea. We experienced a 30-yr-old female patient with SLE who was presented with second attack of severe intractable headache. She was diagnosed pseudotumor cerebri twice and successfully treated with corticosteroid. Headache is the common symptom in patients with neuropsychiatric SLE and attributable to various causes. We suggest that it is important to define the cause of headache in patients with SLE and pseudotumor cerebri should be included in the spectrum of clinical manifestations during the course of SLE as a cause of headache.

Adult↗
PubMed1988

Clinical, serologic and immunogenetic studies in patients with chronic cutaneous (discoid) lupus erythematosus who have verrucous and/or hypertrophic skin lesions.

Verrucous and/or hypertrophic lesions occasionally occur in patients with lupus erythematosus. It has been suggested that these patients have skin disease which is characterized by chronicity and resistance to therapy other than intralesional corticosteroids. We studied 8 patients with chronic cutaneous (discoid) lupus erythematosus and 1 patient with papulosquamous subacute cutaneous lupus erythematosus with verrucous and/or hypertrophic lesions. Serologic, immunogenetic, and clinical data were gathered to confirm the distinctiveness of this subset and to seek specific laboratory correlates.

Adrenal Cortex Hormones↗
PubMed2002

How does one assess and monitor patients with systemic lupus erythematosus in daily clinical practice?

Systemic lupus erythematosus (SLE) is a multisystem autoimmune rheumatic disease (ARD) characterized by flares and remissions. SLE has protean and often complex manifestations, necessitating careful clinical assessment. However, it is important to remember that not all clinical problems reported by a lupus patient are due to the disease. Some may be a consequence of therapy and others may be unrelated to lupus. Therefore it is important to understand the totality of the effect of the disease on the patient. In order to do this measures are needed which distinguish current, potentially reversible disease activity, permanent organ damage and the effect of the disease on the patients' health status. Several measures are in current use in clinical trials, but not all are suitable for use in the routine clinical setting. This chapter discusses the current measures available to assess disease activity and damage in SLE.

Health Status↗
PubMed1994

Prognostication of osteonecrosis of the femoral head in patients with systemic lupus erythematosus by magnetic resonance imaging.

To detect and prognosticate osteonecrosis of the femoral head in the preradiographic stage, 60 patients with systemic lupus erythematosus who had normal hip radiology were followed prospectively for a mean period of 5 years (range, 3-7 years) using magnetic resonance imaging (MRI). The first MRI scans showed a low intensity band in the femoral head of normal fat intensity on T1 weighted images in 16 hips of 9 patients. The MRI findings were used to classify the lesions into three categories. Type A (six hips): the lesions occupied the medial one third or less of the weight bearing portion. Type B (two hips): the lesions occupied the medial two thirds or less of the weight bearing portion. Type C (eight hips): the lesions occupied more than the medial two thirds of the weight bearing portion. At the final followup, all of the Type A and one of the Type B hips were classified as being in Stage 1, and one Type B and two Type C hips had progressed to Stage 2. The MRI appearance of six Type C hips had changed from a band to an inhomogeneous pattern with the femoral head progressing to collapse on radiographs 2-5 years after the diagnosis of systemic lupus erythematosus. The remaining hips, which had been classified as normal at the first MRI, maintained a normal appearance, except for one hip that developed a Type A lesion. The presence of a low intensity band on T1 weighted images was an early specific finding of osteonecrosis of the femoral head, and extensive lesions demarcated by band images signified a poorer prognosis in systemic lupus erythematosus patients. If no MRI abnormalities appeared after 1 year of the startup treatment for systemic lupus erythematosus, there was little risk of femoral head collapse based on the subsequent clinical course of the patients followed in the current study.

Adolescent↗
PubMedDate not supplied

Lupus erythematosus in a patient with amyloidosis, adrenal insufficiency, and subsequent immunoblastic sarcoma: demonstration of the LE phenomenon in the lung.

A patient with systemic lupus erythematosus of long duration developed secondary amyloidosis and finally died after the additional complication of malignant lymphoproliferative disease. Multiple system involvement, typical serologic findings, and postmortem evidence substantiated the diagnosis of lupus erythematosus. Amyloid deposition was found in several organs, but was notably extensive in the adrenal cortex. This extensive deposition resulted in adrenal insufficiency, which was diagnosed clinically and treated until the patient's death from lymphoma. The particular nature of the malignant lymphoma is emphasized; a distinctive feature was the disappearance of positive lupus erythematosus cells from the buffy coat and the reduction in titers of relevant serologic tests toward the end of the illness. In spite of this reduction, many hematoxylin bodies and abundant complete lupus erythematosus cells were found in the lungs on postmortem examination.

Adrenal Insufficiency↗
PubMed1996

Clinical effects of intermittent, intravenous cyclophosphamide in severe systemic lupus erythematosus.

To evaluate the clinical effectiveness of intermittent intravenous cyclophosphamide in the treatment of severe systemic lupus erythematosus, 20 patients with systemic lupus erythematosus (SLE) and evidence of severe renal involvement or systemic vasculitis, consecutively admitted to the hospital were studied. Cyclophosphamide was administered intravenously at a dosage of 1.0 g/m2 monthly, during 6 months and maintained every 3 months during 12 additional months. Of 10 patients with active lupus nephritis, a reduction or disappearance of proteinuria and maintenance of normal renal function was recorded in 6. Improvement of renal function was observed in 4 out of 7 patients with renal insufficiency at initial evaluation; resolution of renal insufficiency was more frequently observed in patients with recent onset renal failure. At the end of the follow-up (18.0 +/- 14.5 months) disappearance or reduction of nephrotic range proteinuria was recorded in 6 out of 14 patients; there was progression toward renal failure in 4 patients (20%). Response to intravenous cyclophosphamide therapy was observed in 4 of 5 patients with severe extrarenal SLE. Side effects, recorded in 12 patients, were mild and transient and in no patient was the treatment discontinued. Four patients died during the follow-up, although in 2 of them the deaths were not attributable to therapy. Even though this was an open and uncontrolled study, intermittent, intravenous cyclophosphamide was an effective therapy for severe, steroid refractory SLE.

Adolescent↗
PubMedDate not supplied

How CAM helps systemic lupus erythematosus.

The symptoms associated with systemic lupus erythematosus can vary in location and severity and often have life-altering effects. Because the disease is chronic, the use of traditional medical therapy may not bring about the maximum benefit in many cases. In light of this, many people diagnosed with lupus may turn to complementary and alternative medicine (CAM) therapies to help treat their disease. This article examines some of the common CAM methods used in addition to conventional medical treatment. Some points to consider include effectiveness, safety, cost, and ethical concerns associated with the use of CAM. The integration of these treatments into the medical regimen of those being treated for lupus is also considered.

Complementary Therapies↗
PubMed1988

Surgical excision of trauma-induced verrucous lupus erythematosus.

Verrucous papulonodules are an uncommon manifestation of chronic discoid lupus erythematosus. The differential diagnosis and pathogenesis of this rare entity are discussed. This is the first report of successful surgical excision of trauma-induced lesions of verrucous lupus erythematosus.

Adult↗
PubMed1990

Systemic lupus erythematosus: obstetric and neonatal implications.

Systemic lupus erythematosus (SLE) is a collagen vascular disease that may have a tremendous impact on pregnancy. The pregnant patient with SLE is at increased risk for fetal wastage, intrauterine growth retardation (IUGR), intrauterine fetal demise (IUFD), pregnancy-induced hypertension (PIH), and exacerbations of the lupus process. SLE is an autoimmune disease with tremendous implications for pregnancy. The diagnosis of SLE is based on criteria developed by The American Rheumatism Association. The recent identification of circulating antibodies associated with women who have lupus has led to some confusion. The circulating antibodies are associated with an increased risk of fetal wastage. However, those antibodies have been documented in women who do not have lupus. The diagnosis of SLE and pregnancy requires intensive obstetrical care. SLE may also affect the neonate, from skin lesions to complete heart block. This article describes the effects of SLE on the mother, pregnancy, and the neonate.

Adrenal Cortex Hormones↗
PubMed1985

[Systemic lupus erythematosus. A new look].

The systemic lupus erythematosus is a chronic inflammatory condition in which many abnormalities of the immune system have been described. Recent work has shown a subset of antiphospholipid antibodies which are apparently independent of the classical antinuclear antibodies. Their association with the false positive VDRL and the lupus "anticoagulant" is statistically significant. Clinically, these antibodies are associated with thrombosis, thrombocytopenia, thrombotic and demyelinating neurological features.

Autoantibodies↗
PubMed1982

The pathogenesis of systemic lupus erythematosus.

Many investigators believe that systemic lupus erythematosus is an autoimmune disease, perhaps caused by inadequate suppressor T lymphocyte activity, which permits the activation of autoantibody producing B lymphocytes. This paper discusses the testable hypothesis that a superoxide-generating, chromosome aberration-inducing factor (clastogenic factor), present in the lymphocytes of lupus patients but absent from normals, is responsible for such a suppressor cell defect. Superoxide or activated oxygen species derived from it, such as hydroxyl radical, may be the molecular mediators of CF activity.

Chromosome Aberrations↗
PubMed2006

Abnormal intracellular distribution of NFAT1 in T lymphocytes from patients with systemic lupus erythematosus and characteristic clinical features.

Systemic lupus erythematosus (SLE) presents various clinical features; however, underlying mechanisms remain unclear. In the immunity of SLE, impaired T cell receptor (TCR) signaling and altered cytokine production are in the center of pathogenesis, although, little is known about NFAT (nuclear factor of activated T cells) in lupus T lymphocytes. TCR stimulation activates NFAT1 through Ca2+/calcineurin (Cn) pathway, facilitating nuclear translocation of NFAT1 from cytosol. Therefore, we investigated relationship of disease activity/features and intracellular NFAT1 localization in T lymphocytes from active lupus patients by fractionation. Results showed no significant relationship between disease activity and NFAT1 distribution. However, interestingly, we observed skewed NFAT1 distribution in pellet in patients with active lupus nephritis or pleuritis. In vitro cyclosporin A treatment suggested autonomously activated Ca2+/Cn pathway in lupus T lymphocytes. Considering these results, NFAT1 might be presenting the clinical heterogeneity in SLE.

Adult↗
PubMed1986

Livedoid vasculitis and central nervous system involvement in systemic lupus erythematosus.

Of 66 patients with systemic lupus erythematosus (SLE), 11 (16.7%) had livedoid vasculitis. The sites of predilection included the elbows, knees, fingers, soles, and back, which are not those of common livedoid vasculitis. Livedoid vasculitis developed in ten (90.9%) of the 11 patients with SLE who eventually developed central nervous system (CNS) lupus. In contrast, such preceding livedoid vasculitis occurred in only three (11.5%) of the 26 patients with severe nephrotic lupus nephritis. Livedoid vasculitis did not occur in any patient with neither CNS lupus nor severe lupus nephritis. These results indicate that livedoid vasculitis in SLE is a very important cutaneous manifestation that may possibly be prodromal to CNS lupus.

Adult↗