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PubMed1990

Liver disease in neonatal lupus erythematosus.

We report the cases of neonatal lupus erythematosus associated with significant hepatic involvement in three living infants and in one infant who died 3 hours after delivery. The three living infants had neonatal cholestasis as a major component of their clinical findings. Pathologic changes included giant cell transformation, ductal obstruction, and extramedullary hematopoiesis. Liver involvement has been noted incidentally in children with neonatal lupus erythematosus, but it has generally been attributed to hemodynamic compromise as a result of congenital heart block or systemic toxic reactions. We speculate that neonatal hepatitis proceeding to hepatic fibrosis may occur in neonatal lupus erythematosus, analogous to the occurrence of "idiopathic" congenital heart block. The neonatal hepatitis associated with neonatal lupus erythematosus is a form distinguishable from the "idiopathic" group. Liver involvement may be more common than was previously recognized, and prospective studies to look for maternal autoantibodies in idiopathic neonatal liver disease should be undertaken.

Antibodies, Antinuclear↗
PubMedDate not supplied

Pleural effusion in systemic lupus erythematosus.

Three cases of systemic lupus erythematosus (SLE) with pleural effusion are reviewed. The characteristics of the effusions are presented, and the literature pertaining to lupus-related effusions is reviewed. There is great heterogeneity in lupus pleural effusions. The presence of polymorphonuclear neutrophils as the predominant white cell, while consistent with SLE, should raise the possibility of complicating bacterial infection. The presence of lupus erythematosus (LE) cells in the fluid seems to be the most specific diagnostic criterion.

Complement System Proteins↗
PubMed1981

Cutaneous bullae following acute steroid withdrawal in systemic lupus erythematosus.

A patient with systemic lupus erythematosus developed severe widespread bullae following sudden steroid withdrawal. Histologically the lesion was suggestive of leukocytoclastic vasculitis with a regenerating subepidermal bulla, but immunofluorescence on non-lesional skin was typical of lupus erythematosus. Treatment led to resolution of the bullous eruption, and despite exacerbations of the patient's lupus nephropathy, the blisters have not recurred. The differential diagnosis of bullous LE is discussed.

Blister↗
PubMed1995

Systemic lupus erythematosus in males.

Systemic Lupus Erythematosus is a disease commonly seen in women. A few male kindreds have however been described. In this study, twelve male patients of a series of 175 patients with SLE have been analysed. Arthritis was the most frequent manifestation observed. Renal involvement was seen in as many as 41.65% of patients.

Adolescent↗
PubMed2006

Abdominal manifestations in childhood-onset systemic lupus erythematosus.

BACKGROUND: Childhood-onset lupus erythematosus is a rare disorder of unknown origin. OBJECTIVES: To describe the frequency of gastrointestinal manifestations at presentation of systemic lupus erythematosus SLE and at follow-up, and discuss the specific causes of these manifestations. METHODS: Medical records of 201 patients with childhood-onset SLE followed up in French paediatric nephrological, haematological and rheumatological centres were reviewed and abstracted for gastrointestinal manifestations. RESULTS: Gastrointestinal involvement was recorded in 39 (19%) children. The median (range) age at the time of initial gastrointestinal manifestations was 11.3 (4.5-16) years. Gastrointestinal symptoms were present at or occurred within 1 month after diagnosis in 32% patients. Abdominal pain was the most frequent symptom, present in 34 (87%) patients. It was mostly related to lupus involvement, especially ascites (n = 14) and pancreatitis (n = 12), more rarely to treatment-induced events (n = 1) or infection (n = 1) and never to events unrelated to SLE. Three children with surgical abdomen underwent a laparotomy before SLE was diagnosed, with a final diagnosis of lupus peritonitis and lupus acalculous cholecystitis. C reactive protein values were <40 mg/l in all but two patients who had surgical abdomen. Abdominal ultrasonography and computed tomography scans were abnormal in 58% and 83% of the evaluated patients, respectively. Corticosteroids, associated with intravenous cyclophospamide in eight patients, led to complete remission of gastrointestinal involvement in 30 of 31 treated patients. CONCLUSION: Gastrointestinal involvement is common in children with SLE, and is mainly due to primary lupus involvement. Corticoidsteroid treatment should be promptly considered in children with lupus presenting with abdominal pain after infectious disease; side effects of treatment and intestinal perforation have been excluded.

Abdomen, Acute↗
PubMed2005

The ananti-alpha-actinin test completes ananti-DNA determination in systemic lupus erythematosus.

In murine systemic lupus erythematosus (SLE) models, nephritogenic anti-dsDNA IgG has been shown to cross-react with a kidney antigen, alpha-actinin, and to be critical in renal pathogenesis. In humans, studies of anti-alpha-actinin antibodies (Abs) are scarce, and these antibodies remain to be evaluated. We have thus far tested sera from patients with SLE (n = 103), rheumatoid arthritis (RA, n = 93), and primary Sjögren syndrome (pSS, n = 34), and from healthy subjects (n = 160), for the presence of anti-alpha-actinin and anti-DNA Abs. The latter were tested using several methods [IIF on Crithidia luciliae (Crit) and ELISA using dsDNA]. Anti-alpha-actinin Abs were confirmed by Western blot. Sera from 23 of 103 SLE patients, 3 of 93 RA patients, 1 of 33 pSS patients, and 1 of 160 controls scored positive for anti-alpha-actinin Abs. In SLE, the positivity was significantly associated with anti-dsDNA reactivity (22 of 23): 19 of 23 sera were alpha-actinin-positive/dsDNA-positive and 13 were alpha-actinin-positive/Crit-positive. Few cases were alpha-actinin-positive/dsDNA-negative: 1 SLE, 3 RA, and 1 control. Furthermore, anti-alpha-actinin Abs have been detected at high level before or at the early stage of lupus nephritis when compared with active and inactive SLE without kidney manifestations.

Actinin↗
PubMed1989

Brainstem auditory evoked potentials with increased stimulus rate in patients suffering from systemic lupus erythematosus.

Central nervous system involvement in systemic lupus erythematosus is frequently occult, may be the presenting sign, and is a bad prognostic indicator. At present, there is no reliable, sensitive laboratory test for the evaluation and diagnosis of subclinical central nervous system involvement of the disease. Brainstem auditory evoked potentials with and without increased stimulus rate have been used to diagnose ischemic lesions in the central nervous system. Brainstem auditory evoked potentials, with and without increased stimulus rate, was used to investigate 15 systemic lupus erythematosus patients, 20 normal participants, and 5 patients receiving corticosteroids for bronchial asthma. A significant statistical difference was found in the net effect of increased stimulus rate in comparisons of the systemic lupus erythematosus patients with the normal group. Brainstem auditory evoked potentials, with increased stimulus rate, demonstrated subclinical involvement of the central nervous system in systemic lupus erythematosus, reinforcing the notion that increased stimulus rate measures are sensitive to ischemic changes, in this case, even in neurologically asymptomatic patients.

Acoustic Stimulation↗
PubMed1975

Immunohistological studies, with anti-connective tissue and anti-immunoglobulin antisera, of the skin in lupus erythematosus and scleroderma.

Skin lesions from six patients with systemic lupus erythematosus, five patients with discoid lupus erythematosus, twelve patients with systemic sclerosis, five patients with localized morphoea and twenty controls were examined by immunohistological techniques using fluorescein-labelled anti-human IgG, anti-human C3 and anti-human renal glomerulus antisera. The major immunohistological changes in systemic sclerosis and in localized morphoea consisted of foci of intercollagenous staining for connective tissue antigens in the reticular layer of the dermis. It is suggested that these findings indicate collagen neogenesis. In lupus erythematosus the major changes occur in the dermo-epidermal junction and consist of deposits of IgG and C3 and thickening and disruption of the membrane as demonstrated by the use of heterologous sera containing antibasement membrane antibodies. Immunohistological techniques are useful in the diagnostic differentiation between scleroderma and lupus erythematosus.

Antibodies, Anti-Idiotypic↗
PubMed1990

Functional asplenia in systemic lupus erythematosus.

A patient with inactive systemic lupus erythematosus was successfully treated for pneumococcal sepsis complicated by disseminated intravascular coagulation, shock, renal failure, and functional asplenia. Functional asplenia was diagnosed from the total absence of uptake of intravenously administered 99mtechnetium-labeled sulfur colloid. Ten similar cases of functional asplenia occurring in patients with systemic lupus erythematosus were noted in a review of the literature. Six of these cases, including the current report, were complicated by pneumococcal (5) or salmonella (1) sepsis. The patient presented here had an excellent antibody response to pneumococcal vaccination. Spleen scan abnormalities fully reversed at 1 year. Although functional asplenia is a rare event in systemic lupus erythematosus, it appears to predispose to severe septic complications.

Acute Kidney Injury↗
PubMed2002

Functional melanonychia due to involvement of the nail matrix in systemic lupus erythematosus.

Longitudinal melanonychia is an unusual finding in systemic lupus erythematosus. We report the case of a patient of Arabic extraction with cutaneous lesions of lupus erythematosus on the cheek and chest and longitudinal melanonychia affecting several fingers. Results of histologic examination of the skin and direct immunofluorescence analysis confirmed the diagnosis of systemic lupus erythematosus. The distal matrix revealed changes suggestive of systemic lupus erythematosus accompanied by increased activity of melanocytes but without melanocytic hyperplasia. In this case, longitudinal melanonychia associated with systemic lupus erythematosus was of functional origin.

Adult↗
PubMed1997

Nodular regenerative hyperplasia of the liver in systemic lupus erythematosus. The relationship with anticardiolipin antibody and lupus anticoagulant.

Recent reports have indicated that nodular regenerative hyperplasia (NRH) associated with systemic lupus erythematosus (SLE) is related to anticardiolipin antibodies (aCL) and/or lupus anticoagulant (LA). We describe a patient with SLE complicated by NRH, who did not show neither aCL nor LA activity. This case suggests that the pathogenesis of NRH in patients with autoimmune diseases is heterogeneous and not confined to aCL and LA.

Adult↗
PubMed2003

[Classification of dermatologic manifestations in lupus erythematosus].

Various dermatologic manifestations are observed in the different lupus subsets. Lupus lesions are characterized by a dermo-epidermal dermatitis. Other lesions, vascular or non vascular, are essentially present in association with systemic lupus erythematosus. Acute, subacute and chronic lupus erythematosus are distinguishable according to their clinical aspects, pathological features and evolution. Acute lesions are either localized to the midface or widespread. Subacute lesions may be annular or psoriasiform. Chronic lupus erythematosus includes localized or widespread discoid lupus, lupus tumidus, chilblain lupus and panniculitis. Therapy of cutaneous lupus is mainly based on antimalarials and avoidance of sun irradiation. In refractory cutaneous lupus, no universal guidelines are currently available. Except for acrosyndromes and urticaria-like lesions, vascular lesions may be due to vasculitis or thrombosis. An accurate diagnosis is absolutely necessary since therapy is different in thrombosis and vasculitis. Non vascular and non lupus lesions are numerous, some of them require special treatment such as dapsone for bullous lupus.

Acitretin↗
PubMed1993

The effect of hormonal changes on cutaneous disease in lupus erythematosus.

The behaviour of cutaneous disease in systemic lupus erythematosus (SLE) and discoid lupus erythematosus (DLE), under the influence of various hormonal states, was studied in 68 patients. In 28 pregnancies, cutaneous disease was essentially unchanged. In a total of 57 patients whose lupus erythematosus (LE) had been diagnosed prior to the menopause, 20% described a premenstrual cutaneous exacerbation. Only three patients had taken an oestrogen-containing contraceptive. The duration of oral contraceptive treatment before the onset of lupus varied: 1 month in a patient presenting with the acute malar rash of SLE, 2 months in a patient who presented with annular weals and later developed systemic features, and 12 months in a patient who developed generalized DLE. Thirty-three patients were menopausal at the time of the study; 4% had noticed a perimenopausal cutaneous flare. There was no deterioration in the skin of the five patients on hormone replacement therapy.

Adolescent↗
PubMed1997

Autoantibodies to evolutionarily conserved epitopes of enolase in a patient with discoid lupus erythematosus.

Although the pathology of discoid lupus erythematosus is well documented the causative agents are not known. Here, we report the identity of the target antigen of an autoantibody present in high titre in the serum of a patient with discoid lupus erythematosus. We have demonstrated that the antigen is enolase; first, because it has properties consistent with this glycolytic enzyme (47,000 MW, cytosolic localization and ubiquitous tissue distribution). Secondly, limited amino acid sequence determination after trypsin digestion shows identity with alpha-enolase. Finally, the autoimmune serum immunoblots rabbit and yeast enolase and predominantly one isoelectric form of enolase (PI approximately 6.1). These results indicate that the reactive autoepitopes are highly conserved from man to yeast. The results also suggest that the autoantibodies are most reactive to the alpha-isoform of enolase, although it is possible that they may also be reactive with gamma-enolase, and have least reactivity to beta-enolase. The anti-enolase autoantibodies belong to the immunoglobulin G1 (IgG1) isotype. This is the first report of IgG1 autoantibodies to evolutionarily conserved autoepitopes of enolase in the serum of a patient with discoid lupus erythematosus. Previous reports of autoantibodies to enolase have suggested associations with autoimmune polyglandular syndrome type I and cancer-associated retinopathy. This report and an earlier report of what is likely to be enolase autoantibodies in two patients without systemic disease suggest that enolase autoantibodies have a broad association and are not restricted to any particular disease.

Aged↗
PubMed2000

[Successful management of systemic lupus erythematosus with IgM enriched immunoglobulins].

Systemic lupus erythematosus is a potentially life-threatening disease. Even the use of potent immunosuppressive drugs often barely controls the disease. In addition, treatment-related side effects are common due to the high doses of immunosuppressive drugs necessary. We report on two cases of acutely exacerbated systemic lupus erythematosus which were difficult to control with prednisone and cyclophosphamide. Both patients were additionally treated with IgM-enriched preparations of intravenous immunoglobulins. In both cases, the doses of the immunosuppressive drugs used could be significantly reduced. After nine treatment cycles, patients remained disease-free for a follow-up period of one year with only minimal immunosuppressive therapy. Our observation shows that IgM-enriched immunoglobulins can be used for therapy of systemic lupus erythematosus.

Adult↗
PubMed1990

Disseminated cutaneous and synovial Mycobacterium marinum infection in a patient with systemic lupus erythematosus.

A patient with systemic lupus erythematosus had a protracted skin infection with Mycobacterium marinum after a puffer fish sting. Disseminated cutaneous and synovial disease was associated with clinically active systemic lupus erythematosus two years after the initial infection. The infection was poorly responsive to multiple antituberculous regimens. Hematogenous spread of infection was the likely route of dissemination.

Adult↗