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High interleukin-10 production in first-degree relatives of patients with generalized but not cutaneous lupus erythematosus.

BACKGROUND: Preclinical research suggests that interleukin-10 (IL-10) is associated with susceptibility to and severity of systemic lupus erythematosus. Chronic cutaneous lupus erythematosus is thought to be immunogenetically different from systemic lupus erythematosus. We hypothesized that high innate production of IL-10 underlies systemic but not chronic cutaneous lupus erythematosus. METHODS: IL-10 production was determined after endotoxin stimulation of whole-blood samples. In whole-blood samples, disease activity and medication influence the IL-10 production in patients. Therefore, healthy first-degree relatives of patients were evaluated. One hundred sixty-six first-degree relatives of patients with systemic lupus, 50 first-degree relatives of patients with chronic cutaneous lupus, and 133 control persons were studied. Innate IL-10 production of the patient was estimated as the mean IL-10 production of the unaffected relatives. Polymorphisms located -1082, -819, and -592 base pairs relative to the IL-10 gene were typed by allele-specific oligohybridization of polymerase chain reaction-amplified DNA fragments. RESULTS: IL-10 production was higher in the families of patients with systemic lupus than in the control families (1517 +/- 94 vs 1180 +/- 59 pg/mL; P = 0.003). IL-10 production in the families of patients with chronic cutaneous lupus was similar to that in control families (1216 +/- 82 vs 1180 +/- 59 pg/ml; P = 0.74). IL-10 production was also similar in families of patients with severe compared with nonsevere systemic lupus (P = 1.0). The frequency of -1082/-819/-592 haplotypes GCC, ACC, and ATA was similar among patients and compared with the control persons (P = 0.29). CONCLUSIONS: High innate IL-10 production underlies susceptibility for systemic lupus erythematosus but not the severity of the disease. It is not related to chronic cutaneous lupus erythematosus.

Adult↗

Pregnancy in systemic lupus erythematosus.

Fertility is usually normal in systemic lupus erythematosus. However, cyclophosphamide therapy has been associated with an increased risk for sustained amenorrhea in these patients. There is still debate as to whether pregnancy increases systemic lupus erythematosus activity. We have known for decades that systemic lupus erythematosus is associated with an increased risk for pregnancy loss. Now we know that most excess fetal loss in women with systemic lupus erythematosus occurs in association with antiphospholipid antibodies, which also are associated with pregnancy loss in otherwise healthy women. Prematurity, intrauterine growth retardation, and preeclampsia are common features of lupus pregnancy, especially in women with antiphospholipid antibodies. Pregnancy complicated by antiphospholipid syndrome requires expert care and a team approach involving obstetricians, obstetric physicians, rheumatologists, and clinical hematologists. Treatment and close monitoring including uterine artery Doppler scans and timely delivery may improve fetal outcome in these cases. Although there is no evidence that maternal prednisone should be used prophylactically, fluorinated steroids may be efficacious after in utero identification of congenital heart block, especially in fetuses with associated myocarditis.

Antiphospholipid Syndrome↗

Natural killer cell activity of peripheral blood mononuclear cells from patients with various forms of lupus erythematosus.

Natural killer (NK) cell activity of peripheral blood mononuclear cells from 28 patients with cutaneous discoid lupus erythematosus, seven with subacute cutaneous lupus erythematosus, and 17 with systemic lupus erythematosus was studied using a 4 h 51Cr release assay using K-562 cells as target cells. NK cell activity was found to be markedly decreased not only in patients with systemic lupus erythematosus but also in a proportion of cases of subacute cutaneous lupus erythematosus and cutaneous discoid lupus erythematosus (57% and 39% respectively). There was a relationship between the activity and severity of LE and the decrease in NK cell activity.

Adult↗

[Skin manifestations of lupus erythematosus].

There are multiple forms of cutaneous manifestations of lupus erythematosus. In general strictly cutaneous forms are distinguished from systemic lupus erythematosus with cutaneous involvement. Systemic lupus erythematosus can be regarded either as primarily with skin signs or secundarily after initial involvement of the skin. Typical forms of cutaneous lupus erythematosus are the subacute cutaneous and the discoid form.

Clinical Trials as Topic↗

The cutaneous pathology of lupus erythematosus: a review.

The presentation of lupus erythematosus (LE) ranges from a skin rash unaccompanied by extracutaneous stigmata to a rapidly progressive lethal multiorgan disease. The diagnosis and subclassification is traditionally based on the correlation of serological and clinical findings. The latter include a photoinduced skin rash, arthralgia, arthritis, fever, Raynaud's phenomenon, anemia, leukopenia, serositis, nephritis and central nervous sysdtem disease. The conventional classification scheme includes systemic, subacute cutaneous and discoid LE. Recent advances in our understanding of the cutaneous histopathology which correlates with the traditional forms of LE, along with certain novel LE subtypes, are the focus of this review. In addition to the main subtypes of LE, we will discuss associated vasculopathic lesions and the contribution of immunofluorescence microscopy to the diagnosis of LE and related connective tissue disease syndromes. Consideration will be given to unusual variants of LE such as anti-Ro/SSA-positive systemic lupus erythematosus (SLE), bullous SLE, lymphomatoid LE, lupus erythematosus profundus, drug induced LE, linear cutaneous LE, chiblains LE and parvovirus B19-associated LE.

Humans↗

[Pulmonary thromboembolism associated with antiphospholipid syndrome and systemic lupus erythematosus].

The association between systemic lupus erythematosus and antiphospholipid syndrome can cause several complications. We report a 45 years old woman, treated with steroids for a systemic lupus erythematosus. She presented with a clinical picture of a lupus pneumonitis and, subsequently, with the picture of a pulmonary thromboembolism. The diagnosis was confirmed by helicoidal computed axial tomography. The patient was treated with anticoagulants, with a favourable outcome.

Antiphospholipid Syndrome↗

Orbital panniculitis as the initial manifestation of systemic lupus erythematosus.

Inflammation of the fat in lupus erythematosus also known as lupus erythematosus profundus (panniculitis) is a rare clinical entity. As the presenting feature of lupus, it is even more unusual. In this report, we describe an elderly woman who presented with an orbital mass infiltrate as the initial manifestation of lupus that had the classical histological features of lupus panniculitis.

Aged↗

[Cutaneous neonatal lupus erythematosus: discordant expression in identical twins].

BACKGROUND: Neonatal lupus erythematosus is a rare syndrome characterized essentially by cutaneous lesions and/or congenital heart block occurring in infants at birth, or shortly after. It is related to transplacental crossing of maternal auto antibodies (usually anti Ro/SS-A, La/SS-B or rarely anti-U(1) RNP) from the mother to the infant. Mothers of affected children have signs of systemic lupus erythematosus or other collagenosis or are asymptomatic. CASE REPORT: We report a case of neonatal lupus erythematosus in one identical twin, revealed at the age of 3 months by erythematous and annular cutaneous lesions of the face and limbs. These lesions were preceded at birth by an asymmetrical livedo pattern of the lower limbs. Her twin sister was unaffected but both infants had a high rate of anti-Ro/SSA antibodies. The diagnosis of neonatal lupus erythematosus permitted to reveal a biological lupus syndrome in their asymptomatic mother. Cutaneous lesions cleared almost completely within 1 year whereas antiRo/SSA antibodies disappeared. CONCLUSION: Cases of neonatal lupus erythematosus in twins are rare and mostly described in heterozygotic twins. Clinical discordance is usual and could partly be explained by genetic factors. In monozygotic twins, like in our case, chromosome X inactivation could be an explanation of the differences observed.

Antibodies, Antinuclear↗

A secondary amyloidosis associated with systemic lupus erythematosus.

Secondary amyloidosis associated with systemic lupus erythematosus has rarely been reported. A 57-year-old female had been diagnosed as having possible systemic lupus erythematosus, although her clinical course was not typical. About one year after the diagnosis, treatment was begun with prednisolone because of progressive renal dysfunction, thrombocytopenia and low serum levels of complements. Recurrent diarrhea and gastrointestinal bleeding soon developed, then amyloidosis was revealed in the stomach and duodenum. Postmortem examination confirmed systemic amyloidosis. We discuss the significance of this rare association of systemic lupus erythematosus and secondary amyloidosis.

Amyloidosis↗

Cardiopulmonary manifestations of systemic lupus erythematosus.

Nine of 17 patients (52%) with systemic lupus erythematosus participating in a controlled prospective study were found to have maximal mid-expiratory flow rates suggestive of obstructive or obstructive and restrictive pulmonary disease. Cigarette smokers with systemic lupus erythematosus had significantly lower (P less than .05) maximal mid-expiratory flow rates than either non-smokers with systemic lupus erythematosus or smokers with degenerative joint disease. By echocardiography, pericardial thickening was seen in six of 17 patients (41%) and mitral valvular abnormalities were noted in four of 17 (24%) patients with systemic lupus erythematosus. Echocardiography was found to be an effective means of detecting pericardial disease in patients with systemic lupus erythematosus.

Adolescent↗

[Cardiac tamponade disclosing systemic lupus erythematosus].

Cardiac tamponade secondary to systemic lupus erythematosus is rare and has a very serious prognosis. The authors report a case of cardiac tamponade confirmed by echocardiography, which constituted the presenting sign of systemic lupus erythematosus in a 20-year-old patient, who required emergency pericardial aspiration. The diagnosis of systemic lupus erythematosus was established on the basis of the combination of pericardial involvement, non-erosive arthritis, leukopenia with lymphopenia, presence of LE cells and anti-native DNA antibodies and positive antinuclear antibody titre of 1/2560. The clinical course was favourable in response to 3 months of corticosteroid treatment. The possibility of SLE should be considered in any case of cardiac tamponade in a young patient in which the aetiology is not explained.

Adult↗

Childhood systemic lupus erythematosus, vasculitis, and rheumatic fever and neonatal lupus.

Studies on the long-term outcome of patients with systemic lupus erythematosus not only give us survival figures but also uncover flaws in our treatment strategies and reveal both disease-associated and other factors that affect prognosis. Among the latter, compliance with treatment and socioeconomic factors are noteworthy. The pathogenesis of neonatal lupus is under active investigation, and new approaches are being developed. This review also draws attention to a number of vasculitis syndromes that are common in adults but very rarely reported in children. Poststreptococcal reactive arthritis has been described as a new entity; however, such patients should probably receive the same attention as patients with rheumatic fever to avoid recurrences of the disease and cardiac sequelae.

Animals↗

Influenza vaccination of patients with systemic lupus erythematosus.

Twenty-nine patients with systemic lupus erythematosus and 29 control subjects matched for age and prevaccination antibody titer received 200 chick-cell agglutinin units of A/New Jersey/76 HswINI influenza virus vaccine. Serum hemagglutination-inhibiting antibodies were measured before and 4 weeks after immunization. Clinical and laboratory evaluations were done before and 4 and 8 weeks after vaccination. Except for one patient with active lupus erythematosus who developed renal disease, there was no evidence of an increase in lupus erythematosus activity after immunization. Fourteen patients and 18 control subjects had a fourfold or greater increase in antibodies to influenza A/New Jersey/76. Mean postvaccination antibody titer of patients tended to be lower than that of controls (Student's t-test, t = 1.52, 0.05 less than p less than 0.10). Since patients with lupus erythematosus may have increased morbidity and mortality with influenza infections, they should receive influenza immunization even though the magnitude of their antibody response may be less than that of normal persons.

Adult↗

Treatment of systemic lupus erythematosus by immunoadsorption in a patient suffering from tuberculosis.

We report on a 40-year-old man, admitted with fever and weight loss, in whom systemic lupus erythematosus (lupus nephritis World Health Organization type IV) and concomitant acute lung tuberculosis were diagnosed. Conventional treatment of diffuse proliferative nephritis with cytotoxic drugs was thought to be too dangerous in the presence of active tuberculosis. A combination of immunoadsorption and steroids was instituted for the treatment of systemic lupus erythematosus. Antibodies against double-stranded DNA decreased, and proteinuria decreased from 10 g/24 hours to less than 1 g/24 hours. Tuberculosis was treated initially with quadruple-drug therapy, then a triple-drug protocol. Primarily enlarged lymph nodes decreased to normal size after 3 months. The combined treatment modality of steroids and immunoadsorption was effective and safe, even in this patient with active tuberculosis.

Administration, Oral↗

[Differentiation of lupus erythematosus and lymphocytic infiltration].

Lymphocytic infiltration and lupus erythematosus have many features in common. The main distinguishing factor is the lack of epidermal involvement in lymphocytic infiltration, as compared to the frequent presence of epidermal changes in discoid lupus erythematosus. Other factors claimed to differentiate between both conditions are lack of antinuclear antibodies, negative immunofluorescence findings, and the more frequent presence of plasmacytoid monocytes in lymphocytic infiltration. To evaluate the significance of those findings, we studied cases of lymphocytic infiltration and lupus erythematosus with and without epidermal changes, using the same set of clinical, histopathological, and immunological criteria. No differences were found between lymphocytic infiltrates in cases with and without epidermal involvement. Based on those findings, lymphocytic infiltration seems to be a morphological variant of lupus erythematosus, rather than a distinct entity.

Diagnosis, Differential↗

Cavitary pulmonary nodules in systemic lupus erythematosus.

Two cases of systemic lupus erythematosus with cavitary pulmonary nodules as a manifestation are presented. The literature concerning the radiographic manifestations of cardiopulmonary involvement in systemic lupus erythematosus is reviewed.

Adult↗

Primary biliary cirrhosis and systemic lupus erythematosus. A new case report.

Systemic lupus erythematosus and primary biliary cirrhosis are two autoimmune disorders that rarely occur in the same patient. Ten well-documented cases have been reported to date. We add the case of a woman who was diagnosed with systemic lupus erythematosus at 54 years of age then with primary biliary cirrhosis 18 years later. Interestingly, primary biliary cirrhosis antedated the systemic lupus erythematosus in all the other reported cases. The possibility that a patient may develop more than one autoimmune disorder should be borne in mind.

Autoimmunity↗

Coronary arteritis in systemic lupus erythematosus.

Acute myocardial infarction in systemic lupus erythematosus may be due to an atheromatous or arteritic process. Confirmation of the latter etiology has previously been made only at postmortem examination. A 45-year-old white woman with known systemic lupus erythematosus developed anginal pain and multiple episodes of acute myocardial infarction. During this period, there was serologic but no other clinical evidence of active systemic lupus erythematosus. Serial coronary angiographic studies were strongly suggestive of an arteritic process based upon (1) a saccular aneurysm with no obstructive lesions in a coronary artery supplying an area of recent transmural myocardial infarction and (2) the development of significant obstructive lesions in a previously normal coronary artery over a period of 18 days. This case illustrates the difficulties in distinguishing between atherosclerosis and arteritis using a single coronary angiographic study. The distinction is significant because of the different therapeutic interventions required.

Acute Disease↗