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[A case of systemic lupus erythematosus associated with multiple intracranial aneurysms].

The author reports a case of systemic lupus erythematosus (SLE) with multiple intracranial aneurysms and subarachnoid hemorrhage. A 31-year-old woman was admitted to the department of internal medicine of Shizuoka General Hospital for the treatment of nephrotic syndrome due to systemic lupus erythematosus on 1984. She spend an uneventful life until April, 1985 when she suddenly complained of severe headache and nausea. The computed tomographic scan revealed subarachnoid hemorrhage and the cerebral angiography showed multiple intracranial aneurysms and stenotic lesion. A review of the literature was made to clarify the incidence, the pathological change, and the prognosis of systemic lupus erythematosus with subarachnoid hemorrhage. The incidence of subarachnoid hemorrhage in SLE was about two percent in the reported clinical cases. The mechanisms of the subarachnoid hemorrhage and the aneurysmal formation in SLE seemed to be due to angitic changes, which involved the vessels of the whole body systematically. The exact prognosis of SLE with subarachnoid hemorrhage is difficult to say, but it seems to be unfortunately poor due to multiplicity of the lesion and the difficulty of its treatment.

Adult↗

Interferon response to dipyridamole in lupus erythematosus patients.

Studies in patients with autoimmune disorders strongly support a role for interferon (IFN) in the disease process. In the present study we investigated the in vivo production of alpha-IFN in lupus erythematosus patients after stimulation with dipyridamole, recently characterized as an alpha-IFN inducer in mice and humans. Levels of IFN were measured in serum samples from 22 patients with systemic lupus erythematosus (SLE) and 12 patients with discoid lupus erythematosus (DLE) before and 24 h after dipyridamole administration. IFN activity was assayed on human and bovine cells in parallel. Initial serum concentrations of alpha-IFN in SLE patients were markedly elevated. The percentage of DLE positive responders to dipyridamole induction was about twice as high as that found for SLE. Studies of factors responsible for IFN production in lupus erythematosus might result in better understanding of the relationship between DLE and SLE.

Dipyridamole↗

Renal amyloidosis associated with systemic lupus erythematosus.

A renal biopsy of a patient who had systemic lupus erythematosus and proteinuria revealed the presence of both amyloidosis and lupus glomerulonephropathy. The association between secondary amyloidosis and systemic lupus erythematosus has only been reported in four previous cases. The significance of this association is discussed.

Adolescent↗

A randomized study of the effect of withdrawing hydroxychloroquine sulfate in systemic lupus erythematosus.

BACKGROUND: The antimalarial drug hydroxychloroquine is thought to be effective in controlling some of the manifestations of systemic lupus erythematosus, but its effectiveness has not been demonstrated conclusively. METHODS: We conducted a six-month, randomized, double-blind, placebo-controlled study of the effect of discontinuing hydroxychloroquine sulfate treatment in 47 patients with clinically stable systemic lupus erythematosus. The patients were randomly assigned to continue their same dose of hydroxychloroquine (n = 25) or to receive placebo (n = 22) for 24 weeks. Ten patients in each group were also taking prednisone. RESULTS: The relative risk of a clinical flare-up, defined as the development of specific clinical manifestations of systemic lupus erythematosus or an increase in their severity, was 2.5 times higher (95 percent confidence interval, 1.08 to 5.58) in the patients taking placebo than in those continuing to take hydroxychloroquine (16 of 22 patients vs. 9 of 25 had flare-ups), and the time to a flare-up was shorter (P = 0.02). The relative risk of a severe exacerbation of disease that required withdrawal from the study was 6.1 times higher (95 percent confidence interval, 0.72 to 52.44) for the patients taking placebo (5 of 22 patients vs. 1 of 25 had severe exacerbations of disease). Changes in the dose of prednisone were not different in the two groups. CONCLUSIONS: Patients with quiescent systemic lupus erythematosus who are taking hydroxychloroquine are less likely to have a clinical flare-up if they are maintained on the drug.

Adult↗

Mitral valve prolapse in systemic lupus erythematosus.

Despite a low incidence of clinical manifestations, autopsy data suggest endocardial and myocardial disease in about 50% of patients with systemic lupus erythematosus. To investigate whether mitral valve prolapse can be considered a clinical manifestation of cardiac involvement in systemic lupus erythematosus, we carried out an echocardiographic study in 51 affected subjects and 102 normals matched for age and sex. Prevalence of mitral valve prolapse was 25% in patients with systemic lupus erythematosus and 9% in healthy controls with a statistically significant difference (p less than 0.01). Neither pericardial effusion nor prolonged (more than 12 months) treatment with corticosteroids were associated with higher prevalence of mitral valve prolapse. Libman-Sacks verrucae on the mitral valve apparatus as well as focal myocardial scars affecting the papillary muscles and adjacent myocardium could be responsible for the development of the valvular dysfunction. We suggest that mitral valve prolapse can be considered a manifestation of cardiac involvement in patients with systemic lupus erythematosus.

Adolescent↗

[New aspects of drug induced lupus erythematosus (author's transl)].

Approximately 8% of patients with systemic lupus erythematosus have drug induced disease. Many drugs are associated with lupus erythematosus-like syndrom (Chart 1). It is also a well known fact that the administration of other drugs may exacerbate corticoid treated systemic lupus erythematosus (Chart 2). This report illustrates that it is obvious that we must learn the relationship of diseases and the drugs which induce antinuclear antibodies.

Adrenal Cortex Hormones↗

[Lupus erythematosus and papules. 4 cases].

Papular skin eruptions are uncommon in lupus erythematosus (LE), and their occurrence may suggest several diagnoses. We report four cases of papular eruptions in LE patients. Two of these patients had purely lupoid papules on acute LE in one and on chronic LE in the other. In the remaining two cases the papules were formed by mucinous deposits that were either secondary to LE or belonged to an associated primary mucinosis. These four cases prompted us to discuss the significance of papular eruptions in lupus erythematosus. LE-specific papular manifestations have a purely lupoid histological and immunopathological substratum. In systemic LE, the frequency of these manifestations varies from one series to another, but they seem to rank fourth after vespertilio, alopecia and photosensitivity. The papules vary in size and number, and they usually complicate a severe and active systemic LE. In chronic LE, a papulo-nodular eruption may be observed, but this is even rarer. Papules occurring in the course of LE may suggest a diagnosis of mucinosis, this disease being either secondary to, or associated with LE. Secondary mucinosis is intricated with LE-specific histological abnormalities. It appears as a symptomatic alcianophilic deposit induced by cytological alterations in some dysimmune collagen diseases, such as dermatomyositis or LE. In the literature, three cases of mucinosis secondary to LE are well documented, and to these we add a fourth case in which the papules contained lupoid lesions and a variable amount of dermal mucinous deposit. The literature has also yielded sixteen cases of primary papular mucinosis associated with systemic LE (12 cases) or chronic LE (4 cases); we add to these a case of systemic LE in which the papular eruptions varied in course, density and size of the papules. As in isolated papular mucinosis, histology regularly shows a copious mucinous deposit. When present, the LE-specific skin manifestations are clearly distinct from those of mucinosis (except for the above-mentioned cases of papular lupus). The course of papular mucinosis usually runs parallel to that of LE which is characterized by its severity and its articular, haematological and renal manifestations. Analysis of the literature also provides various anatomico-clinical elements which help in differentiating between isolated papular mucinosis and primary papular mucinosis associated with LE. In the majority of cases treatment relies on corticosteroid therapy or synthetic antimalarial agents, but the results are irregular since LE seems to be more resistant to treatment than mucinosis.(ABSTRACT TRUNCATED AT 400 WORDS)

Adrenal Cortex Hormones↗

[Detection of lupus anticoagulants in the routine blood coagulation laboratory exemplified by 36 patients with systemic lupus erythematosus].

36 patients suffering from systemic lupus erythematosus (SLE) were subjected to various screening and confirmation tests for the presence of lupus anticoagulants (LA) which are a risk for thrombosis. In five out of the 36 patients (14%) lupus anticoagulants could be found. Five out of the 36 patients (14%) showed increased antiphospholipid antibody (APA) levels whereby only two of these patients were at the same time LA-positive. The specificity, sensitivity and effectiveness of various tests in respect of LA-demonstrability have been assessed and the results taken as the basis for proposal of a largely automated stepwise diagnostic procedure for LA-determination within the routine coagulation laboratory.

Adult↗

Immunological studies of the placenta in systemic lupus erythematosus.

An immunological study was made of the placentae from 5 mothers with lupus erythematosus. 3 of the 5 mothers had anti-DNA antibodies in their sera at the time of delivery and in one of these anti-DNA antibodies were detected in the cord blood. This patient had active renal disease and serological evidence suggestive of circulating immune complexes in her blood at the time of delivery. Immunofluorescence studies showed granular deposition of immunoglobulin and C3 on the trophoblast basement membrane similar to that previously described on the glomerular basement membrane in systemic lupus erythematosus. Anti-DNA antibodies were eluted from the placenta in this case. We suggest that immune complex deposition on the trophoblast basement membrane in patients with active systemic lupus erythematosus may play a part in the increased fetal mortality in this disease.

Adult↗

Neonatal lupus erythematosus in Japan.

BACKGROUND: Although more than 200 cases of neonatal lupus erythematosus (NLE) have been reported, the prognosis, clinical characteristics, and genetic background of the patients are still obscure. Their symptoms seem to vary in different races. OBJECTIVE: We had an opportunity to see two clinically different types of NLE. It is important to define the clinical characteristics in Japanese cases and to compare them to caucasian NLE cases reported earlier. METHODS: Sixty Japanese infants with NLE and their mothers reported in Japan were investigated and compared with cases reported from other countries. RESULTS: Japanese cases were highly associated with anti-SS-A/Ro, anti-SS-B/La, anti-ribonuclear protein, and anti-DNA antibodies. A low frequency of congenital heart block was noted but 8.3% of the cases progressed to systemic lupus erythematosus. HLA-DRw12 was a significantly relative risk in NLE. CONCLUSION: The clinical characteristics of Japanese NLE patients were different from those of caucasian patients reported in the literature.

Adult↗

Pemphigus vulgaris and systemic lupus erythematosus in a 46-y-old man.

Systemic lupus erythematosus (SLE) is an autoimmune disease that may affect many organs in the body. Skin manifestations are frequent and sometimes vesiculobullous lesions may apper such as in bullous lupus erythematosus. SLE may also be exceptionally associated with other blistering diseases such as pemphigus vulgaris (PV). We describe the case of a male diagnosed as having SLE, who one year later developed PV. Only three cases (all in women) of SLE associated with PV have been reported in the literature. Our case is noteworthy in that this exceptional association developed in a middle-aged male.

Humans↗

[Antihistones antibodies in systemic lupus erythematosus, comparison of three assays: Elisa, dot blot and immunoblot].

AIMS: The purpose of our study is to determine and compare the sensitivity of an enzyme linked immunosorbent assay (ELISA), a dot blot assay and an immunoblot assay for the detection of the IgG class antihistones antibodies in a population of systemic lupus erythematosus. The correlation between antihistones antibodies and the main clinical features of SLE or between antihistones antibodies and the presence of anti-double-stranded-DNA antibodies were analysed. MATERIALS AND METHODS: Serum samples from 126 systemic lupus erythematosus patients, classified according to the criteria of the American College of Rheumatology, were analysed for the presence of antihistones antibodies using a dot blot assay and an ELISA. Antihistones subfractions antibodies were assessed using the immunoblot technique on 88 out of the 126 sera. Serum samples from 50 blood-donors were analyzed as negative controls. RESULTS: The sensitivity of antihistones antibodies assessed by dot blot assay and ELISA was 69% and 54% respectively, and was lower than that of anti-double-stranded-DNA antibodies (83%). The sensitivity of the immunoblot assay for the detection of antihistones antibodies was 72%. Incidence of autoantibodies against histones H1, H2 A, H2B, H3 and H4 was 60%, 53%, 48%, 36% and 29.5% respectively. We found a correlation between the presence of antihistones antibodies, detected by the dot blot assay and ELISA, and the presence of anti-double-stranded-DNA antibodies. Antihistones antibodies detected by ELISA were correlated with renal disease in systemic lupus erythematosus; they showed a specificity, a positive and a negative predictive value for renal disease in systemic lupus erythematosus higher than those of anti-double-stranded-DNA antibodies. CONCLUSIONS: The sensitivity of the dot blot assay for the detection of antihistones antibodies is better than that of ELISA, but the latter technique could detect some cases negative by ELISA. Antihistones antibodies detected by ELISA have an important predictive value in the renal complications in systemic lupus erythematosus, better than that of AdsDNA. Antibodies to histone H1 were the most frequent antihistones autoandibodies in systemic lupus erythematosus and they were highly correlated with anti-double-stranded-DNA antibodies and renal disease.

Autoantibodies↗

Temporomandibular joint involvement in systemic lupus erythematosus.

Temporomandibular joint involvement was studied in 37 systemic lupus erythematosus patients not selected with regard to this involvement. One-third had current complaints and two-thirds had a history of severe symptoms. Objective findings indicating systemic lupus erythematosus arthritis were locking or dislocation, tenderness to palpation, and pain on movement of the mandible. Such changes were found in 8 patients (22%) but in none of the healthy controls. Radiographic changes of the condyles, including flattenings, erosions, osteophytes, and sclerosis, were seen in 11 patients (30%), 4 of whom had deformity of other joints as well.

Adult↗

Neonatal lupus erythematosus with multisystem organ involvement preceding cutaneous lesions.

Here we report a case of neonatal lupus erythematosus syndrome presenting with multisystem organ involvement, including anemia, thrombocytopenia, purpura, bloody diarrhea, enzymatic liver abnormalities, splenomegaly and pneumonitis. These findings preceded the cutaneous rash that was the clue for the diagnosis. The patient's mother had an undiagnosed subacute cutaneous lupus erythematosus. The various forms of onset of neonatal lupus erythematosus syndrome are emphasized.

Adult↗

Hormone replacement therapy in postmenopausal women with systemic lupus erythematosus.

Given the female preponderance of systemic lupus erythematosus (SLE) in humans, the adverse effects of female gender and sex hormones in murine lupus, and numerous reports (retrospective, often anecdotal and uncontrolled) describing a temporal association between estrogen exposure and development or exacerbation of SLE, it is easy to accept that estrogens and SLE simply do not mix. While there are valid concerns regarding the use of exogenous estrogens in women with SLE, there are also potential health benefits to be considered. Several salutary effects of postmenopausal estrogens assume particular importance in SLE where the risks of osteoporosis, exaggerated by menopause (natural or cyclophosphamide-induced) and glucocorticoids, are substantial. Moreover, hormone replacement therapy (HRT) is associated with a 40% reduction in the risk of coronary artery disease, higher levels of high-density lipoprotein-cholesterol, and decreased levels of low-density lipoprotein-cholesterol and plasminogen-activator inhibitor type 1, benefits that should be especially applicable to post-menopausal women with SLE. More recent studies, albeit retrospective and absent the use of validated measures of disease activity, suggest that HRT may be well tolerated. In counseling patients regarding lupus and pregnancy, there are now clinical predictors of pregnancy outcome, and patients in remission tend to have good outcomes. The same principles may be true regarding advice on the use of HRT; patients with inactive or stable/moderate disease and at low risk for thrombosis may benefit without a change in lupus activity. Large prospective double-blind placebo controlled studies inclusive of all ethnic groups such as the Safety of Estrogens in Lupus Erythematosus-National Assessment (SELENA) trial should provide the basis for definitive recommendations.

Adult↗

Hypertensive disease in pregnancies complicated by systemic lupus erythematosus.

OBJECTIVE: The purpose of this study was to assess the percentage of hypertensive disease in pregnancies complicated by systemic lupus erythematosus at a single institution. STUDY DESIGN: We conducted a retrospective analysis of medical records between 1992 and 2003 of 68 pregnancies that were complicated by systemic lupus erythematosus from 48 parturients. Patients were categorized into 3 groups: no chronic hypertension (n = 49 women), chronic hypertension-no medication (n = 6 women) and chronic hypertension-treated (n = 13 women). Analyses of variance (with Tukey-Kramer adjusted follow-up evaluation) and chi-squared/Fisher's exact tests were used for the analyses of continuous and categoric variables, respectively. Significance was defined by a probability value of < or = .05. RESULTS: Chronic hypertension complicated 28% of systemic lupus erythematosus pregnancies. Mean systolic blood pressures at intake were significantly different between the normotensive and no chronic hypertension groups and between the chronic hypertension-no medication and chronic hypertension-treated groups; the differences in diastolic pressures reached significance only between the no chronic hypertension and the chronic hypertension-treated groups. Maternal age, gestational age at delivery, birth weight, lowest platelet count, and highest serum creatinine levels were similar between the hypertensive and the nonhypertensive groups. There were no differences in the percentage of aspirin or heparin treatments among the groups, but the percentage of the chronic hypertension-treated group who received steroids was significantly greater than the percentage of women who received steroids in the other 2 groups (P < .05). Preeclampsia developed in 23% of the no chronic hypertension pregnancies and in 32% of the hypertensive pregnancies (P = .54). When pregnancies that were treated with prednisone (n = 34 pregnancies) were compared with those pregnancies that were managed with other agents (n = 34 pregnancies), the percentages of preeclampsia were similar (26% and 24%, respectively; P = .78). CONCLUSION: The percentage of parturients with systemic lupus erythematosus in whom preeclampsia develops is increased, regardless of the presence of underlying chronic hypertension. Prednisone therapy was not associated with a higher risk of preeclampsia in this series.

Adult↗

Hereditary deficiency of C5 in association with discoid lupus erythematosus.

A 29-year-old woman with discoid lupus erythematosus had undetectable classic pathway complement activity. Hypocomplementemia was due to selective deficiency of C5. One of her children was also deficient. To our knowledge this is the first documented case of an association between discoid lupus erythematosus and C5 deficiency.

Adult↗

[Necrotizing vasculitis of the appendix and cecum in systemic lupus erythematosus].

A 20-year old female patient with systemic lupus erythematosus with butterfly-erythema, arthritis, high positive ANA and anti-ds-DNA titres has to have laparatomy because of an acute abdomen. Histological diagnosis is a necrotizing vasculitis of the appendix. In systemic lupus erythematosus an intestinal vasculitis can be found which might be as severe as in polyarteritis nodosa.

Abdomen, Acute↗