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Cardiac tamponade as the first manifestation of systemic lupus erythematosus in the elderly.

Although pericardial involvement is very frequent in systemic lupus erythematosus, cardiac tamponade is extremely rare as the first manifestation of the disease. On the other hand, systemic lupus erythematosus is a disease that predominantly affects young women, and it is a very uncommon condition in the elderly. We report a 91-year-old woman diagnosed with cardiac tamponade, which was the presenting clinical feature of a previously undiagnosed case of systemic lupus erythematosus.

Age of Onset↗

A family survey of lupus erythematosus. 1. Heritability.

First degree relatives and spouses of 36 patients with systemic lupus erythematosus (SLE) and 37 with discoid lupus erythematosus (LE) were assessed using the ARA criteria. They were compared with relatives and spouses of patients with other rheumatic and related complaints. Definite SLE was present in 3.9% of relatives of SLE probands, 2.6% of discoid relatives and 0.3% of controls. Discoid LE was diagnosed in 0.6% of SLE and 3.5% of discoid families compared with 0.5% of controls. None of the spouses of LE probands had SLE or discoid LE. The data gave the best fit for a polygenic inheritance with a heritability of 66 +/- 11% for SLE and 44 +/- 10% for discoid LE. Genetic factors are thus less important in SLE and discoid LE than in generalized osteoarthritis, spondylitis or gout with heritabilities of 90, 72 and 90%, respectively.

Arthritis, Rheumatoid↗

[Papular mucinosis associated with lupus erythematosus. A case presentation and review of the literature].

A 36-year-old woman presented with a widespread papulonodular eruption followed by cutaneous and systemic manifestations of lupus erythematosus. Both conditions, papular mucinosis and lupus erythematosus were investigated by histopathology, immunofluorescence and electron microscopy. Referring to seven other cases reported in the literature, the possible relationship between lupus erythematosus and cutaneous mucinosis is discussed. It seemed that the cutaneous deposits of mucine were secondary to lupus erythematosus and not a simultaneous occurrence of the two diseases.

Adult↗

Treatment of systemic lupus erythematosus.

Reports on a variety of therapies for systemic lupus erythematosus have been published over the past year. Most of these are single case reports or open studies. As reported last year in this journal, the potential uses of intravenous gamma globulin and plasmapheresis continue to be explored. The use of cyclophosphamide for nonrenal manifestations follows studies last year of its use for lupus nephritis. No major double-blind studies of therapy for lupus nephritis were published; however, the course of lupus patients receiving dialysis or grafts was the subject of three interesting studies. An interesting study on the ability of hydroxychloroquine to prevent disease exacerbations was also published. In summary, the reports of therapy over the past year represent variations on themes. The use of new agents will most likely be based on improvements in our understanding of the pathogenesis of systemic lupus erythematosus.

Hematologic Diseases↗

[Soluble interleukin 2 receptor as activity parameter in serum of systemic and discoid lupus erythematosus].

The evaluation of disease activity in systemic lupus erythematosus (SLE) is important for selection of the appropriate therapeutic regimen. In addition to the clinical picture, various laboratory parameters are taken into account. However, no validated criteria for the evaluation of the disease activity in SLE have yet been established. Recently, serum levels of soluble interleukin-2 receptor (sIL-2R) have been proposed as a potential parameter for disease activity in SLE. However, the studies reported on this subject so far have focused mainly on certain subsets of the disease, and the evaluation of the disease activity was based on a very limited number of parameters. In the present study, we determined serum levels of sIL-2R in 23 patients with SLE and 30 patients with discoid LE (DLE). Evaluation of disease activity in SLE was based on a comprehensive scale which considered numerous clinical signs and laboratory parameters. In SLE, serum levels of sIL-2R showed a better correlation with disease activity than all the other parameters investigated, including proteinuria, erythrocyte sedimentation rate, serum globulin concentration, titre of antibodies against double-stranded DNA, serum albumin concentration, serum complement levels and white blood cell count. For the first time, we report on elevated serum levels of sIL-2R in DLE, which also correlated with disease activity.

Adolescent↗

Amyloidosis and systemic lupus erythematosus.

A 59 year old man with systemic lupus erythematosus developed proteinuria and renal insufficiency. Renal biopsy revealed both crescentic glomerulonephritis and amyloid in glomeruli and blood vessels. The amyloid was characterized as secondary because of its sensitivity to potassium permanganate pretreatment of Congo red stained sections. Amyloidosis is very uncommon in systemic lupus erythematosus but may be a cause of steroid unresponsive proteinuria and renal insufficiency.

Amyloidosis↗

Selective expansions of T cells expressing V beta 8 and V beta 13 in skin lesions of patients with chronic cutaneous lupus erythematosus.

There are several clinical types of cutaneous lupus erythematosus (LE), including acute cutaneous LE (ACLE), which occurs in 50-60% of patients with systemic LE (SLE), chronic cutaneous LE (CCLE), which is almost the same as discoid LE (DLE), and subacute cutaneous LE (SCLE). Although several important hypotheses have been proposed to explain cutaneous LE, the pathomechanisms still remain complicated and obscure. Of special interest is whether and how the T cell receptor (TCR) repertoire of infiltrating lymphocytes is involved in the development of the different types. To address this issue, we immunohistochemically examined the V beta usage of infiltrating T cells in skin lesions, as well as in peripheral blood mononuclear cells (PBMC) of patients with cutaneous LE. The number of V beta 3.1 CD3+ cells in the PBMC of patients with ACLE and CCLE was significantly lower than in controls. In contrast, the number of V beta 3.1 CD3+ cells was elevated in the skin lesion of CCLE over that in psoriasis vulgaris or atopic dermatitis. Furthermore, skin lesions in CCLE patients showed a higher incidence of V beta 8.1 CD3+ and V beta 13.3 CD3+ cells than did those in ACLE patients. These results suggest that skin lesions of CCLE are oligoclonally associated with selective expansions of TCR V beta chains and may be induced by antigen stimuli, including superantigens.

CD3 Complex↗

Serum concentrations of alpha tocopherol, beta carotene, and retinol preceding the diagnosis of rheumatoid arthritis and systemic lupus erythematosus.

OBJECTIVES: Because oxidative damage has been implicated in the pathogenesis of rheumatoid arthritis and systemic lupus erythematosus, this study was designed to see if serum concentrations of alpha tocopherol, beta carotene, and retinol, substances believed to be involved in the prevention or repair of oxidative damage, might be lower among persons who develop rheumatoid arthritis or systemic lupus erythematosus than among those who do not. METHODS: For this prospective case-control study, persons with rheumatoid arthritis and systemic lupus erythematosus that developed two to 15 years after donating blood for a serum bank in 1974 were designated as cases. For each case, four controls were selected from the serum bank donors, matched for race, sex, and age. Stored serum samples from cases and controls were assayed for alpha tocopherol, beta carotene, and retinol. RESULTS: Cases of both diseases had lower serum concentrations of alpha tocopherol, beta carotene, and retinol in 1974 than their matched controls. For rheumatoid arthritis, the difference for beta carotene (-29%) was statistically significant. CONCLUSIONS: These findings support those of a previous study that low antioxidant status is a risk factor for rheumatoid arthritis. They suggest a similar association for systemic lupus erythematosus.

Adult↗

HLA antigens in systemic lupus erythematosus.

Forty-five patients suffering from systemic lupus erythematosus were studied in respect of their serologically defined HLA antigens. HLA-B8 antigen was found in 37-8% of patients as compared to 22% of controls. Individuals carrying the HLA-B8 antigen have a 2-15 times greater risk of developing systemic lupus erythematosus than those not carrying this antigen.

Adolescent↗

Hypertrophic lupus erythematosus: a clinicopathological study of 14 cases.

BACKGROUND: Hypertrophic lupus erythematosus (HLE) is a distinct and rare subset of lupus erythematosus (LE). It is characterized by verrucous lesions, chronic in its course, and resistant to treatment. The purpose of this study was to identify clinical and histological characteristics of HLE. METHODS: We review our experience with 14 cases of HLE identified in a group of 220 patients with different forms of LE, at the UNICAMP Hospital, between 1976 and 2002. RESULTS: All patients presented verrucous plaques concomitantly with discoid lesions. The most common sites of involvement were the face and the arms. Histology of HLE lesions revealed pseudoepitheliomatous hyperplasia engulfing elastotic material. Elastic fibers were seen in migration throughout the epidermis. Classic features of LE were noted in all cases. Three of the patients developed hyperkeratotic papules with central keratinous plug on their arms at the previous LE sites. These lesions resemble clinically and histopathologically keratoacanthomas. In one patient, HLE lesion progressed to squamous cell carcinoma (SCC), 26 years after the onset of the disease. CONCLUSIONS: Transepithelial elimination of the elastotic material may be a feature of HLE. Some HLE lesions may present as keratoacanthoma, but classical features of LE aid the correct diagnosis. SCC may arise on a long-standing HLE lesion; therefore HLE requires clinical and histopathological follow up.

Adult↗

Familial lupus erythematosus.

The child of a woman with discoid lupus erythematosus (DLE) had lesions of DLE develop at the age of 2 months. The original lesions persisted and new lesions continued to develop to date; the child is now 28 months old. In a second child, now 11 months old, a probable lupus erythematosus rash developed at 1 week of age.

Adult↗

Erythema gyratum atrophicans transiens neonatale: a variant of cutaneous neonatal lupus erythematosus.

We cared for a patient with neonatal lupus erythematosus with cutaneous and hepatic involvement that was clinically and histologically indistinguishable from erythema gyratum atrophicans transiens neonatale, an entity described by Gianotti and Ermacora in 1975, which we consider to be a subtype of cutaneous neonatal lupus erythematosus. The differential diagnosis of any annular erythema in the newborn requires careful clinicopathologic evaluation, with direct immunofluorescence studies and determination of anti-Ro/SS-A, anti-La/SS-B, and anti-nRNP antibodies in both the baby and the mother.

Age Factors↗

High IgM antibody to human T-lymphotropic virus type I in systemic lupus erythematosus.

Twenty-six percent of 53 systemic lupus erythematosus sera had high levels of IgM antibody to human T-lymphotropic virus Type I, significantly more than the 5% of normal controls. Neither IgG antibodies to Type I virus nor IgM or IgG antibodies to Type II virus were increased in lupus. Further analysis using competition immunoassay and Western blot techniques also suggested that the IgM Type I antibodies in lupus sera were directed against viral antigens but did not completely exclude a nonviral reaction. Other studies also have not found IgG antibodies to the Type I virus but have not tested for IgM antibodies. Our study suggests that human T-lymphotropic virus Type I or a related virus may be involved in the pathogenesis of some cases of systemic lupus erythematosus.

Adolescent↗

[Immunological staging in lupus erythematosus. Basic data and practical significance].

A complete staging of immunity in lupus erythematosus includes immunoserologic, immunocytologic, and immunohistologic findings. However, not all of these laboratory findings are of clinical relevance. The most important immunologic parameters for lupus erythematosus can be summed up in three obligatory and two facultative tests. This "immunologic staging" is considered to be of decisive importance for the risk classification of patients with lupus erythematosus and for the choice of the therapeutic procedure.

Antibodies, Anti-Idiotypic↗

Subacute cutaneous lupus erythematosus presenting as erythroderma.

We report the first case of subacute cutaneous lupus erythematosus presenting with exfoliative erythroderma. Although our patient had uniquely extensive skin disease, she had the clinical, histologic, serologic, and immunogenetic features of subacute cutaneous lupus erythematosus. Complete remission was obtained with a short course of systemic corticosteroids and long-term hydroxychloroquine therapy. We briefly discuss the usual features of subacute cutaneous lupus erythematosus.

Acute Disease↗

Nervous system involvement in systemic lupus erythematosus: report of three cases.

Central nervous system involvement in systemic lupus erythematosus is rather frequent whereas peripheral nervous system involvement is much less common. The three patients studied by us had isolated manifestations uncommon in nature. The first one developed a sensory-motor polineuropathy with signs of axonal degeneration. It responded to the therapeutic association of corticosteroids with an immunosuppressive agent. Satisfactory recovery took place over a time span of a year. The second patient had encephalic and cerebral trunk involvement from which an irreversible dementia resulted. The third patient, who had recurrent aseptic meningitis, is asymptomatic for six months now. Patients one and two had no systemic manifestations at the time of nervous system involvement. Suspicion of systemic lupus erythematosus was made on the basis of past inspecific articular symptoms. The neurological and systemic manifestations may be sometimes simultaneous; they are usually followed by serologic changes. Isolated nervous system involvement may be seen with and without sorologic changes, and there may be found antibodies reactive with phospholipids (anticardiolipin, antigangliosides and anticerebrosides). The employment of nonsteroid immunosuppressive drugs associated with corticosteroids in small doses seems to be useful in cases of systemic lupus erythematosus with nervous system involvement.

Adolescent↗

[Chorea and systemic lupus erythematosus].

The appearance of chorea due to systemic lupus erythematosus is extremely rare. A greater frequency of circulating anticoagulant, false positive luetic serology and antiphospholipid antibodies has been recently detected in these patients. For this reason, it has been postulated that these could be involved in the genesis of the chorea through a thrombotic mechanism or autoimmune encephalitis. We describe a case or chorea in a female patient with systemic lupus erythematosus.

Adolescent↗

Recurrent systemic lupus erythematosus involving renal allografts.

Two cases of recurrent lupus erythematosus involving renal transplants are described. Neither case showed changes of transplant rejection. The clinical course, serologic tests, and renal histopathologic findings remarkably paralleled those seen during each patient's primary disease presentation. Typical and severe extrarenal manifestations of dermatitis, pleuritis, and polyarthritis were noted before renal allograft lupus involvement. Rapid renofunctional deterioration was halted in one patient by plasmapharesis. In the second patient a more protracted course involving the allograft is anticipated. Although previous experiences suggest that lupus disease activity "burns out" after the development of chronic renal failure, findings from these two cases argue against this presumption. Longer periods of transplant follow-up may show more recurrence of lupus erythematosus.

Adult↗