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A man with longstanding systemic lupus erythematosus who developed anterior dislocation of both shoulder joints is described. He also had typical Jaccoud's deformities of his hands and feet.
OBJECTIVE: Systemic Lupus Erythematosus (SLE) is an autoimmune process in which cutaneous lesions occur in majority of patients. This study from Karachi, Pakistan was conducted to determine the pattern and prevalence of such lesions in SLE in Pakistani patients. METHODS: One hundred ninety eight patients with SLE fulfilling the clinical and laboratory criteria of the American Rheumatology Association were examined between 1986 and 2001 for the presence of cutaneous manifestations. RESULTS: Skin changes noted were: noncicatricial diffuse alopecia (22%), malar rash (31%), mucosal lesions (20%), discoid eruptions (15%), photosensitivity (33%), vascular lesions (20%), pruritus (17%), and pigmentary changes (22%). Peripheral gangrene, chronic ulcers, Raynauds phenomenon, urticaria, chilblains, thrombophlebitis, palmar erythema, and erythema multiform were rare. Anti ANA and anti dsDNA were positive in 93% and 83% patients respectively. CONCLUSION: A different clinical pattern was noted in our patients than reported previously.
A young woman with systemic lupus erythematosus (SLE) was infected with human immunodeficiency virus 1 (HIV-1) and about 6 years later developed persistent bilateral parotid gland enlargement. It was unclear whether this represented salivary gland involvement as a component of her SLE (secondary Sjögren's syndrome) or the initial clinical manifestation of her HIV-1 infection. HIV proviral DNA was found in individual salivary glandular secretions and in whole saliva. Additionally, cells positive for HIV RNA were isolated from whole saliva. A parotid gland biopsy revealed infiltrating lymphocytes containing large amounts of HIV RNA.
Systemic lupus erythematosus (SLE) rarely presents with massive ascites secondary to severe peritonitis before the occurrence of major diagnostic features. We described a 13-year-old girl who developed four episodes of severe abdominal pain with massive ascites. During the first episode, the patient experienced deterioration with massive ascites over a 3-day period, after a 3-month history of intermittent abdominal pain. This episode resolved under conservative treatment. During the second episode, her acute abdominal findings mimicked appendicitis or gynecologic emergency. An exploratory laparotomy was initially planned, but not performed. Thrombocytopenia postponed the surgical intervention and led to further investigation. Autoimmune studies confirmed SLE and prompted steroid therapy, which rapidly improved her painful ascites and spared an unnecessary operation. After improvement of peritonitis with ascites, and following discharge, other major clinical features of SLE ensued. The third episode of peritonitis with massive ascites responded well to steroid pulse therapy. The fourth episode of ascites was accompanied by intracranial hemorrhage that led to her death. To the best of our knowledge, this is the first case report of recurrent peritonitis with massive ascites as the initial and major manifestation of SLE in a pediatric patient.
Seventy unselected patients with systemic lupus erythematosus (SLE) were studied to determine the prevalence of cognitive impairment and the association with other clinical variables. Twenty-five patients with rheumatoid arthritis (RA) and 23 healthy subjects were used as controls. All patients were evaluated with a battery of standardized neuropsychological tests to determine ability in 8 areas of cognitive function. Clinically overt neuropsychiatric (NP) SLE, cumulative disease manifestations and concurrent medications were documented. In patients with SLE, generalized disease activity was expressed using the SLE disease activity index. Cognitive impairment was identified in 15/70 (21%) patients with SLE, 1/25 (4%) patients with RA and in 1/23 (4%) healthy subjects (p = 0.042). The prevalence was higher in patients with active NP-SLE at the time of assessment (2/5, 40%) compared to patients with inactive NP-SLE (2/10, 20%) but was also increased in those patients who had never had known clinical NP-SLE (11/55, 20%). A history of serositis (p = 0.015), active SLE (p = 0.064) and corticosteroid use (p = 0.027) at the time of assessment were more common in patients with cognitive impairment. The results suggest that cognitive impairment is increased in patients with SLE. It may occur independently of clinically overt NP-SLE and is more common in patients with active disease who are receiving corticosteroids.
Twelve patients suffering from systemic lupus erythematosus (SLE) were treated with piroxicam (Hotemin-EGIS) for 9-18 (mean: 13.7) months. At the beginning, the patients were in a moderately active stage of the disease, 10 patients also received low dose corticosteroid treatment. After the study was completed, clinical and immunological improvement was seen in 10 cases. The average daily dose of prednisolone could be reduced (from 10.5 mg to 5 mg). These patients tolerated the treatment well, no adverse effect was seen. No elevation of serum transaminase, creatinine or change in blood cell count and glomerular filtration rate were observed. Piroxicam, therefore, is recommended for the treatment of mildly active SLE.
Acute psychotic manifestations in systemic lupus erythematosus (SLE) have been considered usually to occur early in the course of illness, although these findings are primarily based on studies with few patients and limited lengths of observation. To further investigate the chronology of acute psychiatric disturbances in SLE, the time course of these episodes was examined in 36 patients over a median duration of observation of 72 months. Twenty-two of 36 (61.1%) initial psychiatric episodes occurred within the first year of diagnosis of SLE, although several patients experienced the initial onset of psychiatric manifestations several years after the onset of SLE. Recurrent episodes occurred in 10 of 36 patients at a median of 8 months after the initial episode. Although tending to occur early in SLE, primary acute psychiatric events may first occur in patients with long durations of illness, and the time of onset of these episodes does not appear helpful in their differential diagnosis.
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.
A female in whom systemic lupus erythematosus had been diagnosed 5 years before suddenly developed headache and recurrent vomiting. Cranial computed tomography (CT) was consistent with subarachnoid hemorrhage, and cerebral arteriography disclosed cerebral aneurysms, multiple microaneurysms and cerebral vasculitis. After 7 days she developed left hemiparesis, and a cerebral infarct area was apparent in a new CT scan. Despite the poor prognosis of these lesions, the patient had a favorable outcome, without residual neurological deficit, after having been treated with intravenous corticosteroid boluses and oral cyclophosphamide.
In ten patients with systemic lupus erythematosus lymphocyte suppressor cell function was repeatedly monitored over a period of nine months. The method used is based on the finding that in vitro addition of ConA to PWM-stimulated lymphocytes activates suppressor T cells which in turn inhibit immunoglobulin synthesis. Nine of ten SLE patients exhibited significant impairment of IgG suppression but no correlation was found between the outcome of the IgG suppressor cell assay and disease activity or the treatment protocol applied.
Forty three patients with systemic lupus erythematosus (SLE) were divided into three groups, inactive, active without treatment and active on treatment with steroids. T lymphocytes from peripheral blood were determined and the results compared with those of twenty five normal individuals. In further experiments, the mitogenic capacity of SLE lymphocytes to Con A and the serum's activity of these patients upon normal lymphocytes were analized. Decreased T lymphocytes were found in all groups of patients with SLE. There were no difference between the mitogenic response of SLE patients and controls when the lymphocytes were incubated with normal human AB serum. When normal lymphocytes were incubated with serum from SLE patients with active disease, and inhibition of mitogenic response to Con A was observed. These results suggests that the decrease cellular immunity observed in SLE is due to serum factors rather than to intrinsic T cell abnormality.
Four patients with active systemic lupus erythematosus and eleven patients at quiescent stage were subjected to research in order to determine the serum concentration of C3 and to estimate the presence of anti-n-DNA antibodies and complement-fixing anti-n-DNA antibodies by the indirect immunofluorescent technique on the Crithidia luciliae. The medium title of anti-n-DNA antibodies was significantly more present in the sera of patients with active disease rather than in patients with quiescent disease and only in the first ones were found complement-fixing anti-n-DNA antibodies. Their title is expression of the title of total anti-n-DNA antibodies. Only sera with titles greater than 1:80 had antibodies able to fix the complement and at the same time they showed an important fall of C3. So the course of complement-fixing anti-n-DNA antibodies seems to have a certain prognostic meaning on the disease development.
One hundred patients with systemic lupus erythematosus (SLE) were investigated for serological markers of hepatitis B virus (HBV) and had liver function evaluated. One patient had hepatitis B surface antigen and 25 had antibodies to HBsAg which is not significantly different from normal blood donor controls. Minor liver function abnormalities were found in 35 patients and there was no difference between those with and without anti-HBs. A single patient without HBV markers had more severe liver enzyme elevation. Our data do not show a relationship between SLE and HBV and support the contention that liver diseases in lupus is mild.
In 16 patients with systemic lupus erythematosus, blood vessels in the affected region of the skin were examined regarding localization of immune complexes. Deposition of IgG, IgA, IgM, IgE, beta 1C and fibrin in the vessel walls was more frequently observed in intramural and perivascular sites than in intimal sites. IgM and fibrin were usually detected in intramural sites only. In large caliber arteries of the skin the intima was the main site of deposition of immune reactants, whereas, in small-caliber vessels, including capillaries, immune reactants were more often found in intramural and perivascular sites.
During the course of systemic lupus erythematosus in a young female patient the peripheral blood smear incidentally showed the features of hyposplenism. Silent lupus vasculitis with splenic infarction was suspected as the cause of this.
The clinical manifestations of pediatric systemic lupus erythematosus (SLE) are similar to those seen in adults with SLE with increased frequency of the following features: hepatosplenomegaly, chorea, nephritis, and avascular necrosis. Similarly, pediatric SLE patients are now showing the same improvement in survival as adult SLE patients, and it is no longer felt that the course of childhood-onset SLE is more severe than that seen in adult-onset SLE. Children of mothers with SLE can develop both transient and persistent features of SLE in the neonatal period. Transient features include photosensitive discoid rash, cytopenia, hepatosplenomegaly, myocarditis, and pericarditis; the permanent features include congenital complete heart block, endomyocardial fibroelastosis, and other structural cardiac defects.
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.
Systemic lupus erythematosus (SLE) is one of a group of three uncommon arthritic diseases, including psoriatic arthritis and scleroderma, that superficially resemble rheumatoid arthritis (RA). These diseases frequently involve the hand and wrist and therefore are significant to the hand surgeon. SLE has certain specific characteristics that separate it from the other two conditions. Each of these diseases has distinct features affecting the nature of the deformities as well as the treatment indicated to adequately care for these patients.