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Persistence of macroprolactinemia due to antiprolactin autoantibody before, during, and after pregnancy in a woman with systemic lupus erythematosus.

A woman with systemic lupus erythematosus (SLE) with marked increases in circulating 150-kDa PRL was studied from before conception, throughout pregnancy, and after pregnancy. The clinical features of the patient included idiopathic hyperprolactinemia without clinical symptoms such as amenorrhea and galactorrhea before pregnancy. No clinical lupus activity was present during follow-up. Serum PRL increase during pregnancy in this patient was considerably higher at weeks 27 and 33 than in normal pregnant women. In contrast, serum-free PRL levels were considerably lower at weeks 20, 27, and 33 than in normal pregnant women. A 150-kDa PRL (big big PRL) species persisted as the predominant circulating form of PRL throughout each measurement in this woman with SLE. In contrast, the predominant form of PRL in serum from healthy pregnant women was little PRL (or monomeric PRL). The nature of big big PRL was due to the presence of anti-PRL autoantibodies forming an IgG-23 kDa PRL complex, in accordance with the studies by affinity chromatography for IgG and Western blot analysis. The IgG-PRL complex was fully bioactive in vitro (Nb2 rat lymphoma cell assay). Injection of the serum into the rats demonstrated that the IgG-PRL complex was cleared more slowly than serum containing predominantly monomeric PRL. The data suggest that the IgG-PRL complex has biological activity; the absence of symptoms in this woman may be attributed to the fact that due to its large molecular weight, big big PRL does not easily cross the capillary walls. Delayed clearance may account for increased serum PRL levels in this SLE patient with anti-PRL autoantibodies.

Adult↗

Acute myocarditis and left ventricular aneurysm as presentations of systemic lupus erythematosus.

A case of systemic lupus erythematosus (SLE) associated with fever, heart failure, and left ventricular (LV) aneurysm is reported. A diagnosis of SLE was suspected owing to the presence of active lymphocytic myocarditis and fibrinous endocarditis at LV endomyocardial biopsy and was confirmed by identification of 4 of the 11 criteria proposed by the American Rheumatism Association for the definition of SLE. A 2-month period of steroid therapy was followed by a remarkable recovery of LV function and progression of endomyocarditis to a healed phase at control LV biopsy. The LV aneurysm disappeared, likely because thrombosis occurred as a result of the hypercoagulable state accompanying the presence of anticardiolipin antibodies. This is the first reported case of LV aneurysm induced by SLE and is a rare clinicohistologic documentation of the effectiveness of steroid treatment on lupus endomyocarditis.

Acute Disease↗

Bilateral phrenic paralysis in a patient with systemic lupus erythematosus.

Respiratory manifestations of systemic lupus erythematosus (SLE) are frequent. They include respiratory muscle abnormalities, which have been implicated in the pathogenesis of the "shrinking lung syndrome" (SLS). We report the case of a patient with this syndrome, in whom diaphragmatic paralysis due to demyelinating phrenic lesions was diagnosed at the same time as SLE. Follow-up studies showed a favorable clinical and diaphragmatic outcome with corticosteroid therapy, but little change in spirometry. It is concluded that severe diaphragm palsy is possibly due to phrenic nerve lesions in SLE, and that the link between diaphragm dysfunction and the SLS is probably not a straightforward one.

Adult↗

Reactive hemophagocytosis in systemic lupus erythematosus.

A patient with systemic lupus erythematosus (SLE) developed reactive hemophagocytosis. This case did not show any underlying diseases such as infection or malignancy other than SLE itself. The mechanisms inducing hemophagocytosis in SLE seem to be heterogeneous and remain to be elucidated. Although an immune complex-mediated mechanism in cases with acute lupus hemophagocytic syndrome has been proposed, we suggest the possible involvement of IL-1beta as the pathogenesis of our case.

Adult↗

Acquired hemophilia A in a patient with systemic lupus erythematosus.

A patient with systemic lupus erythematosus (SLE) developed acquired hemophilia A. The patient, a 24-year-old Japanese woman, was referred to our hospital because of uncontrollable bleeding following a tooth extraction. Laboratory examination revealed prolonged APTT (116 seconds), reduced factor VIII activity (2.8 %) and the presence of factor VIII inhibitor at a titer of 46.5 Bethesda units/ml. Transfusion of prothrombin complex concentrate and activated prothrombin complex concentrate followed by administration of prednisolone and cyclophosphamide successfully arrested bleeding and reduced the factor VIII inhibitor level. Acquired hemophilia A is a rare but lethal condition. Rapid diagnosis and introduction of adequate therapies are critical.

Adult↗

Acquired aortic stenosis in systemic lupus erythematosus.

Two patients with systemic lupus erythematosus had aortic valvular stenosis resulting from massive thrombotic deposits on the valve. This condition has not been described previously, but a similar process has been reported to have caused mitral stenosis in a patient.

Adult↗

[Contraception and pregnancy in severe systemic lupus erythematosus: a case report].

Systemic Lupus Erythematosus (SLE) during pregnancy may cause serious complications in the mother and fetus. We report a case of pregnancy in 23 years old woman with SLE. Pregnancy was complicated with spontaneous abortion and septic shock. Furthermore in our paper we discussed contraceptive methods proper for women with SLE. The choice of an optimal contraceptive method (OC) therapy is a significant problem in female SLE patients. In view of the influence of sex hormones on the evolution of SLE, oral contraceptive (OC) therapy has to be efficient, reversible and safe, without aggravating disease activity and causing metabolic and vascular side effects. As regards oral contraceptives, all authors agree that oestrogen-progestin combination pills are harmful, but the best alternative hormonal contraception remains to be determined. The management of the case and the review of current literature are presented. Our experience demonstrates the importance of interdisciplinary care.

Abortion, Spontaneous↗

[Diffuse alveolar hemorrhage in systemic lupus erythematosus].

Of the 120 systemic lupus erythematosus (SLE) patients treated by the authors, two have developed diffuse alveolar haemorrhage. The authors' objective is to present this rare, but severe manifestation. Patients 1 and 2 were 66- and 22-year old women, respectively. Both had SLE with multi-organ involvements including diffuse proliferative lupus nephritis. Before the diagnosis of the disease, both patients had experienced pneumonitis that resolved on corticosteroid treatment. Soon after the diagnosis, respiratory failure, haemoptoea and acute anaemia developed, accompanied by a rapid deterioration in the general condition. Chest radiographs revealed bilateral, diffuse, alveolar infiltrates. The pulmonary haemorrhage temporarily ceased in response to corticosteroid treatment, but both patients later died in consequence of active SLE and mixed bacterial and fungal sepsis. Post mortem examination demonstrated fibrosing alveolitis and alveolar bleeding in Patient 1, and an immune complex deposition-induced alveolocapillary inflammation with alveolar haemorrhage in Patient 2. Diffuse alveolar haemorrhage is a life-threatening manifestation of SLE. Its onset may be preceded by episodes of pneumonitis resolving on corticosteroid treatment. An active diagnostic workup, intensive observation and aggressive immunosuppressive treatment are the cornerstones of the management. The early detection and the active treatment of secondary infections are obligatory. The authors consider the most difficult challenge to be the optimum coordination of the above treatment modalities.

Adult↗

[Deforming hand arthropathy in systemic lupus erythematosus].

A case of systemic lupus erythematosus with deforming hand arthropathy (DHA) is reported in a woman observed for 15 years. DHA was presented with stable ulnar deviation, deformation of the fingers. This resulted in functional insufficiency of the hand of the third degree.

Adult↗

[Association of HLA-A, B, and DR haplotypes with genotype in Chinese children with systemic lupus erythematosus].

OBJECTIVE: Development of systemic lupus erythematosus (SLE) is not only associated with single loci of HLA gene, but also possibly related to certain haplotypes and genotypes of MHC. In the present study the authors explored association of HLA-A, B, DR haplotype and genotype with SLE in Chinese children, analyzed a large family with multiple SLE patients and genetic origin of SLE patients with HLA-DRB1 * 15, to discover the influence of linkage disequilibrium of HLA gene on SLE. METHODS: HLA-A, B, DR alleles were tested in 53 patients with SLE and 40 cases with their parents, 35 patients with SLE and HLA-DRB * 15 positive and 27 cases with their parents, a large family with SLE (18 members of three generations) and also 78 normal controls and 43 cases with their parents by microlymphocytotoxicity test and polymerase chain reaction - sequence specific primers (PCR-SSP). HLA-A, B, DR haplotype and genotype of SLE patients and controls were statistically calculated. The SLE patients with HLA-DRB1 * 15 and controls were analyzed for either the gene originated from the paternal or the maternal side. RESULTS: The variety of the haplotype in patient group (64/80) was less than that in control group (74/86). Only 9 haplotypes were found common between the patient group and control group. The frequency of the haplotype HLA-A9B40DRB1 * 15 was significantly higher in patient group than that in control group (P < 0.05), RR was 10.726 0. Five members of the large family had haplotype A9B40DRB1 * 15, 2 of them were patients with SLE, 1 of them was positive for ANA and had Raynaud's phenomenon and 2 of them were normal. The rest of the family members were normal. The frequency of genotypes DRB1 * 09/DRB1 * 15 and DRB1 * 03/DRB1 * 15 in SLE group was significantly higher than that of control group (P < 0.05), RR was 7.772 7 and 14.272 7, respectively. The number of SLE children with gene HLA-DRB1 * 15 derived from their fathers was significantly higher than that of the children with the gene derived from the mothers. CONCLUSION: These findings suggested that haplotype HLA-A9B40DRB1 * 15 and genotypes HLA-DRB1 * 09/DRB1 * 15, HLA-DRB1 * 03/DRB1 * 15 were correlated with SLE. The predisposition of multiple loci seems to have an additive effect. The children with their gene HLA-DRB1 * 15 derived from their fathers might more easily suffer from SLE than those with the gene derived from their mothers, the underlying mechanism needs further studies.

Adolescent↗

Hyperinsulinemia, insulin resistance, and circulating oxidized low density lipoprotein in women with systemic lupus erythematosus.

OBJECTIVE: Women with systemic lupus erythematosus (SLE) have increased risk of coronary heart disease (CHD) that is not fully explained by the classic CHD risk factors. Insulin resistance is an established risk factor for CHD in the general population. We compared insulin secretion and sensitivity in patients with SLE and healthy controls, and assessed the prevalence of the metabolic syndrome in women with SLE and its relation to circulating oxidized low density lipoprotein (ox-LDL). METHODS: Fasting insulin, glucose, and lipid profiles were measured in nondiabetic women with SLE (>or= 4 revised 1997 criteria) not undergoing antimalarial therapy (n = 44), and in age matched controls recruited from the hospital staff and the local community (n = 45). Using the Homeostatic Model Assessment equations, insulin sensitivity (HOMA-S) and pancreatic beta cell function (HOMA-B) were calculated from fasting insulin and glucose. The metabolic syndrome, defined according to the Adult Treatment Panel (ATP III) criteria, was determined in a consecutive series of 61 women with SLE. RESULTS: Patients with SLE had significantly higher fasting insulin [median (range) 10 (2.8-38) vs 6.6 (3.1-26) mU/l; p < 0.01], higher pancreatic beta cell function (HOMA-B) [165 (54-1567) vs 111 (28-653); p < 0.01], and lower insulin sensitivity (HOMA-S) [0.46 (0.09-1.9) vs 0.73 (0.16-1.3); p < 0.01]. SLE patients also had significantly higher triglycerides (p < 0.01) and lower high density lipoprotein cholesterol (p < 0.01) than controls. HOMA-S did not correlate with disease activity or steroid therapy, but was associated with components of the insulin resistance syndrome. HOMA-S showed a significant negative correlation with levels of ox-LDL in patients, but not in controls. Eleven (18%) patients had the metabolic syndrome. Again, this was not related to current steroid therapy. SLE patients with the metabolic syndrome had no difference in LDL, but had significantly higher levels of ox-LDL. CONCLUSION: Nondiabetic patients with SLE have evidence of significant decrease in sensitivity to insulin, and overall this population has a high prevalence of the metabolic syndrome (18%). Insulin resistance in the context of SLE was not strongly related to current or recent steroid therapy; it was, however, associated with higher levels of ox-LDL. Insulin resistance may therefore represent an additional CHD risk factor in patients with SLE.

Adult↗

Systemic lupus erythematosus.

Recent investigations in systemic lupus erythematosus (SLE) have disclosed the presence of paramyxo-virus-like nucleocapsids in the cytoplasm of renal endothelial cells of patients with this disease. Elaborate techniques have failed to demonstrate conclusively that these are indeed viruses. Concomitantly, there is increasing evidence that SLE patients have defective cell-mediated-immunity. A 3-year-old child with SLE exhibited clinical evidence of defective cellular immunity. At postmortem, paramyxoviruslike tubular structures were demonstrated in her kidneys. Additional findings included the presence of intracytoplasmic viral particles consistent morphologically with herpes simplex virus in neurons from the lateral geniculate body and numerous "nuclear bodies" in neurons and glial cells. A dysplastic thymus gland demonstrated virtual absence of lymphocytes and was devoid of Hassall corpuscles, unlike the thymus usually seen in SLE.

Autopsy↗

Predictors of carotid atherosclerosis in systemic lupus erythematosus.

OBJECTIVE: Patients with systemic lupus erythematosus (SLE) are at increased risk for cardiovascular and cerebrovascular events, even after adjustment for traditional risk factors. We examined the association of traditional risk factors, novel markers of cardiovascular disease (C-reactive protein, homocysteine, lipoprotein(a), plasminogen activator inhibitor-1, fibrinogen), and markers indicative of SLE activity (including C3, C4, anti-dsDNA, and prednisone use) with the presence of significant plaque on carotid duplex imaging. METHODS: Six hundred five patients with SLE enrolled in the Hopkins Lupus Cohort Study (92% female, 38% African-American) underwent carotid duplex testing. Prospectively gathered clinical, laboratory, and serologic data from their quarterly followup visits in the Hopkins Lupus Cohort were used in the analyses. For predictors that varied over time, such as cholesterol, the mean values during cohort participation were calculated for the analysis. Informed consent was obtained from all patients. RESULTS: The presence of carotid plaque was strongly associated with age, ranging from 1% among those less than 30 years of age to 61% among those 60 years or older. After adjusting for age, there were moderate or strong associations of carotid plaque with male gender (age-adjusted risk 25% vs 13%; p = 0.051), hypertension (age-adjusted risk 18% vs 8%; p = 0.0001), diabetes mellitus (age-adjusted risk 19% vs 13%; p = 0.075), C3 > 120 mg/dl (age-adjusted risk 18% vs 11% and 14% for normal and low C3, respectively; p = 0.046), serum creatinine > 1.3 (age-adjusted risk 32% vs 13%; p = 0.039), and mean systolic blood pressure > 140 (age-adjusted risk 23% vs 13%; p = 0.028). There was no strong evidence of an association between plaque and SLE disease activity (age-adjusted risk 14% among those with adjusted mean SLEDAI > 3 vs 14% among those with lower SLEDAI) or with time since SLE diagnosis (age-adjusted risk 12%, 14%, and 16% among those with SLE for < 2, 2-8, and > 8 years, respectively; p = 0.49). CONCLUSION: Traditional cardiovascular risk factors were associated with carotid plaque in SLE. However, SLE disease activity and duration of SLE are not strongly associated with carotid plaque. A "lupus factor" separate from traditional risk factors remains unidentified.

Adult↗

T-zone lymphoma in association with systemic lupus erythematosus.

Two patients with systemic lupus erythematosus (SLE) and T-zone lymphoma are described. On admission, both showed arthralgia, generalized lymphadenopathy, hypergammaglobulinemia and positive antinuclear antibody. Lymph node biopsies revealed diffuse infiltration of atypical T-lymphocytes in the expanded interfollicular area (T-zone), a finding characteristic for the T-zone lymphoma. Renal biopsy showed lupus nephritis and neoplastic lymphoid cell infiltration in the glomeruli of one patient, but only diffuse infiltration of neoplastic lymphoid cells and mature plasma cells were observed in the interstitium of the other patient. Both patients responded remarkably well to prednisolone (1 mg/kg/day).

Humans↗

Subretinal neovascularization in systemic lupus erythematosus.

Choroidal involvement in systemic lupus erythematosus (SLE) occurs infrequently. We describe a patient with unusual manifestations of lupus oculopathy: prominent choroidal vasculopathy associated with multiple chorioretinal scars and subretinal neovascularization in the macular area. Choroidal disease was the primary feature of ocular involvement. SLE should be considered in the differential diagnosis in patients with inflammatory choroidal lesions.

Aged↗

[Polymyalgia rheumatica as a form of presentation of systemic lupus erythematosus].

Three cases of systemic lupus erythematosus (SLE) with clinical presentation similar to a rheumatic polymyalgia (RP) are described. There was no evidence of underlying myopathy in any of the cases thus suggesting that the synovitis is the pathogenic mechanism. The late onset and the poor serologic expression are the relevant clinical signs which could delay or hinder the diagnosis.

Aged↗

[Erythrocyte CR1 receptor reactivity in patients with systemic lupus erythematosus].

Erythrocytes from 42 systemic lupus erythematosus (SLE) patients and 80 healthy volunteers were tested for the immunoadherence (CR1) receptor reactivity, observed by hemagglutination (IAHA) when incubating erythrocytes and aggregated human gamma-globulin (AHGG)-complement in appropriate proportions. Reactivity was expressed as the highest two-fold dilution of AHGG (2n) that induced hemagglutination. Erythrocytes of 15 SLE patients (35.7%) showed reactivity compared with 70 normal controls (87.5%). Both groups showed a trimodal distribution of IAHA titers in accordance with the three phenotypic groups described by other authors. Of the healthy population 72.5% belong to the intermediate reactivity mode (2(6) to 2(8)) and 64.3% of the SLE patients to the low reactivity group (negative). There was no correlation between CR1 defective expression and conventional activity parameters. This erythrocyte receptor involved in the immunocomplex clearance process, which constitutes 95% of the circulating CR1, is another factor that contributes to the pathophysiology of the disease when it is defective.

Erythrocytes↗

[Role of T lymphocytes in systemic lupus erythematosus].

In very active systemic lupus erythematosus (SLE), the serum levels of interleukin-2 (IL-2) are increased, indicating activation of T lymphocytes in the organism. The peripheral blood, however, contains T cells with a strongly reduced functional capacity, which results from a post-activation/refractory state; this hypofunction is reversible. Thus, the activity of the T lymphocytes in the circulating pool is the "mirror image" of the activity of these cells at the level of the whole organism. The B lymphocyte hyperactivity in SLE is T-dependent. A highly specific mechanism is involved in the activation of the B cells producing anti-native DNA antibodies. The investigation of lymphocyte functions enables one to follow the effect of immunosuppressive therapy.

B-Lymphocytes↗