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[A case of late-onset SLE complicated with EDTA-dependent pseudothrombocytopenia].

A 57-year-old man was admitted to our clinic with complaints of proximal myalgia in extremities. He was diagnosed as late-onset SLE based on the findings of pleuritis, pericarditis, arthritis and antibodies to DNA and cardiolipin. Aggregation of the platelets and the decreased counts of platelets were observed when EDTA was used as anticoagulant for the blood tests. However, the platelet aggregation was not noted with normal counts of platelets when Heparin-Theophylline was used as anticoagulant. From this observation, EDTA-dependent pseudothrombocytopenia was diagnosed and IgM class of EDTA-dependent anti-platelet antibody was detected by means of flow cytometry. Administration of prednisolone at 40mg/day reduced the symptoms and EDTA-dependent pseudothrombocytopenia, and EDTA-dependent anti-platelet antibody disappeared. His clinical course suggested that EDTA-dependent pseudothrombocytopenia was closely associated with the disease activity of SLE.

Autoantibodies↗

Inappropriate secretion of antidiuretic hormone (SIADH) in a patient with systemic lupus erythematosus (SLE): a case report.

The Syndrome of Inappropriate Secretion of Antidiuretic Hormone (ADH) or SIADH has been reported in a great variety of diseases and disorders of the central nervous system. However, to our knowledge only three cases [Kaplan et al., Decaux et al. 1981, Agus et al. 1983] of SIADH associated with systemic lupus erythematosus (SLE) have been described in the literature. We report here a new case of SIADH in association with SLE.

Adult↗

[Case report of collagen lung in SLE presenting with cough variant asthma: relation between the localization of responsible receptors and cough].

A 27-year-old nonsmoking woman complained of cough and chest oppression for two years since an episode of pneumonia. Clinical tests showed decrease in FEV1.0 during attacks of coughing and evidence of bronchial hypersensitivity. While these events fitted the picture of bronchial asthma, the nonwheezing cough suggested cough variant asthma. Antinuclear antibody and anti-ds DNA antibody were increased and leukopenia was recognized, suggesting the diagnosis of systemic lupus erythematosus (SLE). Bronchoalveolar lavage showed lymphocytic alveolitis and decreased T4/T8. These results were suggestive of collagen lung induced by SLE. Inhalation challenge with capsaicin and rapid intravenous injection of lobelin and alinamin indicated that peripheral c-fiber receptors were involved in the induction of coughing. We conclude that the peripheral lesion of collagen lung stimulates the peripheral c-fiber receptors, leading to cough variant asthma.

Adult↗

[Systemic lupus erythematosus (SLE): sexual hormones, contraception and pregnancy].

Sexual hormones, particularly estrogens, appear to play a role in the development of SLE. Pregnancy as well as the use of exogenous sexual hormones prescribed as contraceptives, as substitutive therapy, or to induce ovulation can be associated with disease flares. However, pregnancy can be envisaged in SLE patients with well controlled disease, on the condition that medical follow-up is observed. To prevent fetal loss due to antiphospholipid syndrome prophylactic anticoagulation is required. The presence of maternal anti-SSA antibodies is an indication for a tight pregnancy follow-up for early detection of fetal cardiac conduction block. Finally, in the absence of antiphospholipid antibodies, oral contraception with low-dose estrogens and progestatives may be contemplated.

Adult↗

Histamine-binding receptors and Fc receptors of T lymphocytes in SLE.

The participation of TG cells forming the suppressor T cell population and of histamine-binding cells was determined in the peripheral blood of patients with SLE. Former was considerably diminished in the active phase of the disease, latter was found diminished in both the active and the inactive phase. The results published in this paper support the functional disorder of suppressor T cells in SLE, an assumption based only on sporadic data before.

Female↗

[Retrospective evaluation of pregnancy in women suffering from systemic lupus erythematosus (SLE)].

OBJECTIVES: Systemic lupus erythematosus (SLE) is an autoimmune disease predominantly affecting women of childbearing age, which may negatively influence the pregnancy course and outcomes. The objective of the study was to estimate the risk of fetal loss and prematurity in lupus patients. These data seem to be an important component of family counselling in lupus patients. DESIGN: The pregnancies' course and outcomes in lupus patients and in healthy controls were compared. MATERIAL AND METHODS: 47 lupus patients with pregnancies occurring after the diagnosis of SLE and 108 healthy controls were included in this study. The pregnancies were analysed in terms of delivery term and outcomes (live births/ miscarriage/ neonatal deaths/ congenital defects). The structured review and case histories analysis were used to gather the data. RESULTS: The percentage of spontaneous abortions (24.1%) and preterm deliveries (24.1%) were significantly higher in lupus patients when compared with healthy controls (9.3% and 5.3% respectively). CONCLUSIONS: The caution in pregnancy planning in lupus patients is necessary and the role of physicians' information in patients' awareness of fetal loss and prematurity is vital.

Abortion, Spontaneous↗

Lupus anticoagulant and the skin. A longterm follow-up study of SLE patients with special reference to histopathological findings.

Skin manifestations were described in lupus anticoagulant (LA) positive and in LA negative SLE patients. Necrotic ulcers appearing at the beginning of the disease process characterized the 33 LA positive patients. Thirteen patients had a "peripheral vascular syndrome"; small leg ulcers of livedoid vasculitis type following deep venous thromboses, in 3 patients developing into pyoderma gangrenosum like ulcers and in 2 patients into pseudo-sarcoma Kaposi. The lesions were histologically characterized by capillary angiogenesis with extravasated red blood cells, sparse inflammatory cell infiltrates and microthromboses. Three patients had ulcers clinically and histologically resembling those seen in Degos' disease. Five patients had anetoderma showing elastic tissue depletion and microthromboses histologically. A different pattern of skin changes was seen in the LA negative patients. Our findings suggest that antiphospholipid antibodies play a pathogenetic role in the described skin manifestations of LA positive SLE patients.

Adolescent↗

[Primary hepatic lymphoma in a patient with autoimmune hemolytic anemia and SLE].

A 29-year-old Japanese male was diagnosed as having autoimmune hemolytic anemia (AIHA) in 1985, and systemic lupus erythematosus (SLE) in 1988. In 1989 a large mass was found of the right posterior area in the liver by computed tomography and ultrasonography. The liver biopsy specimen showed non-Hodgkin lymphoma, diffuse large cell type. No adenopathies or other extranodal involvements were detected. He was treated with five courses of CHOP, followed by involved field irradiation 24 Gy. On completion of chemoradiotherapy, the mass was smaller in size with a sign of partial necrosis. Gallium scan was negative suggesting the achievement of a complete remission. Primary hepatic lymphoma is extremely rare, and its occurrence in patients with AIHA and SLE has not been reported previously. We also review the previously reported cases of primary hepatic lymphoma with respect to its clinical management and abscess the therapeutic strategy of this unusual extranodal lymphoma.

Adult↗

[Graves' disease complicated with systemic lupus erythematosus (SLE): a case report].

A 25 year old man had been subjected to subtotal thyroidectomy under the diagnosis of Graves' disease. About a year later the patient developed systemic lupus erythematosus (SLE). Reports on cases of Graves' disease complicated with SLE have barely been observed so far. Consequently, the authors reckoned, with reference to the other literatures on the subject, our case worth reporting.

Adult↗

Low sulpho-conjugated steroid hormone levels in systemic lupus erythematosus (SLE).

In a clinical study the blood levels of dehydroepiandrosterone sulphate (DHEAS), pregnenolone sulphate (5-PS), testosterone sulphate (TS) and their respective unconjugated steroids were measured in: 20 patients with systemic lupus erythematosus (SLE) who were receiving either no treatment (11 patients) or else treatment with chloroquine (9 patients), in some cases combined with non-steroidal anti-inflammatory drugs (NSAIDs); in 26 patients receiving corticosteroid (Prednisolone) treatment; and in healthy men and women. The patients not on corticosteroid exhibited substantially reduced DHEAS, 5-PS and TS levels (geom. mean: 2300 vs. normal 4300 nmol/l DHEAS; 200 vs. 320 nmol/l 5-PS; and 120 vs. 360 nmol/l TS; p less than 0.001), irrespective of the difference in sex, age or chloroquine treatment. The patients on corticosteroid treatment displayed a similar pattern of levels, but the reduction was much more marked than in the patients not on the steroid (geom. mean: 610 nmol/l DHEAS, 55 nmol/l 5-PS; and 35 nmol/l TS; p less than 0.001). No consistent changes were observed in the unconjugated steroid levels, although they were also reduced by the treatment with prednisolone. The data indicate that a deficiency in sulpho-conjugated steroids is a permanent feature of patients with SLE and that this is accentuated by the administration of corticosteroid derivatives. Further studies are needed to establish the pathophysiological significance of these findings.

Adolescent↗

Scintigraphic quantitation of splenic function in SLE: correlation with IgM levels in serum.

The relationship between quantitative splenic uptake of sulfur colloid and immunoglobulin M level in serum was studied in 15 patients with systemic lupus erythematosus (SLE). Splenic function correlated positively with IgM level (r = 0.58, p = 0.02) and inversely with antibody titre to n-DNA (r = -0.74, p = 0.003), and was reduced (p greater than 0.001) in patients with depressed IgM levels. IgM level in serum may be a useful correlate of splenic phagocytic function in SLE patients.

Adult↗

[Analysis of systemic lupus erythematosus (SLE) involving the central nervous system by magnetic resonance imaging (MRI)].

Involvement of the central nervous system (CNS) commonly occurs in systemic lupus erythematosus (SLE). But definitive diagnosis remains difficult even with computed tomography (CT). In this study, we used the recently developed technique, magnetic resonance imaging (MRI) for CNS lupus and compared it with CT scans. CT was performed with a General Electric 8800 CT/T scanner. MRI was performed using a Mitsubishi Electric MMI-150 S. Ten patients with CNS lupus were divided into 3 groups. Group I included 4 cases with neurological manifestations alone. All lesions seen on CT were also detected by MRI, with greater clarity and extent. Furthermore, MRI depicted several microinfarcts in white matter without symptoms. Group II included 5 cases with psychiatric features alone. MRI detected a thalamic microinfarct in only one case while CT showed no abnormality in all cases. Group III included 1 case with both neurological and psychiatric symptoms. MRI demonstrated a small infarct of midbrain corresponding with neurological symptoms, more clearly than CT. Therefore MRI demonstrates the degree of brain involvement in SLE more accurately than CT.

Adult↗

Autoantibody to proliferating cell nuclear antigen (PCNA) in SLE: a clinical and serological study.

In a clinical and serological follow-up study on a large series of subjects with different connective tissue disorders, anti-PCNA/cyclin autoantibodies were found in about 3% of patients with SLE. Positive subjects showed a higher incidence of diffuse proliferative glomerulonephritis and hematological disorders than the general SLE population. A highly significant serological association between PCNA and SL/Ki autoantibodies (p less than 0.001) has been observed. Persistence or disappearance of serum PCNA antibodies were independent of any clinical and serological feature or the therapeutic regimen employed.

Adolescent↗

Null cell immunoregulation in SLE.

Fresh normal T cells do not lyse MDA-157 target cells. Normal null cells, cultured for 4 days with MDA-157 stimulators, and then mixed overnight with fresh normal T cells, induce cytotoxicity on MDA-157 targets and suppressor activity in the T-cell acceptor population. With the same normal acceptor T cells, MDA-157 activated null cells from 13/18 patients with Systemic Lupus Erythematosus (SLE), unlike activated cells from a disease control population, induce little or no T-cell cytotoxicity or suppression. These results provide further evidence for abnormal null cell function in SLE.

Cells, Cultured↗

CR1 deficiency in SLE: acquired or genetic?

Erythrocytes from 30 patients suffering from systemic lupus erythematosus (SLE) were tested for CR1 activity by an immune adherence haemagglutination technique. Defective CR1 activity (CR1D) was found in 11 (37%) of the patients on initial testing. On repeat testings, however, CR1 activity often varied from time to time and was shown to be inversely related to serum anti-DNA binding and apparent complement activation in vivo. Two of the 19 patients with normal CR1 activity acquired CR1D during the study. One patient with previously defective CR1 attained normal activity in the course of the study. The increased occurrence of CR1D in patients with SLE is largely or wholly acquired rather than genetically determined.

Adolescent↗

[Characterization of subepidermal immune deposits in clinically unchanged skin in patients with lupus erythematosus (SLE)].

Subepidermal immune deposits containing IgG and C3 by 9 patients suffering from SLE were exposed to extreme excess of antigens in order to solubilize tissue-bound immune complexes. Sections of 4 micron were incubated at room temperature either with calf thymus DNA (0.3-30 mg/ml) or with human gamma-globulin (HGG) (1.6-16 mg/ml). The sections thus pretreated were compared with "native" samples by means of direct immunofluorescence technique. Only HGG resulted in drastic removal of IgG deposits along the dermoepidermal junction. DNA proved to be ineffective; deposits of C3 remained uninfluenced. Our results are discussed in the light of the composition of the subepidermal immune complex in SLE.

Antigen-Antibody Complex↗

Demonstration of nuclear antigen binding lymphocytes by the resette method in SLE-patients.

A rosette method suitable for the demonstration of deoxyribonucleic acid and deoxyribonucleoprotein-binding peripheral lymphocytes is described. The results confirmed the existance of a human peripheral lymphocyte subpopulation capable of binding nuclear antigen specifically. The subpopulation was demonstrated in 63 out of 75 SLE-patients; 57 out of 59 SLE-patients being in the active period of the disease revealed nuclear antigenbinding lymphocytes, as against 6 our of 16 patients being in remission.

Adolescent↗

Expression of MHC class I determinants on erythrocytes of SLE patients.

Strong expression of MHC Class I determinants had been observed on the erythrocytes of three genetically C4 deficient patients who all had SLE. In a study of 35 other SLE patients who were not C4 deficient, 30 showed a marked increase in the expression of MHC Class I on their erythrocytes. There was a correlation between the expression of erythrocyte Class I and disease activity. The polymorphic HLA determinants were detected by haemagglutination with human cytotoxic antisera from untransfused pregnant women. A shared monomorphic epitope of HLA-A, -B and -C, and beta 2-microglobulin were detected by haemagglutination with monoclonal antibodies. A monoclonal antibody for a monomorphic epitope on MHC Class II alpha and beta chains did not react. Erythrocytes from a group of RA patients and a group of normal controls had moderate and low expression respectively. We suggest that MHC Class I may be induced on erythrocytes maturing in a milieu containing mediators derived from activated cells of the immune system. Aberrant tissue expression of MHC antigens may be more widespread than has been previously recognized in diseases mediated by immune mechanisms.

Arthritis, Rheumatoid↗