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Autosplenectomy in systemic lupus erythematosus.

Autosplenectomy in systemic lupus erythematosus (SLE) is associated with a high mortality rate due to Streptococcus pneumoniae sepsis. We describe 2 patients who developed autosplenectomy in the setting of an acute SLE exacerbation. Followup of both patients 12 and 5.5 years after the initial diagnosis of autosplenectomy revealed persistent absence of the spleen. Pneumococcal vaccine was given to both patients. In contrast with reports describing life threatening infections in 46.7% of asplenic patients with SLE, none developed during prolonged followup of our patients. Our data and review of 13 other patients with SLE with autosplenectomy suggest that the 23-valent pneumococcal vaccine should be given promptly to patients with SLE when a diagnosis of autosplenectomy is established.

Adult↗

Low-grade non-Hodgkin's lymphoma in a patient with systemic lupus erythematosus.

Coexistence of systemic lupus erythematosus (SLE) with low-grade non-Hodgkin's lymphoma (LGNHL) has been described occasionally in the literature with the potential pathogenetic role of monoclonal B CD5+/CD19+ cells. We report a case of LGNHL which developed 18 months after diagnosis of SLE. The monoclonal population of lymphocytes in the peripheral blood and bone marrow was CD5/CD19 negative but CD19/CD22 positive. The SLE responded well to treatment with prednisone and the course of the LGNHL was stable and cytotoxic treatment was not required.

Antigens, CD↗

HLA-DR antigens and anticardiolipin antibodies in northern Italian systemic lupus erythematosus patients.

Eighty systemic lupus erythematosus (SLE) patients attending 3 clinical centers were evaluated immunologically and immunogenetically. No HLA class II antigens were found to be significantly associated with SLE in these patients. A highly significant (P = 6.17 x 10(-7) association was observed between anticardiolipin antibodies and DR7. A lesser association (P less than 0.025) was also observed between DR2 and/or DR3 and anti-Ro (SS-A) antibodies. No relationship was found between any DR antigen and anti-Sm/RNP, anti-double-stranded DNA, or anti-La (SS-B) antibodies.

Adolescent↗

Mapping the systematic lupus erythematosus susceptibility genes.

Systemic lupus erythematosus (SLE) is a prototype systemic, autoimmune inflammatory disease that can involve virtually any organ or tissue type. The disease has a strong familial tendency but, like most human illness, has a complex pattern of inheritance that is consistent with multiple susceptibility genes as well as environmental risk factors. Association studies have been performed, especially for the major histocompatibility complex on chromosome 6 and for various complement components. Several large familial studies have begun to report results for genetic linkage. Linkage has been established for many genetic intervals. SLE is a complex clinical illness, and investigation of the genetics of the illness based on clinical manifestations revealed linkages not found without consideration of the phenotype of the disease.

Chromosome Mapping↗

Effect of counselor training on skills development and psychosocial status of volunteers with systemic lupus erythematosus.

Volunteers with systemic lupus erythematosus were recruited for a telephone service to provide psychosocial support to peers. The volunteers attended an 8-week counselor training program. The aim was to evaluate the impact of the training on counselors' skill development and to record possible changes in the counselors' psychosocial status. A second group of trainees (n = 15) was used as a delayed control for the first group (n = 14). The data were analyzed using multivariate repeated measures analysis of variance. Tests chosen to monitor psychosocial status included: Arthritis Impact Measurement Scales Anxiety and Depression subscales, Arthritis Helplessness Index, Wallston General Self-Efficacy Scale, University of California at Los Angeles Loneliness Scale, Rosenberg Self-Esteem, and Campbell Personal Competence scales. Tests selected to measure skills and knowledge.

Clinical Competence↗

ALK-negative T-cell anaplastic large cell lymphoma associated with systemic lupus erythematosus.

Patients with systemic lupus erythematosus (SLE) appear to have an increased risk of developing malignancies, especially lymphomas. We report the development of a systemic ALK-negative T-cell anaplastic large cell lymphoma, stage IIB, in a 53-yr-old Caucasian female with a 12-yr history of stable SLE. The patient responded poorly to chemotherapy and died 2 yr after diagnosis. Lymphomas that develop in patients with SLE and other autoimmune diseases are virtually always of B-cell origin. To our knowledge this is the first report of a T-cell anaplastic large cell lymphoma in a patient with SLE. This article discusses the association of SLE and lymphoma, with an emphasis on T-lymphoproliferative states.

Adult↗

The BILAG index: a reliable and valid instrument for measuring clinical disease activity in systemic lupus erythematosus.

The British Isles Lupus Assessment Group (BILAG) index is a computerized index for measuring clinical disease activity in systemic lupus erythematosus (SLE), which was developed according to the principle of the physician's 'intention to treat'. The index allocates separate alphabetic scores to each of eight organ-based systems; a total score is not calculated. This study demonstrated good between-rater reliability for the BILAG index for each organ-based system. There was no evidence of bias between observers. The BILAG index had good overall sensitivity (87%) and specificity (99%) when compared with the 'gold standard' criterion (starting or increasing disease-modifying therapy). There were high positive predictive values overall (80%), and for each organ-based system, with the exception of the neurological system.

Adolescent↗

Anti-ribosomal ribonucleoprotein autoantibodies in systemic lupus erythematosus.

Sera from systemic lupus erythematosus (SLE) patients giving a fluorescent ribosomal pattern on tissue and cell preparations also showed precipitating autoantibodies against purified rat liver ribosomes. Ribosomal antigen is also present in rabbit thymus cellular extract (RTE), since the same sera gave precipitin lines against RTE in identity with ribosomes. Immunofluorescent staining was completely inhibited by serum absorption with ribosomes or with RTE. However ribosomal RNA and RNase or trypsin-treated ribosomes failed to react with these autoantibodies as demonstrated in immunoabsorption and immunodiffusion studies. These data suggest that these sera contain autoantibodies directed against some antigenic site composed of a portion of both RNA and ribosomal protein. Ribosomal autoantibodies were detectable at a low frequency in SLE patients characterized by an active disease and renal involvement.

Antigens↗

Laboratory criteria for a diagnosis of systemic lupus erythematosus.

Patients with systemic lupus erythematosus (SLE) and other connective tissue diseases were surveyed for the presence of antibodies to normal DNA, antibodies to a ribonuclease-insensitive acidic nuclear protein, and immune deposits in normal skin. While 80% of patients with SLE had abnormal values for at least two of these three tests, none of the patients with other connective tissue diseases had more than one abnormal value. The presence of RBC autoantibodies was found along with one of the other abnormal laboratory tests in 76% of patients with SLE, including 14% of patients not found to have two abnormal tests. None of the other patients tested had RBC autoantibodies. These findings suggest that a set of laboratory tests can be constructed as criteria for a diagnosis of SLE that would be as specific as the presently employed American Rheumatism Association criteria.

Antibodies, Antinuclear↗

Cloning and nucleotide sequence of cDNA for Ki antigen, a highly conserved nuclear protein detected with sera from patients with systemic lupus erythematosus.

Patients with systemic lupus erythematosus (SLE) produce autoantibodies against a variety of nuclear antigens including Ki antigen. Although anti-Ki autoantibodies were found in a significant number of SLE patients, the nature of Ki antigen is poorly characterized. By using anti-Ki serum as a probe we have cloned a bovine cDNA directing the synthesis in Escherichia coli of a polypeptide immunologically indistinguishable from the authentic Ki antigen. A homologous human cDNA was also cloned and its nucleotide sequence predicted the entire primary structure of a novel nuclear protein with a molecular weight of 29 508 and with highly hydrophilic and weakly acidic character. The gene is highly conserved not only in the coding region but also in the 3'-untranslated region. The bacterially produced Ki antigen would be valuable for diagnosis of SLE.

Amino Acid Sequence↗

Restriction fragment length polymorphism (RFLP) analysis in the TNF genes of patients with systemic lupus erythematosus (SLE).

Although systemic lupus erythematosus (SLE) is known to be positively associated with certain major histocompatibility complex (MHC) class I and/or class II antigens, it is not clear whether the MHC genes are the predisposing genes of the disease rather than markers for other closely linked gene(s). Because of the involvement of tumor necrosis factor (TNF) in the inflammation process and localization of the TNF genes in the proximity of the HLA-B locus, we studied the restriction fragment length polymorphism (RFLP) of the TNF-alpha and -beta genes in 20 SLE patients and 23 normal individuals using restriction endonuclease NcoI. The frequency of a 5.5 kb NcoI fragment from SLE patients was significantly higher than that from normal controls. This result suggests that the polymorphic TNF genes may be involved in the pathogenesis of SLE.

Alleles↗

Determinants of cognitive performance in systemic lupus erythematosus.

Patients with systemic lupus erythematosus (SLE) may have a variety of neuropsychiatric syndromes. Assessment of cognitive functioning for these patients is complicated by increased prevalence and disease severity among groups obtained lower scores on measures of cognitive functioning in normative national samples. Cognitive ability was quantified in a diverse cohort of patients with SLE and a demographically matched group of control participants. Hierarchical regression demonstrated a small increase (6%) in explained variation in cognitive functioning when presence of SLE was added to the equation derived from demographic variables. No significant interaction was found between race and disease. These results suggest that increased frequency of cognitive impairment in African Americans with SLE is due to the additive effects of psychosocial variables.

Adult↗

[Association of systemic lupus erythematosus and dermatopolymyositis].

Systemic lupus erythematosus (SLE) associated with dermatopolymyositis (DM). This association is rare. Diagnosis may be difficult because of their common clinical findings. We report here a case. A 22-year-old man was admitted for arthritis with fever, diffuse myalgia and periorbital skin heliotrope rash. Electromyogram and muscular biopsy were suggestive of DM. The patient was treated with oral prednisone. Two months and a half later, he was admitted for impure nephrotic syndrome in relation with diffuse proliferative glomerulonephritis. Antibodies against native double-stranded- DNA were positive, and normal skin biopsy showed immune complex deposit on dermo-epidermic junction, suggestive of SLE. The patient was treated with high doses of prednisone and 6 monthly intravenous pulses of cyclophosphamide. Skin lesions and nephrotic syndrome improved. Presently, the patient remains asymptomatic. While being of different pathogenesis, SLE and DM may coexist in the same patient.

Adult↗

Phenotypic and functional deficiencies of monocyte-derived dendritic cells in systemic lupus erythematosus (SLE) patients.

Systemic lupus erythematosus (SLE) represents an autoimmune disease for which alterations of T cells, B cells as well as various antigen-presenting cell (APC) populations have been described. In order to better define APC-associated deficiencies, we analyzed morphologic, phenotypic and functional characteristics of in vitro-generated monocyte-derived dendritic cells (MoDC) from SLE patients as compared with healthy controls. Analysis of MoDC at different stages of maturation revealed substantial phenotypic and functional defects of MoDC from SLE patients as compared with healthy controls. In particular, we observed a significantly reduced up-regulation of MHC class II molecules on MoDC upon activation which correlated with disease activity scores and functional deficiencies in mixed lymphocyte reaction experiments. Our data imply a crucial role of APC in the immunological imbalance in SLE for foreign and self-antigen reactivity.

Adult↗

Pulmonary manifestations of systemic lupus erythematosus: review of twelve cases of acute lupus pneumonitis.

Acute lupus pneumonitis was the presenting manifestation of systemic lupus erythematosus in six of 12 cases in this series. The clinical picture was characterized by severe dyspnea, tachypnea, fever and arterial hypoxemia. Radiographic findings included an acinar filling pattern which was invariably found in the lower lobes and was bilateral in 10 of the cases. Studies failed to reveal evidence of infection as a cause of the acute pulmonary infiltrates. All patients were treated with oxygen and corticosteroids; seven received azathioprine. Six patients survived and are clinically well 14 months to four years following their acute illness. Three of these patients have residual interstitial infiltrates with persistent pulmonary function test abnormalities indicating progression to chronic interstitial pneumonitis. Histologic sections of the lungs available from four patients revealed hyaline membranes and interstitial edema (four cases), acute alveolitis (two cases), arteriolar thrombosis (one case) and a prominent lymphocytic interstitial pneumonitis with organizing bronchiolitis (one case).

Acute Disease↗

The fibrinolytic response to venous occlusion and the natural anticoagulants in patients with antiphospholipid antibodies both with and without systemic lupus erythematosus.

Patients with systemic lupus erythematosus (SLE) have an increased risk of thrombosis and this is increased in the presence of antiphospholipid antibodies (APA). These APA are also associated with thrombosis in patients who do not have SLE. We compared haemostatic parameters in SLE patients with and without APA, and also compared patients who had APA but not SLE with healthy normal controls. No relationships between the natural anticoagulants, antithrombin III, heparin cofactor II, protein C and protein S, and the presence of APA were found. In the patients with SLE both tissue plasminogen activator antigen and plasminogen activator inhibitor (PAI) were increased, but these changes were not due to APA which had no effect on fibrinolysis in these patients. In the patients with APA who did not have SLE the fibrinolytic response to venous occlusion was reduced due to raised levels of PAI; similar changes have, however, been reported in some patients with idiopathic thrombosis.

Adolescent↗

Osteonecrosis in systemic lupus erythematosus, steroid-induced or a lupus-dependent manifestation?

Osteonecrosis (ON) is a well-known complication in patients with systemic lupus erythematosus (SLE) often associated with steroid therapy. In a cohort of 280 SLE patients followed over the last 10 years, seven patients developed symptomatic ON, one of them after septic arthritis of the hip. Two other patients developed ON several years after discontinuing steroids. One patient developed ON of both humeral and femoral heads within a few months after the diagnosis of SLE. When we compared the cumulative steroid doses taken by our patients with those described in other reports (43,700 mg and 45,300 mg, respectively), our patients received less steroids (38,834 mg). We found no increased frequency of Raynaud's phenomenon, leukopenia, anti-phospholipid antibodies, or a flare of SLE activity in our patients with ON, factors which have been reported to be associated with ON. Various pathogenic mechanisms, which could lead to ON in SLE patients are discussed.

Adrenal Cortex Hormones↗

Salmonella infection in systemic lupus erythematosus.

Patients with systemic lupus erythematosus (SLE) are susceptible to infections, notably salmonellosis. We report 37 cases of salmonella infection in 24 patients with SLE. These cases were detected in a group of 770 patients with SLE. All the patients were women, with a mean age of 25.6 years. At the onset of salmonella infection, 81% were taking prednisolone and 27% were taking cytotoxic drugs. Renal involvement was present in 75% of patients, which was approximately the same as in other SLE patients. Bacteremia, arthritis, osteomyelitis and rare manifestations of salmonellosis, including pulmonary and urinary tract involvement, were encountered. Diagnosis was based on isolation of the microorganism, mostly from blood cultures. Salmonella species other than typhi and paratyphi were often responsible. Widal agglutination test was positive in less than half the cases, and leukopenia was not seen frequently. Recurrence of infection in 29% of the patients and the high mortality rate (28.5%), despite the conventional period of appropriate antimicrobial treatment, show a poor prognosis of salmonellosis in SLE patients. This special picture of salmonellosis argues for a much longer period of treatment in these patients.

Adolescent↗