bluuebunny/crossref_metadata_2025 huggingface dataset:
Heart rate variability and cardiac autonomic function in systemic lupus erythematosus
Authors: Lagana' B. , Tubani L. , Maffeo N. , Vella C. , Makk E. , Baratta L. , Bonomo L.
Published: November 2007

The cardiac autonomic function was evaluated in 23 patients with Systemic Lupus Erythe matosus (SLE) without clinical expression of dysautonomia and in 14 healthy volunteer subjects as a control group, by analysis of Heart Rate Variability (HRV) from 24 h ambula tory electrocardiography. All the patients were taking corticosteroids and 10 of them also Ciclosporin A (CsA). The following parameters of HRV were performed: Time domain: standard deviation of the RR intervals average (SDNN) and percentage of RR adjacent intervals differing from each other more than 50 msec (pNN50). Frequency domain: low frequencies (LF) and high frequencies (HF).

Significant lower values were detected in SLE patients vs controls: SDNN = 69.40 vs 127.72; pNN50 = 16.44 vs 25.95; LF = 8.34 vs 34.97; HF = 3.21 vs 12.18. The incidence of autonomic dysfunction in our SLE population evaluated by considering intervals of nor mality is approximately 78% for SDNN; 17% for pNN50; 91% for LF and, finally, 56% for HF. The analysis of HRV may be a valuable technique in the study of the incidence of dysautonomia for these patients.
Valvular heart disease in primary antiphospholipid syndrome (PAPS): clinical and morphological findings
Authors: García-Torres R. , Amigo M-C. , de la Rosa A. , Morón A. , Reyes PA
Published: November 2007

Purpose. To describe clinically and pathologically the valvular lesion of the primary anti phospholipid syndrome (PAPS).

Patients and Methods. We studied six patients with PAPS and valvulopathy. Four of them died and had autopsy and two had valvular replacement. The study comprised 18 heart valves, 16 from autopsy and two, one mitral and one aortic, resected at surgery. Results. Murmurs and echocardiographic findings kept correlation with gross pathology. Abnormalities were found in one or more valves in all patients including two of five aortic, two of five mitral, one of four pulmonary and two of four tricuspid. Co-existence of new and old lesions was observed. Pathologic findings included intravalvular thrombosis with focal necrosis and hemorrhage, vascular proliferation, mild histiocytic/fibroblastic infiltration, laminated and verrucous fibrin deposits, laminated and/or nodular fibrosis, and focal calcifi cation.

Conclusion. The PAPS valvular lesion consists mainly of superficial or intravalvular fibrin deposits and its subsequent organization: vascular proliferation, fibroblast influx, fibrosis and calcification. This results in valve thickening, fusion and rigidity leading to functional abnormalities. Inflammation is not a prominent feature of this lesion.
Laboratory detection of antiphospholipid antibodies
Authors: Kandiah DA , Krilis SA
Published: November 2007

'Antiphospholipid' (aPL) antibodies are a heterogenous group of autoantibodies that are now considered to be directed mainly to plasma proteins or protein-phospholipid complexes 1. Standardisation of assays for anticardiolipin (aCL) antibodies and lupus anti coagulants (LA) have been fraught with difficulty despite numerous attempts to perform this by International Standardisation Workshops and Committees.
Clinical manifestations and management of the antiphospholipid syndrome
Authors: Derksen Rhwm
Published: November 2007

Since the original description of associations between lupus anticoagulant and anti cardiolipin antibodies with thrombosis, fetal loss and thrombocytopenia, several distinct clinical manifestations related to this so-called antiphospholipid syndrome have appeared in the literature. Retrospective studies indicate that long-term treatment with oral anticoagu lants can effectively reduce the chance of recurrent thrombosis. However, data from con trolled prospective studies are urgently needed to enable optimal counselling of patients suffering from this syndrome.
Neuropsychiatric manifestations in patients with systemic lupus erythematosus: Diagnostic and predictive value of longitudinal examination of anti-ribosomal P antibody
Authors: Watanabe T. , Sato T. , Uchiumi T. , Arakawa M.
Published: November 2007

Anti-ribosomal P (anti-P) antibodies (IgG, IgA, IgM) were measured by an enzyme-linked immunosorbent assay (ELISA) in serial serum samples from the patients with systemic lupus erythematosus (SLE), and these clinical significance was investigated. Neuropsychi atric manifestations were observed in 24 of 144 patients with SLE. Twelve patients had lupus psychosis, nine convulsion or coma and three primary schizophrenia. In the group with lupus psychosis, serum C3 was significantly higher ( p < 0.05) and anti-ds-DNA anti body was significantly lower ( p < 0.05) than in the other patients. The titer of anti-P anti bodies was significantly higher in the group with lupus psychosis than that of the other patients. In a longitudinal study, IgA and IgM classes of anti-P antibody were elevated at the onset of psychosis. The titer decreased following a remission of psychosis. Therefore, the measurement of immunoglobulin classes of anti-P antibody will be useful for the diagnosis and follow-up of patients with lupus psychosis.
The role of HLA-DR alleles and complotypes through the ethnic barrier in systemic lupus erythematosus in Mexicans
Authors: Granados J. , Vargas-Alarcón G. , Andrade F. , Melin-Aldana H. , Alcocer-Varela J. , Alarcón-Segovia D.
Published: November 2007

Alleles of the major histocompatibility complex (MHC) have been recognized as genetic factors for the development of SLE. The [HLA-B8; SC01; DR3] extended haplotype seems to be relevant in patients from white European descent, pertinent alleles, however, are diffi cult to select on haplotypes with linkage disequilibrium. Studies in non-Caucasian patients are therefore mandatory. Admixture estimates in Mexicans have shown a proportion of 56% of Indian genes, 40% of Caucasian genes and from 4 to 12% of Black genes. In order to determine the relevant MHC loci in the genetic susceptibility for SLE we studied Class I, II and III alleles in 102 Mexican SLE patients and 350 of their first degree relatives and compared these two groups to another one composed by 200 ethnically matched normal individuals. We found significantly increased frequencies of HLA-DR3 (pC = 0.03, RR = 2.56) and DR7 (pC = 0.004, RR = 3.08) in SLE patients as compared to controls. On the other hand, first degree relatives had a significantly increased frequency of HLA-DR7 (pC = 0.01, RR = 2.98). There were 21 out of 33 HLA-DR3 haplotypes with complotypes other than SC01 and 25 out 37 SC01 haplotypes with DR alleles other than DR3. Neverthe less, [SC01; DR3] haplotypes were also increased (pC = 0.01, RR = 12.4). After removing [HLA-B8; SC01 ; DR3] haplotypes, DR3 was the only allele that remained significantly increased (p = 0.04, RR = 2.1). We also found in SLE patients significantly decreased fre quencies of the autochthonous Mexican alleles (A30, B39 and DR4) and no deviation from normality of any of the HLA-DQ alleles. These data suggest a fundamental role of the HLA-DR3 allele in the predisposition to SLE in Mexican patients which might be high tened by genes located around the class III MHC region. They also substantiate the perti nence of ethnic admixture estimates in modern human populations.
Binding affinity and quantity of estrogen receptor in peripheral blood monocytes of patients with systemic lupus erythematosus
Authors: Suenaga R. , Mitamura K. , Evans MJ , Abdou NI
Published: November 2007

Binding affinity and quantity of the estrogen receptor in monocytes of patients with sys temic lupus erythematosus (SLE) were studied. The tritiated-estradiol binding assay was performed using peripheral blood adherent cells (>95% monocytes) derived from six lupus patients (SLEDAI score: 2-30) and five age-comparable normal women during the mid- follicular phase of the menstrual cycle. Dissociation constant (Kd) and number of binding sites (Ro) were estimated by Scatchard analysis. The specificity and sensitivity of the assay were verified by using estrogen receptor-positive ZR75-1 human breast cancer cells. Kd and Ro of the type I receptor for the SLE patients were 12.2 ± 6.5 (nM) and 69.0 ± 42.4 ( x 1000/cell), respectively, while those of the normals were 14.5 ± 3.7 and 86.8 ± 23.4, respectively. Three patients displayed relatively low Kd or Ro values. While those low values fell within the mean —3 s.d. of the normal controls, precise statistical comparison was not possible. No clear correlation between the receptor parameters and the SLEDAI scores was noted. Although further studies of a larger number of samples are needed to conclude, these results suggest that peripheral blood monocytes of SLE patients express the estrogen receptor whose Kd and Ro are similar to those of normals.
Review : Restriction fragment length polymorphisms of constant region genes of immunoglobulin lambda chains in Venezuelan patients with systemic lupus erythematosus
Authors: Blasini AM , Delgado MB , Valdivieso C. , Guevara P. , Ramirez JL , Stekman IL , Rodriguez MA , Williams RC
Published: November 2007

Previous studies suggest a potential association between human immunoglobulin (Ig) genes and susceptibility to systemic lupus erythematosus (SLE). Ig allotypic determinants seem to confer an increased risk for the disease in various ethnic patient populations. In this study we have examined the pattern of restriction fragment length polymorphisms (RFLP) of constant region lambda (Cλ) light chain genes in a group of 78 Venezuelan patients with SLE and 70 healthy controls. The frequency of the 8-kb allele and the 8/8 genotype was significantly lower in normal Venezuelan controls as compared to healthy British Cauca sians ( P = 0.0002 and 0.0007 respectively). In turn, Venezuelan controls showed a higher frequency of the 18-kb allele and the 18/18 genotype ( P = 0.0002 and 0.0052 respectively). However, there were no statistically significant differences in either parameter between Venezuelan SLE patients and healthy controls. Our study argues against a role for lambda light chain constant region genes in predisposition to SLE.
Review : Factor V Arg506 → Gln mutation in patients with antiphospholipid antibodies
Authors: Montaruli B. , Borchiellini A. , Tamponi G. , Giorda L. , Bessone P. , van Mourik JA , Voorberg J. , Schinco P.
Published: November 2007

Antiphospholipid antibodies (APA) have thought to be implicated in the pathogenesis of both arterial and venous thrombosis. Because of heterogeneity of APA, direct evidence of their involvement in a thrombotic event is not yet available. Development of thrombosis in the antiphospholipid antibody syndrome (APS) may occur because of the presence of addi tional risk factors. Here we have analysed 60 patients with APA for the presence of the Arg506 → Gln mutation in factor V. Among them 26 suffered from deep venous thrombosis, 13 from arterial thrombosis and 21 had no history of arterial or venous thrombosis. In the first group four patients were found to be heterozygous and one homozygous for the factor V Arg506 → Gln mutation. None of the patients with the factor V mutation was found in the second and third group. The incidence of factor V mutation was significantly elevated in the group of patients with venous thrombosis. These data suggest that in patients with anti phospholipid antibodies the factor V Arg506 → Gln mutation may play a major role in the occurrence of venous thrombosis.
Review : Antiphospholipid antibodies and retinal vascular disease
Authors: Dunn JP , Noorily SW , Petri M. , Finkelstein D. , Rosenbaum JT , Jabs DA
Published: November 2007

Background. Antiphospholipid antibody retinopathy is a rare and poorly defined clinical entity in which the proper diagnosis has important ocular and systemic implications. The clinical course of five patients with antiphospholipid antibody retinopathy is described and the role of such antibodies in idiopathic retinal venous occlusive disease is investigated.

Methods. Five case reports are presented highlighting the presentation, course, therapy, associations and outcomes of patients with antiphospholipid antibody retinopathy. In addi tion, twenty additional patients presenting with idiopathic retinal venous occlusive disease were tested for the presence of antiphospholipid antibodies.

Results. All five patients with antiphospholipid antibody retinopathy had diffuse retinal vascular occlusion. All five patients presented with associated rheumatologic disease, includ ing three with lupus or lupus-like disease. Antibodies to antiphospholipid were not detected in any of the twenty patients with idiopathic retinal venous occlusive disease. Prompt pan- retinal photocoagulation together with varying regimens of corticosteroids, immunosup pressives, or warfarin was partially successful in stabilizing the ocular and systemic disease.

Conclusion. The diagnosis of antiphospholipid antibody retinopathy should be suspected in patients with diffuse retinal vaso-occlusion, particularly when characterized by involvement of both arteries and veins, neovascularization at presentation, and symptoms of systemic rheumatologic disease. Antiphospholipid antibodies do not appear to play an important role in idiopathic retinal vein occlusions.
Review : Osteonecrosis in systemic lupus erythematosus, steroid-induced or a lupus-dependent manifestation?
Authors: Rascu A. , Manger K. , Kraetsch H-G. , Kalden JR , Manger B.
Published: November 2007

Osteonecrosis (ON) is a well-known complication in patients with systemic lupus erythema tosus (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 fre quency 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.
Review : Modulation of experimental systemic lupus erythematosus with linomide
Authors: Zandman-Goddard G. , George J. , Levy Y. , Blank M. , Slavin S. , Shoenfeld Y.
Published: November 2007

Objective. The objective of this study was to assess the beneficial effects of an early adminis tration of low dose linomide, a new immunomodulator, in an animal model of experimental systemic lupus erythematosus (SLE).

Methods. Experimental SLE was induced in naive BALB/c mice, by immunization with anti-DNA mAb (MIV-7). Control Mice immunized with irrelevant human IgM served as controls. The immunized mice were treated with linomide (0.1 mg/ml in the drinking water), four weeks prior to the first immunization, at an early stage of the disease induction (one month after boost injection), or at a later stage (3 months following boost immunization). The treatment duration was 3 months in all schedules. The follow-up studies continued for 8 weeks after discontinuation of the treatment.

The presence in the serum of autoantibodies against ssDNA, dsDNA, histones, phospho lipids and an irrelevant autoantigen - pyruvate dehydrogenase, was determined by enzyme- linked immunosorbent assay (ELISA). The clinical parameters assessed included erythrocyte sedimentation rate, peripheral blood cell counts and proteinuria.

Results. There was a 50-64% decrease in autoantibody levels in the sera of mice immunized with anti-DNA (MIV-7) mAb at the early stage of experimental SLE in mice which received linomide for a period of 3 months. No effect of linomide was noted in mice which received the drug during the later stages of experimental SLE when the disease was fully developed. Linomide had a preventive effect on the induction of experimental SLE in naive mice, when the treatment was initiated before the induction of the disease. This effect was abolished following cessation of the treatment.

Conclusions. Linomide proved to be effective at the early stages of induction of the experi mental SLE. However, the autoantibody levels rose following discontinuation of the therapy.
TNF microsatellites in systemic lupus erythematosus—a high frequency of the TNFabc 2-3-1 haplotype in multicase SLE families
Authors: Sturfelt G. , Hellmer G. , Truedsson L.
Published: November 2007

TNFa, TNFb and TNFc microsatellites were analyzed in 102 SLE patients recruited from a defined area in Southern Sweden. Furthermore we studied 27 SLE patients belonging to 10 multiplex families in which a majority of the members were living within the same area in Southern Sweden. As a control population 98 healthy blood donors from the same region was used. The TNFa2 allele was found more often in the SLE patients (48%) than in the normal controls (33%) (P < 0.01). A low frequency of the TNFa4 allele (10%) vs 16% in controls was observed. The family study showed that the TNFabc haplotype 2-3-1 was more common in SLE associated haplotypes than in non-SLE haplotypes (P < 0.001). The 2-3-1 haplotype was associated with the extended haplotype MHC haplotype [HLA-B8,SC01,DR17]. The results suggest that TNF haplotypes do not constitute special markers of susceptibility to SLE but reflect the increased frequencies of specific intact haplotypes already known to be associated with the disease.
Anti-Ro(SS-A) antibody positive Sjögren's/lupus erythematosus overlap syndrome
Authors: Provost TT , Watson R. , Simmons-O'Brien E.
Published: November 2007

Unlike anti-Ro(SS-A) antibody positive subacute cutaneous lupus erythematosus (SCLE) patients, anti-Ro(SS-A) antibody positive lupus patients with Sjögren's syndrome (SS/LE) appear to have a more guarded prognosis. Our studies indicate these SS/LE patients have an increased frequency of pulmonary, neurological, and renal disease compared to SCLE patients.
Systemic lupus erythematosus in Malaysia: a study of 539 patients and comparison of prevalence and disease expression in different racial and gender groups
Authors: Wang F. , Wang C.L. , Tan C.T. , Manivasagar M.
Published: November 2007

The aims of this study were to examine the clinical and laboratory features of Malaysian patients with systemic lupus erythematosus (SLE) and to identify any difference in disease expression between the different genders and among the three major ethnic groups of Malaysia. Retrospective analysis of all patients with SLE admitted to and followed-up at University Hospital Kuala Lumpur from 1974—90 was undertaken. Ethnic Chinese had the highest prevalence of SLE compared to other ethnic groups. There was a high incidence of renal disease, 74% of patient had significant proteinuria and half of these had associated nephrotic syndrome. Indian patients had significantly less incidence of skin manifestation compared to other racial groups. No difference in disease expression was detected between the ethnic Chinese and Indians and between the male and female patients. The overall 5 y and 10 y survival rates were 82% and 70% respectively. Indian patients had the poorest survival rates. Survival rates are similar among the Chinese and Malay patients. Our findings are in broad agreement with those previously reported.
Effect of β2glycoprotein I and human monoclonal anticardiolipin antibody on the protein S/C4b-binding protein system
Authors: Atsumi T. , Khamashta MA , Ames Prj , Ichikawa K. , Koike T. , Hughes Grv
Published: November 2007

The effect of β2glycoprotein I (β2GPI) and human monoclonal anticardiolipin antibody (aCL) on the protein S/C4b-binding protein (C4BP) system was evaluated. The binding of C4BP to protein S was assessed by ELISA in the presence of β2GPI with/without human monoclonal aCL. β2GPI downregulated the binding between S and C4BP significantly. Human monoclonal aCL abolished the β2GPI inhibitory effect in a calcium (Ca++) independent fashion. In separate experiments, the reactivity of aCL towards protein S in the presence or absence of β2GPI and cardiolipin was investigated. Monoclonal aCL bound to protein S only in the presence of a combination of β2GPI and cardiolipin. This binding was Ca++ dependent. These findings suggest that human monoclonal aCL increases the affinity of C4BP for protein S, and that protein S may represent one of the targets for aCL when combined with β2GPI and cardiolipin. Both issues may explain acquired free protein S deficiency and the attendant risk of thrombosis in patients with aCL.
Correlation between CD29 density on CD8 + lymphocytes and serum IgG in systemic lupus erythematosus
Authors: Pallis M. , Lim KL , Gardner-Medwin JM , Robins RA , Powell RJ
Published: November 2007

The purpose of this paper is to establish whether there is increased lymphocyte adhesion molecule density in systemic lupus erythematosus (SLE), which could alter the migration pathways and activation thresholds of lymphocytes and thus contribute to the pathogenesis of the disease. We analysed the CD11a, CD29 and CD2 bound antibody molecule (bam) density on the CD4 + and CD8 + CAMhigh (primed) lymphocytes of 28 SLE patients (8 active and 20 inactive by BILAG), using reproducible flow cytometric measurements, standardized with fluorescent beads and antibodies of known fluorescein : protein ratios. In a second patient cohort (17 patients), we investigated whether CD29 density on CD8 + cells correlated with measures of humoral (serum IgG) or cellular (urine neopterin) activation. In the first cohort, 36% of patients had elevated CD29 (β1 integrin) density on CD8 + cells. In the second cohort, CD29 density on CD8 + cells was found to be closely associated with total plasma IgG ( r = 0.71, P = 0.001), but not with urine neopterin, disease activity (BILAG) or drug treatment. We conclude that CD29 on CD8 + cells is associated with B cell activation in SLE.
Successful treatment of early secondary myelofibrosis in SLE with IVIG
Authors: Aharon A. , Levy Y. , Bar-Dayan Y. , Afek A. , Zandman-Goddard G. , Skurnik Y. , Fabrrizzi F. , Shoenfeld Y.
Published: November 2007

Myelofibrosis has been reported as a rare cause of pancytopenia in patients with autoimmune diseases. We describe a 54 y old female patient who was admitted with severe anemia subsequently found to be due to marrow fibrosis. During the course of her hospitalization, relying both on her clinical symptoms as well as the results of a wide range of laboratory tests and diagnostic procedures, the diagnosis of systemic lupus erythematosus was established. The patient was treated with high dose steroids, but improvement of her clinical symptoms as well as normalization of her peripheral blood count were achieved only after high dose intravenous therapy with gamma globulin (IVIG) was instituted. Along with the improvement in the peripheral blood parameters normalization of the bone marrow architecture was recorded on a repeated bone marrow biopsy. IVIG therapy should be considered in extreme cases of bone marrow suppression in SLE.
Autonomic nerve dysfunction in systemic lupus erythematosus: evidence for a mild involvement
Authors: Altomonte L. , Mirone L. , Zoli A. , Magaro M.
Published: November 2007

Neurologic manifestations are known to occur in patients with systemic lupus erythematosus (SLE) and significantly affect the clinical course of the disease. Nevertheless, the prevalence, pattern and severity of autonomic impairment in such patients have yet to be defined.

In the present study a series of 38 female SLE patients was assessed for the presence of autonomic dysfunction. Five noninvasive standardized cardiovascular reflex tests were used. The grading system proposed by Ewing and Clarke was applied to classifying autonomic impairment according to severity. Seventeen out of 38 patients, that is 44.7%, had evidence of autonomic impairment. Most of the patients had a mild degree of dysfunction. No correlation was found for the duration of the disease while an apparent lack of the commonly described chronological sequence of autonomic involvement was observed.

We suggest that in SLE patients the prevalence of autonomic impairment, when investigated, does not significantly differ from that of other SLE-associated neurological events. The contribution of a direct immunological damage to components of neural pathways in the pathogenesis of the autonomic involvement can be postulated. Clinical consequences of autonomic impairment in patients with systemic lupus erythematosus need to be elucidated.
Pulmonary hemorrhage in systemic lupus erythematosus
Authors: Barile LA , Jara LJ , Medina-Rodriguez F. , Garcia-Figueroa JL , Miranda-Limón JM
Published: November 2007

In order to assess the clinical characteristics and survival rate of pulmonary hemorrhage (PH) in systemic lupus erythematosus (SLE), we studied SLE patients who developed this complication. We found 34 patients within a total lupus cohort of 630 patients.

A11 the patients had severe respiratory failure. We identified three different treatment regimens: (a) oral prednisone (1 mg/kg); (b) conventional methylprednisolone (3 g total dose) and (c) massive methylprednisolone ( > 4 g).

The overall survival rate was 38.2% and it was correlated with the massive regimen and early treatment (within the first 48 h after the onset of the acute event).
Review : A peculiar form of amicrobial pustulosis of the folds associated with systemic lupus erythematosus and other auto-immune diseases
Authors: Lagrange S. , Chosidow O. , Piette JC , Wechsler B. , Godeau P. , Frances C.
Published: November 2007

Amicrobial pustulosis of the folds is a new entity which was initially described in association with systemic lupus erythematosus (SLE). We report four cases of this rare pustulosis occurring in the course of various auto-immune disorders.

Dermatological features were similar in all patients. The primary lesions were small pustules which coalesced and formed erosive areas underlined with pustular lesions. They were localized in the folds. Three of the four patients had also isolated pustules on the scalp. Spongiform pustules were histologically present. A primary bacterial or fungal infection was ruled out by cultures on appropriate media. Serum calcium level was normal. Associated auto-immune diseases were: systemic lupus erythematosus, incomplete systemic lupus erythematosus, 'idiopathic' thrombocy topenic purpura and myasthenia gravis without thymoma (one of each). The relationship between this peculiar form of pustulosis and autoimmunity remains to be elucidated. The pustulosis did not respond to antibiotics but disappeared with systemic or topical corticosteroids.
Review : Anti-endothelial cell antibodies in systemic autoimmune diseases: prevalence and clinical significance
Authors: Navarro M. , Cervera R. , Font J. , Reverter JC , Monteagudo J. , Escolar G. , López-Soto A. , Ordinas A. , Ingelmo M.
Published: November 2007

Objective: To investigate the prevalence and characteristics of anti-endothelial cell antibodies (AECA) in a large cohort of patients with several well defined systemic autoimmune diseases, in order to determine their relationship with the clinical and laboratory features of these diseases.

Methods: Clinical and laboratory features of 216 consecutive Caucasian patients were prospectively studied. One hundred and seven patients had been diagnosed as having a primary systemic vasculitis-specifically, 39 had temporal arteritis (TA), 25 polyarteritis nodosa (PAN), 9 Wegener's granulomatosis (WG), and 34 Behçet's disease (BD)-, 90 patients had systemic lupus erythematosus (SLE), and 19 had a primary Sjögren's syndrome (SS). The AECA were determined by ELISA.

Results: One hundred and four (48%) patients with systemic autoimmune diseases were found to have a positive titre of AECA. Specifically, AECA were detected in 41 (38%) patients with a primary systemic vasculitis (13 (33%) with TA, 14 (56%) with PAN, 5 (56%) with WG and 9 (26%) with BD), in 58 (63%) patients with SLE, and in 5 (26%) patients with a primary SS. In patients with a primary systemic vasculitis, those with AECA were found to have an increased prevalence of disease activity ( P < 0.05). In SLE patients, those with AECA were found to have an increased prevalence of vascular lesions ( P < 0.05), lupus nephropathy ( P < 0.05), and antic ardiolipin antibodies (aCL) ( P < 0.001).

Conclusions: Patients with systemic autoimmune diseases have a high prevalence of AECA and they are associated with the presence of vascular lesions, nephropathy, and aCL in SLE, as well as with disease activity in several primary systemic vasculitis (TA, PAN, WG and BD).
Autologous marrow stem cell transplantation for severe systemic lupus erythematosus of long duration
Authors: Marmont AM , van Lint MT , Gualandi F. , Bacigalupo A.
Published: November 2007

A 46 year woman with severe long-lasting SLE received an autologous bone marrow transplantation utilising CD34+ haematopoietic progenitors following a 3 log T-lymphocyte depletion. The immunosuppressive regimen (conditioning) consisted of 15mg/kg Thiotepa followed by 100 mg/kg of cyclophosphamide over 2 d. Granulocytic recovery was aided by G- CSF. The post-transplant course was uneventful, and a good clinical and immunologic remission (ANA negativisation) was achieved. This is the first case of SLE having received an autologous progenitor cell transplant for the autoimmune disease by itself, unaccompanied by a haematologic condition requiring transplantation.

The potential, advantages and limits of this procedure, which are currently being explored worldwide, are briefly discussed.
Central vein thrombosis in the antiphospholipid syndrome
Authors: Alcazar-Ramirez JD , Femández-Nebro A. , Abarca M. , Salgado F. , De Haro-Liger M. , Rodriguez-Andreu J. , González-Santos P.
Published: November 2007

We report the case of a 38 y old man with antiphospholipid syndrome and exceptionally extensive central vein thrombosis in the right internal jugular vein, superior vena cava, and both subclavian veins. In spite of intensive anticoagulation therapy there was only a partial response. We suggest the MR angiography be the reference standard for diagnosis in this type of patient.
Thrombosis associated with antiphospholipid antibody in juvenile chronic arthritis
Authors: Andrews A. , Hickling P.
Published: November 2007

Anticardiolipin antibodies (aCL) have been reported in patients with juvenile chronic arthritis (JCA) without associated thrombotic events. We describe a patient with longstanding JCA who developed central retinal vein occlusion in the presence of antiphospholipid antibodies (APL).
Subclinical multisystemic autoimmunity presenting as a progressive myelopathy
Authors: Linardaki G. , Skopouli FN , Koufos C. , Moutsopoulos HM
Published: November 2007

Autoimmunity can manifest clinically in many ways; however, despite the various efforts to classify autoimmune disorders into specific disease entities, the borders between these disorders remain, in many cases, unclear.

In this report we describe a young woman with subclinical Sjögren's syndrome and biliary cirrhosis, who presents clinically with symptoms exclusively from the central nervous system. This neurological syndrome is consistent with a progressive myelopathy. Although the patient has a serologically and histologically confirmed multisystemic autoimmune disorder, she fulfills none of the classification criteria for the diagnosis of a specific connective tissue disease.
Elevated soluble fas production in SLE correlates with HLA status not with disease activity
Authors: Rose LM , Latchman DS , Isenberg DA
Published: November 2007

Evidence from animal models of lupus suggests that disruption of Fas-mediated apoptotic events may play a role in systemic lupus erythematosus (SLE). The recently described secreted form of Fas (sFas) could interfere with apoptotic events by blockading Fas/Fas ligand interactions. We describe elevated secreted Fas protein in sera from 60 patients with SLE compared with controls but neither sFas protein nor sFas mRNA levels correlated with disease activity. At the mRNA level there is strong evidence that individuals with human leucocyte antigens common in SLE patients have a genetic predisposition for increased secreted Fas production.
Development of systemic lupus erythematosus in a rheumatoid arthritis patient with anti-ribosomal P protein antibody
Authors: Hamasaki K. , Mimura T. , Kanda H. , Morino N. , Yazaki Y. , Nojimal Y.
Published: November 2007

We describe a patient with rheumatoid arthritis (RA) whose sera contained a high titre of an antibody targeting cytoplasmic ribosomal P proteins (anti-P). This association preceded by 6 years the development of serological and clinical manifestations of systemic lupus erythematosus (SLE). The clinical significance of anti-P for the diagnosis of SLE is discussed.
Professional Nurses Should Have Their Own Ethics: the Current Status of Nursing Ethics in the Dutch Curriculum
Authors: Kanne Mariël
Published: November 2007

Should nurses have their own ethics to match specific problems met in their daily routines? How do nurses act in a society that is changing from a 'monocultural' to an 'intercultural' structure? What are the ethical consequences of these changes for their many tasks? How can the ethical aspects be taught to nurses? This article describes the current status of nursing ethics in the curriculum taught in schools of higher education for nurses in The Netherlands. Aspects of the debate on nursing ethics are outlined and ideas about its future as a subject and discipline in nurse education are offered.

Est-ce que le personnel infirmier doit avoir son éthique à soi pour faire face aux problèmes spécifiques dans la routine journalière? Et comment les infirmiers/ères se comportent-ils dans une société qui est en train de changer de la 'monoculture' en 'interculture'? Quels sont les conséquences éthiques de leur tâches? Comment peuvent ces aspets éthiques être enseignés aux infirmiers/ères? Cet article parle de l'état courant de l'éthique infirmier dans l'enseignement dans les écoles de l'education supérieure pour infirmiers/ères en Hollande. En plus, il donne une esquisse de la discussion sur l'éthique infirmier et donne quelques idées pour le futur de l'éthique infirmier comme sujet et discipline en soi dans l'éducation des infirmiers/ères.

Sollte das Pflegepersonal seine eigene Ethik haben um den spezifischen Problemen seiner täglichen Routine gewachsen zu sein? Wie soll das Pflegepersonal in einer Gesellschaft handeln, die im Wechsel ist von einer 'Monokultur' zu einer 'Interkultur'? Was sind die ethischen Konsequenzen ihres handeln? Wie können diese ethischen Aspekte dem Pflegepersonal gelehrt werden? Dieser Artikel beschreibt den gegenwärtigen Zustand der Krankenpflege-Ethik im Lehrplan der Schulen für Höhere Ausbildung für Pflegepersonal in Holland. Er gibt auch einen Umriss über die Debatte über Krankenpflege-Ethik und formuliert einige Ideen für die Zukunft der Krankenpflege- Ethik als ein Thema und eine Disziplin in der Ausbildung des Pflegepersonals.
Building Bridges: Teaching Ward-Based Ethics
Authors: Kendrick Kevin
Published: November 2007

Ethics has traditionally been taught in the 'ivory towers' of academia. Recent develop ments and reforms in nurse education have given ethics a prominent position in most curricula. However, the vast majority of ethics teaching continues to take place in academic departments. This approach fuels the practitioner's views that nursing is a pragmatic activity whilst ethics is a cognitive endeavour; such perspectives entrench ethics firmly in the traditional gap between theory and practice. The focus of this paper presents an argument that the teaching of ethics must be versed in clinical reality if bridges are ever to be built and the theory-practice gap crossed.

Suivant la tradition, l'éthique est enseigné dans les tours d'ivoire des universités. Des développements et réformes récents dans l'éducation infirmier donnent à l'éthique une position prominante dans la plupart des programmes d'études. Mais la majorité de l'enseignement éthique continue dans les départements académiques. Cette façon d'enseigner alimente la vue des infirmiers/ères que soigner est une activité pragmatique pendant que l'éthique est un effort intellectuel. Ces attitudes retranchent l'éthique dans le vide traditionel entre la théorie et le pratique. Le thème de cet article présent l'argument que l'enseignement de l'éthique doit être posé dans la réalité clinique, si des ponts peuvent jamais être bâtis et le vide entre théorie et pratique peut être traversve.

Ethik wurde traditionsmässig im Elfenbeinturm einer Universität gelehrt. Neuliche Entwicklungen und Reformen in der Ausbildung des Pflegepersonals haben der Ethik eine prominente Position gegeben in ihren Lehrplänen. Die meiste Lehre von Ethik findet jedoch weiterhin in akademischen Abteilungen statt. Dieses Vorgehen treibt die Ansicht des praktizierenden Pflegepersonals, dass Krankenpflege eine pragmatische Aktivitat ist und Ethik eine wissenschaftliche Bemühung. Solche Ansichten verwurzeln die Ethik in der traditionellen Kluft zwischen Theorie und Praxis. Das Zentrum dieses Artikels ist die These, dass die Lehre von Ethik auf klinischer Realität basiert sein muss so dass Brücken gebaut werden können und die Kluft zwischen Theorie und Praxis überschritten werden kann.
Codes and Declarations
Authors: Thompson Anne
Published: November 2007

A regular feature in Nursing Ethics will be the 'Codes and declarations' section. Suggestions and/or contributions from readers are welcome and should be sent to the editor, Verena Tschudin, at the address shown on the inside cover.
Breast Cancer Patients' Perceived Participation in Health Care: How Do Patients Themselves and Nurses Assess this Participation?
Authors: Suominen Tarja , Leino-Kilpi Helena , Laippala Pekka
Published: November 2007

The purpose of this study was to compare breast cancer patients' perceived partici pation in their own care with nurses' perceptions of such participation. Both groups reported that patients are able and willing to take part in their own care more actively than allowed under the present health care system. Nurses also reported that they do provide patients with opportunities for participation.
Practical Ethical Issues Related To the Care of Elderly People With Dementia
Authors: Watson Roger
Published: November 2007

The care of elderly people with dementia poses ethical problems in several respects. This paper considers the problems in relation to treatment, withdrawal of treatment (including nutrition and hydration) in terminal care, and consent to involvement in research. It is ultimately the responsibility of the physician to take the decision about whether or not to proceed with treatment, according to the best interests of the patient, but nurses, families and significant others can be involved in making the decision. When withdrawal of treatment or withdrawal of nutrition and hydration in the terminal stages of dementia are being considered it is necessary to take into account the nature of the treatment, whether or not it is extraordinary, and the goals of continued nutrition. In some circumstances it may be possible for them to be withdrawn. Only research which is aimed at evaluating treatment for dementia may involve subjects who are suffering from dementia.

Soigner des personnes âgés souffrant de la démence pose plusieurs problèmes éthiques. Cet article considère les problèmes concernant le traitement, cessation de traitement (y inclus nutrition et hydration) dans la phase terminale, et consentement de participation dans les recherches. C'est ultimement la décision du docteur si oui ou non de continuer avec le traitement selon les meilleurs intérêts du malade, mais les infirmiers/ères, les familles et les personnes importantes autour du malade peuvent jouer un rôle dans les décisions. Si la cessation de traitement ou cessation de nutrition et hydration dans la phase terminale de démence est envisagée il est nécessaire de tenir compte de la nature du traitement, s'il est 'extraordinaire' ou non, et le but de nutrition si on continuait. Dans quelques circonstances il est possible de les discontinuer. Seuls les recherches qui ont pour but d'évaluer les traitements pour la démence peuvent utiliser des personnes souffrant de démence.

Die Pflege von älteren Personen mit Demenz stellt einige ethische Probleme. Dieser Artikel betrachtet die Probleme in Beziehung auf die Pflege, Zurückziehung von Pflege (auch von Nahrung und Getrank) der Sterbenden und der Entscheidung, in der Forschung beteiligt zu sein. Der Arzt hat die schlussendliche Verantwortung über die Entscheidung ob mit der Pflege weitergefahren werden soll oder nicht nach dem besten Interesse der Patienten. Aber das Krankenpflegepersonal, die Familie und bedeutende Andere können auch an dieser Entscheidung teilnehmen. Wenn es sich um Zurückziehung der Pflege, oder Zurückziehung von Nahrung oder Getrank in der Sterbepflege von Personen mit Demenz handelt, is es nötig, das Wesen der Pflege und die Ziele einer fortfahrenden Nahrung zu kennen. In gewissen Fällen ist es möglich, diese zurück zu ziehen. Es sollen nur Patienten mit Demenz an Forschungen teilnehmen die über die Pflege der Demenz einschätzt.
Achieving a Better Life On the Planet. Are We Our 'Brothers' Keepers'?
Authors: Sofaer Beatrice
Published: November 2007

All living human beings have the moral right not to be denied reasonable help in life threatening situations. Disasters take on many forms, but their consequences for individuals may be prolonged and stretch into the next generation. We have a moral obligation to create a better life for people by speaking out and trying to prevent man-made disasters and their consequences. To do this the commitment to life and its value must be reinforced. A number of suggestions are made, particularly in relation to the role of nurses.

Toutes les personnes ont le droit moral de recevoir l'aide raisonable dans des situations menaceant à la vie. Des désastres se présentent sous multiples formes, mais leur conséquences peuvent être prolongées pour des individus jusqu'à la prochaine génération. Nous avons une obligation morale de créér une meilleure vie pour tous les peuples en parlant en faveur de la prévention de désastres et leur conséquences. Un engagement à la vie et ses valeurs doit donc être renforcé. Quelques suggestions concernant le rôle des infirmiers/eres sont faites ici.

Alle lebenden Menschen haben das morlaische Recht, in lebensdrohenden Situationen angemessene Hilfe zu erhalten. Katastrophen sind vielseitig, aber ihre Konsequenzen können sich für einzelne Menschen verlängern bis in die nächste Generation. Wir haben eine moralische Verpflichtung, ein besseres Leben zu erarbeiten für alle Menschen, in dem wir eintreten um künstliche Katastrophen und deren Konsequenzen zu verhüten. Eine Verpflichtung zum Leben und seinen Werten muss deshalb verstärkt werden. Eine Anzahl von Vorschlagen, besonders was die Rolle des Krankenpflegepersonals anbetricht, sind hier gemacht.
Hiv and Aids: the Nursing Response and Some Ethical Challenges
Authors: McHaffie Hazel E.
Published: November 2007

AIDS has challenged many concepts and practices within nursing. Because of the serious implications attending a positive diagnosis, and because patients with AIDS have become articulate and well informed, familiar principles have been exposed to renewed scrutiny. Anomalies and dilemmas have been revealed. Results from a recent Institute of Medical Ethics survey carried out by the author have illustrated some of the theoretical concepts. Confidentiality has assumed new dimensions. Partnership and mutual empowerment are seen as keys to sound practice involving the patient in decision-making. A vital ingredient relates to the preparation of professionals; they need to be aware of those attitudes and prejudices within themselves which potentially stand in the way of caring well.
Patient Advocacy At the End of Life
Authors: Love Mary Brewer
Published: November 2007

Caring for the competent, fragile, elderly patient at the end of life is becoming increasingly challenging. This case explores several ethical areas of concern that arise when caring for patients who have written durable powers of attorney for health care decisions and face life or death choices. Areas covered are informed consent with the elderly patient, the family's right to be involved in decision-making, futility of treatment, and the nurse's role as patient advocate during times of difficult decision-making. Recommendations for increased nursing intervention during these times are incorporated.

Les soins des personnes âgés, fragiles mais toujours compétentes et dont la vie touche à sa fin, posent des problèmes de plus en plus urgents. Cet article discute de plusieurs aspets qui se manifestent dans les soins des malades ayant écrit des mandats concernants leurs soins et qui doivent maintenant prendre des décisions graves concernant la vie ou la mort. Les aspets dont il s'agit dans cet article sont: le consentement informé des personnes âgés: le droit de la famille de participer à la décision; les traitements inutiles; et le rôle des infirmiers/ères pour plaider leur cause pendant des moments difficles de prises de décisions. Des recommendations pour de plus importantes interventions infirmières sont comprises.

Die Pflege der geistig kompetenten aber hinfälligen Personen im hohen Alter stellt immer grössere Anforderungen. Dieser Artikel untersucht verschiedene ethische Aspekte, die bei der Pflege von Patienten, die eine schriftliche Vollmacht über medizinische Behandlung ausgestellt haben für ihre Pflege und jetzt Entscheidungen über Leben oder Tod treffen müssen. Der Artikel umfasst folgende Bereiche: informierten Zustimmung des Patienten, das Mitbestimmungsrecht der Familie, Zwecklosigkeit der Behandlung, und die Rolle des Pflegepersonals als Fürsprecher der Patienten bei schwierigen Entscheidungen. Empfehlungen für eine grössere Beteiligung oder Mitbestimmung des Pflegepersonals in diesen Fällen sind eingeschlossen.