bluuebunny/crossref_metadata_2025 huggingface dataset:
The role of specialist home care teams: views of general practitioners in south London
Authors: Boyd Kirsty J
Published: November 2007

This study aimed to assess south London general practitioners' views on the role of specialist home care teams and their working patterns, particularly involvement in out-of-hours care. In February 1992, postal questionnaires were sent to the 705 general practitioners working in three adjacent FHSA areas. The response rate was 79%. There was approval of the teams' current role and of extending this to include patients with nonmalignant disease. Most thought a 24-hour on-call and visiting service desirable, and wanted to share out-of-hours care with the teams.

The implications of changing and extending the role of specialist teams and the issue of providing adequate out-of-hours care for patients wishing to remain at home are discussed.
A regional survey of opioid use by patients receiving specialist palliative care
Authors: Brooks DJ , Gamble W. , Ahmedzai S.
Published: November 2007

A study was conducted to determine the patterns of opioid use in patients under the care of specialist palliative care (SPC) teams in Trent Region, both in the community and in inpatient settings. The design was a survey of point prevalence by case note and drug chart review.

The case notes and prescription records of 1007 patients were reviewed, and data collected on age, sex, diagnosis, date of referral, care settings, opioid form and dose on referral, and most recent opioid form and dose.

Nine hundred and seventy patients had cancer; their ages ranged from 18 to 98 years (mean 66.5; median 69). Care was delivered by community specialist nurse for 39%, hospice daycare (DC) for 31 %, hospice based homecare for 11 %, hospice inpatient unit (IPU) for 15%, hospice outpatient (OP) for 5%, and other for 0.5%.

There was no record of medication in 2% of the notes. No opioids had been prescribed for 43% of patients (range 24% IPU to 49% DC), 10% of patients were using weak opioids only (range 0.9% IPU to 16% OP), and 45% of patients were using strong opioids (range 39% DC to 75% IPU), the majority being on morphine (87% of total strong opioids).

Daily oral morphine equivalence (OME) dosages ranged form 0.4 mg to 3600 mg (mean 166; median 60; mode 60). Dose changes under SPC ranged from -780 mg to +3900 mg OME, 42% patients having had no change in their dosage and 15% having reduced their opioid dose whilst under SPC.

This survey challenges the popular impression that patients receiving SPC require large doses of morphine. The highest prevalence of potent opioid prescribing was in hospice IPUs, largely owing to the use of parenteral diamorphine. Conversely, IPUs had the lowest prevalence of weak opioids. Staff caring for patients with cancer must consider the need for downward as well as upward titration of opioid dosages.
Palliative care services: what needs assessment?
Authors: Robbins Margaret A , Frankel Stephen J
Published: November 2007

A survey of all district health authorities in England was conducted in order

to describe current patterns of needs assessment and contract setting for palliative care services. Outcome measures included the completion of needs assessments in the past five years, the type of data used for needs assessment, and recommendations for service development. Copies of contracts for palliative care services were requested and analysed according to duration of contract, and audit requirements. A 74% response rate was achieved. Over half the health authorities had not carried out a needs assessment or service review
Blood transfusion and its benefits in palliative care
Authors: Gleeson Catherine , Spencer David
Published: November 2007

The value of blood transfusion as a supportive treatment in haematological disease and oncology is well established and is seen as an essential part of treatment. The place of blood transfusion in the alleviation of symptoms within palliative care units is less well established. There has been no evaluation of its benefits in terms of symptom relief and impact on the quality of life.

This study was designed to identify the benefit achieved by transfusion as well as possible indicators for its appropriate use in the future.

Ninety-seven patients were recruited over one year, from the beginning of September 1992 to the end of August 1993, from eight centres, all members of the South West Thames Palliative Medicine Collaborative Audit Group.

Patients completed visual analogue scales before and on two occasions after transfusion, to assess its impact on dyspnoea, weakness and overall sense of well-being. Results indicated that a significant proportion showed improvement in all three parameters. Those whose main indication for transfusion was weakness showed a particular benefit. The group of patients entered into the study were anaemic in comparison with our normal patient population, but the degree of improvement seen did not correlate with the degree of anaemia prior to transfusion.

We conclude that transfusion does offer symptom relief and improvement in well-being in patients with advanced malignant disease. It should be considered as a worthwhile option in palliative treatment of weakness, dyspnoea and impaired overall sense of well-being, when associated with anaemia.
A randomized controlled trial of the cost-effectiveness of a district co-ordinating service for terminally ill cancer patients
Authors: Raftery JP , Addington-Hall JM , MacDonald LD , Anderson HR , Bland JM , Chamberlain J. , Freeling P.
Published: November 2007

The objective of this paper is to compare the cost effectiveness of a co- ordination service with standard services for terminally ill cancer patients with a prognosis of less than one year.

We designed a randomized controlled trial, with patients randomized by the general practice with which they were registered. Co-ordination group patients received the assistance of two nurse coordinators whose role was to ensure that patients had access to appropriate services.

The setting was in a South London health authority. Complete service use and outcome data were collected on 167 patients, 86 in the co-ordination group, and 81 in the control group.

Our results, as previously reported, show that no differences in outcomes were detected between the co-ordination and control groups; the mean total costs incurred by the co-ordination group were significantly less than those of the control group. The co-ordinated group used significantly fewer inpatient days (mean 24 versus 40 inpatient days; t = 2.4, p = 0.002) and nurse home visits (mean 14.5 versus 37.5 visits; t = 0.3, p = 0.01). Mean cost per co- ordinated patient was almost half that of the control group patients (£4774 versus £8034, t = 2.8, p = 0.006). Although the unit cost data were relatively crude, these cost reductions were insensitive to a wide range of unit costs. These differences persisted when, in order to control for any putative differences in severity between the two groups, the analysis was restricted to patients who had died by the end of the study. The ratio of potential cost savings to the cost of co-ordination service was between 4:1 and 8:1.

In conclusion, the co-ordination service for cancer patients who were terminally ill with a prognosis of less than one year was more cost effective than standard services, due to achieving the same outcomes at lower service use, particularly inpatient days in acute hospital. Assuming that the observed effects are real, improved co-ordination of palliative care offers the potential for considerable savings. Further research is needed to explore this issue.
Xerostomia — a review
Authors: Cooke C. , Ahmedzal S. , Mayberry J.
Published: November 2007

Xerostomia, or dry mouth, is a very common symptom amongst the terminally ill and can have profound negative effects on patients' quality of life. Yet, it is often considered trivial and therefore tends to be neglected and ignored. This review examines the pathophysiology, aetiology and methods of assessing xerostomia and its effects and discusses its management, with particular emphasis upon palliation. If xerostomia is managed sensitively and effectively, it is possible to improve patients' comfort and sense of well-being and so improve overall care.
Problems at presentation in 440 patients with advanced cancer in a south Indian state
Authors: Sureshkumar K. , Rajagopal MR
Published: November 2007

A new palliative care unit in Kerala, a southern state of India, treated 440 patients with advanced cancer in the first 15 months of operation. This paper presents an analysis of the problems these patients presented with. Besides the physical problems due to the disease, these patients were found to have a multitude of other concerns peculiar to the region. Lack of financial resources and facilities for follow-up, concerns about the welfare of the family in the absence of proper state-sponsored social security schemes and lack of proper communication between doctors and patients were the major problems that the patients faced. The availability of numerous systems of alternative medicine and a hope for cure even at a late stage of the disease meant that many patients depended on these alternative therapies as their main form of treatment.
The ideology and organization of spiritual care: three approaches
Authors: Walter Tony
Published: November 2007

The development of holistic, multidisciplinary care of the terminally ill has prompted discussion of what spiritual care might mean, but how can this be done in what is largely a secular context? This paper analyses the three options. (1) The idea of the hospice as a religious community enables total care to be given, but conflict can develop as such institutions expand and take on less devout staff. (2) An organizationally less problematic approach, fitting a widespread understanding of religion, is that only some people are religious and they may be referred by staff to the chaplain - but this undermines the goal of holistic care. (3) Recent discussion of spiritual needs, however, argues that everyone has a spiritual dimension, entailing a search for meaning. All staff can help in this area, so this approach is welcomed by nurses seeking to practice holistically and also by chaplains seeking to expand their domain, but it also has certain costs. The article concludes by raising some problems that may emerge if this third approach becomes institutionalized.
Attitudes and views of physicians and nurses towards cancer patients dying at home
Authors: Porta Miquel , Busquet Xavier , Jariod Manuel
Published: November 2007

The objective was to study attitudes and views of primary care professionals towards terminally ill cancer patients who die at home, using a cross- sectional study based in the health district of Manresa (province of Barcelona, Catalonia, Spain) of the Catalan Health Service, involving 151 primary health care professionals (87 physicians and 64 nurses).

By using a self-responded anonymous questionnaire (response rate 89%) it was found that despite excellent motivation, primary care professionals reported widespread frustration and a poor opinion of the quality of care provided to terminally ill cancer patients. Attitudes and views clearly differ by age, sex and geographic setting. In the study area, most professionals are reluctant to disclose the diagnosis of cancer, and this attitude is associated with a more favourable assessment of the support provided to the family. The idea that the most appropriate place of death is at home is strongly linked to the belief that patients ought to be informed of their illness, to feelings of frustration and to youth. These findings further substantiate the need and the potential for ample changes in terminal cancer care in Spain.
A comparison of the use of sedatives in a hospital support team and in a hospice
Authors: Stone Patrick , Phillips Clare , Spruyt Odette , Waight Catherine
Published: November 2007

This study examines how frequently and for what indications sedatives are prescribed in a hospital support team and in a hospice. We also looked at the survival of sedated patients from the date of admission and from the start of sedation.

Overall 26% of patients were prescribed sedatives in order to sedate them (31 % at the hospice and 21 % at the hospital) and 43% of patients were given sedatives for symptom control (67% at the hospice and 21 % at the hospital). Sedated patients survived for a mean of 1.3 days after the start of sedation, and there was no detectable difference in survival from the date of admission between sedated and nonsedated patients.
The use of the genogram in palliative care
Authors: Liossi Christina , Hatira Popi , Mystakidou Kyriaki
Published: November 2007

A genogram is a format for drawing a family tree that records information about family members and their relationships over at least three generations. The genogram has been widely promoted as a useful tool for gathering, recording and displaying family information in order to practice family-oriented health care. We present a format for constructing the genogram and to outline the principles underlying its interpretation and application in clinical practice, so as to facilitate its use by physicians, nurses, psychologists, family therapists, and other professionals working with families in palliative care. Finally, the future potential of the genogram as a research and clinical tool in palliative care is discussed.
Bed configuration and microscale processes in alluvial channels
Authors: Robert André
Published: November 2007

Numerous recent studies on fluvial processes, both in Canada and internationally, have focused on small-scale phenomena. Investigations on the characterization of surface roughness in coarse-grained channels and its links with flow resistance and sediment transport processes have been a dominant field of research. Closely related is a second major area of investigation on turbulent flow structures in boundary layers over both sand and gravel beds and their relations with the transport of bed material. Phenomena potentially related to 'bursting' have been shown to control bedload transport processes and the concentration of sediment in suspension. Detailed investigations have also been conducted on the links between flow turbulence, bed material movement, and bed morphology at channel junctions. Finally, selective entrainment and transport of individual coarse particles have been studied from field measurements and laboratory experi ments. Emphasis has been put on bed microtopography, surface structure and texture, and on a probabilistic approach to bedload transport.
Snow avalanches
Authors: Keylock Chris
Published: November 2007

Snow avalanches have a substantial impact upon human activity in mountainous regions. In this article I employ a climatological framework to examine the mechanisms by which snow avalanches occur. This is followed by a discussion of the hydrologic and geomorphic aspects of avalanches, and an examination of the current state of research as regards predicting maximum avalanche runout and avalanche risk. It is hoped that this article brings to the reader's attention the wide range of literature that focuses upon this very hazardous natural phenomenon.
Rare association of multiple sclerosis and systemic lupus erythematosus
Authors: Kyrozis A. , Kararizou E. , Georgila E. , Loukaidis P.N. , Koureas A. , Rombos A. , Sfagos C. , Vassilopoulos D.
Published: November 2007

Although both multiple sclerosis (MS) and systemic lupus erythematosus (SLE) are relatively common autoimmune disorders, especially in young women and often coexist in families, they are only rarely reported to coexist in a single patient. We here present a case of a young woman with a history of MS from many years who diagnosed as suffering as well from SLE. Lupus (2007) 16, 991—992.
High Prevalence of Significant Heart Valve Lesions in Patients with the 'Primary' Antiphospholipid Syndrome
Authors: Cervera Ricard , Khamashta Munther A. , Font Josep , Reyes Pedro A. , Vianna João L. , López-Soto Alfons , Amigo Mary-Carmen , Asherson Ronald A. , Azqueta Manuel , Paré Carles , Vargas Jesús , Romero Angel , Ingelmo Miguel , Hughes Graham R.V.
Published: November 2007

A prospective echocardiographic study was carried out on 55 patients with the recently described 'primary' antiphospholipid syndrome derived from three university medical centres. The prevalence of valvular lesions in patients with this syndrome was 38% compared with 4% in a control group of 55 healthy volunteers (P < 0.001). Mean age of patients with valve was 42 ± 12 years and of those without, 30 ± 10 years (P < 0.05). One patient had a morphologic echocardiographic pattern suggestive of non-infective verrucous mitral endocarditis. Twenty patients had a two-dimensional or Doppler echocardiographic pattern of significant valvular dysfunction —either regurgitation or stenosis—without evidence of vegetations. Mitral and aortic regurgitation were the most common lesions in these patients. During follow-up of patients with valvular disease, haemodynamically significant clinical valve disease developed in four and surgery was required in one. Eleven patients had cerebrovascular occlusions. Thus, valvular heart disease, particularly affecting the mitral and aortic valves, is common in patients with the 'primary' antiphospholipid syndrome, especially in. those over 40 years old.
A Phospholipid-β2-Glycoprotein I Complex Is an Antigen for Anticardiolipin Antibodies Occurring in Autoimmune Disease But Not with Infection
Authors: Hunt John E. , McNeil H. Patrick , Morgan Gary J. , Crameri Regina M. , Krilis Steven A.
Published: November 2007

Anticardiolipin antibodies (aCL) purified from patients with autoimmune disease have recently been shown to interact with a phospholipid-binding plasma protein, β2-glycoprotein I (β2-GPI). The aim of this study was to determine whether aCL purified from patients with infection also interact with β2 -GPI.

aCL purified from 23 patients with malaria, infectious mononucleosis, tuberculosis, hepatitis A or syphilis did not require the presence of β2-GPI to bind cardiolipin (CL). In contrast, aCL were purified from 11 out of 12 patients with autoimmune disease that bound CL only in the presence of β2-GPI. Thrombotic complications appear to be associated with aCL occurring in autoimmune disease but not with aCL associated with infections. We postulate that this increased risk of thrombosis in the autoimmune group may be due to the presence of aCL that bind CL in association with β2-GPI, a plasma protein with anticoagulant activity.
Management of Antiphospholipid Antibody Positivity and Elective Orthopedic Procedures
Authors: Schnitz William M. , Lister Kathleen A. , McCarty Gale A.
Published: November 2007

Current management of primary or secondary antiphospholipid antibody (aPL) syndromes with known embolic phenomena requiring anticoagulation is empiric in the setting of elective orthopedic procedures. Short-term withdrawal of warfarin with continuance of aspirin and glucocorticoid therapy was undertaken for sequential bilateral knee replacements in a lupus patient with aPL. Her course was successfully managed without thrombo-embolic complications.
Review: Treatment of Cutaneous Lupus Erythematosus
Authors: Rothe Marti J. , Kerdel Francisco A.
Published: November 2007

The mainstays of treatment of cutaneous lupus erythematosus (LE) are topical and intralesional steroids, sunscreen, and the antimalarials. However, the practitioner is often faced with the challenge of cutaneous disease unresponsive to these conventional measures. We present our approach to the management of cutaneous LE and review the literature regarding therapy of refractory disease.
The Heart and Antiphospholipid Antibodies in MRL-lpr/lpr Mice
Authors: Vianna J.L. , Trotter S. , Khamashta M.A. , Chikte S. , Olsen E. , Hughes G.R.V.
Published: November 2007

Our objective in this study was to determine possible associations between antiphospholipid antibodies (aPL) and histologically defined heart valve lesions in the MRL-lpr/lpr mouse, a suitable model for the antiphospholipid syndrome (APS). At monthly intervals, from 2 to 6 months of age, three MRL-lpr/lpr mice (two with anticardiolipin antibodies, one without) and two sex- and age-matched Balb/c mice (controls) were sacrificed for histological studies. Serum binding to phospholipids and DNA was studied at this time.

We found thickened heart valves in 68% of MRL-lpr/lpr mice and in 80% of Balb/c mice, and no association with any of the antibodies tested was found. No evidence of coronary vasculitis or thrombi was found in any of the mice studied. Platelet counts in MRL-lpr/lpr mice were significantly lower (640.530 ± 211.818 × 106/ml) than in Balb/c mice (780.0 + 112.5 × 106/ml) (P < 0.05), and no association was found between platelet counts and aPL. In this model of murine APS, aPL bear no importance in heart valve pathology.
Lack of Specific Binding of Anticardiolipin Antibodies to Intact Platelets
Authors: Haga Hans-Jacob , Christopoulos Costas , Machin Samuel J. , Khamashta Munther , Hughes Graham R.V.
Published: November 2007

The aims of this study were to investigate whether anticardiolipin antibodies (aCL) bind to intact (resting or activated) platelets in vitro. Suspensions of resting, activated (with a mixture of thrombin and collagen) and freeze-thawed platelets from healthy subjects were incubated with either affinity-purified aCL or pooled normal human immunoglobulin G (IgG). Platelet-bound IgG was measured by flow cytometric analysis of platelets incubated with a fluorescein-conjugated polyclonal goat anti-human IgG.

There was no significant binding of IgG aCL to intact resting or activated platelets, while significant specific binding to freeze-thawed platelets was demonstrated. These results question the theory that aCL bind/activate intact platelets in vivo.
High-Dose Intravenous Methylprednisolone Therapy Associated with Osteonecrosis in Patients with Systemic Lupus Erythematosus
Authors: Massardo Loreto , Jacobelli Sergio , Leissner Mauricio , González Myriam , Villarroel Luis , Rivero Santiago
Published: November 2007

Osteonecrosis is related to the use of steroids in patients with systemic lupus erythematosus (SLE); its association with the use of 'pulses' of methylprednisolone (PMP) is not clear at present. In a retrospective analysis of 190 patients with SLE we found that 19% of 36 patients treated with PMP had osteonecrosis compared with 6% of 154 patients without that treatment (P < 0.04). Risk factors associated with osteonecrosis were PMP treatment, cushingoid appearance, steroid doses ≥ 40 mg/day during the first month of treatment, a ratio of steroid dose in grams/year ≥ 12, hematuria and proteinuria. In a stepwise regression model, when cushingoid appearance was excluded, PMP became the only significant factor (P = 0.045).

We conclude that osteonecrosis can be considered a long-term complication of PMP treatment in SLE patients.
Systemic Lupus Erythematosus in : An Overview
Authors: Catoggio Luis J.
Published: November 2007

This brief report will attempt to analyse systemic lupus erythematosus in Argentina and the contributions made from this part of the world to the medical literature in English.
Genetic, Immunologic and Biotransformation Studies of Patients on Procainamide
Authors: Adams Louis E. , Balakrishnan Kamala , Roberts Stephen M. , Belcher Rick , Mongey Anne-Barbara , Thomas T.J. , Hess Evelyn V.
Published: November 2007

This report represents follow-up observations of a unique long-term study of patients on procainamide (PA) for various cardiac arrhythmias. Serologic and clinical evaluations associated with drug-related autoimmunity were assessed and patients were characterized for factors postulated to influence susceptibility to autoimmunity, including acetylator phenotype, oxidative metabolism of PA, HLA class profile, and production of interleukin-1 (IL-1) and tumor necrosis factor (TNF). Fifty-two percent had IgM and 70% IgG antibodies to total histones; 67% had IgG antibodies to histone H2A/H2B. Patients were equally divided between fast and slow acetylators. N-oxidative metabolism of PA was indicated by the presence of urinary nitroprocainamide, which correlated with elevated titers of antihistone antibodies. There was a significant incidence of the DQw7 split of DQw3 in PA patients when compared to controls, and the frequency of antibodies to total histones and H2A/H2B was significantly increased in the DQw7 patients. C4A*QO and C4B*QO alleles were more frequent in the PA patients than in controls. IL-1 and TNF production was not different in patients compared to controls. These data suggest that certain genetic factors may serve as markers for PA-related autoimmunity.
Lymphocyte Subsets in a Large Cohort of Patients with Systemic Lupus Erythematosus
Authors: Erkeller-Yüksel Feza , Hulstaart Frank , Hannet Irene , Isenberg David , Lydyard Peter
Published: November 2007

In search of markers of disease activity in patients with SLE we have investigated blood lymphocyte subsets from a large cohort of patient. Seventy-one patients were studied using a well-defined panel of fluorescent monoclonal antibodies which recognize the major T, B and NK lymphocyte subsets and activated cells. Flow cytometry was used with standard automated software.

Overall, SLE patients were lymphopenic. The proportion of activated T cells was increased and NK cells were decreased in both proportion and absolute numbers ( P < 0.001). This decrease was more pronounced in the more active patients.

None of the T cell activation markers was shown to distinguish different degrees of disease activity. However, the percentage of NK cells was significantly reduced in active disease states ( P < 0.01). Decreased numbers of NK cells could potentially reduce the resistance of SLE patients to infectious organisms.
Detection of Anti-Phospholipid and Anti-DNA Antibodies and their Idiotypes in Newborns of Mothers with Anti-Phospholipid Syndrome and SLE
Authors: Zurgil N. , Bakimer R. , Tincani A. , Faden D. , Cohen J. , Lorber M. , Valesini G. , Shoenfeld Y.
Published: November 2007

The titers, isotypes and idiotypes of antiphospholipid and anti-dsDNA antibodies were determined in seven pairs of mothers with antiphospholipid syndrome (APLS) and their offspring, in I 1 pairs of SLE mothers and their matched infants and in seven respective pairs of healthy subjects. In addition, maternal as well as fetal sera were evaluated for the presence of anti-SSA (Ro), anti-SSB (La) and anti-70 kd RNP autoantibodies.

In the sera from APLS patients, as well as in the sera from their offspring, the mean antibody titer of IgG aCL was found to be significantly higher then the corresponding value in the control group ( P < 0.01). Highly significant increased titers of IgG anti-DNA antibodies were found in the sera of SLE mothers and their matched offspring ( P < 0.0008).

The prevalance of anti-SSA, anti-SSA, and anti-7OK RNP antibodies was lower then that of antiphospholipid and anti-dsDNA antibodies. Only one of the respective offspring had increased levels of these antibodies.

The quantity of maternal antibodies transferd to the fetus was depended on their concentration in the maternal circulation, as well as on their type and specificity. Follow-up of newborn sera showed a progressive decrease in the antiphospholipid antibody titers during 3 months. After 6 months it was undetected.

Our results point to a transplacental transfer of aCL and anti-DNA antibodies, a phenomenon which is not necessarily associated with respective clinical manifestations, in contrast to the classical humoral mediated autoimmune diseases (e.g. myasthenia gravis).
Maternal Autoantibodies and Congenital Heart Block: No Evidence for the Existence of a Unique Heart Block-associated Anti-Ro/SS-A Autoantibody Profile
Authors: Meilof J.F. , Frohn-Mulder Lm.E. , Steward P.A. , Szatmari A. , Hess J. , Veldhoven C.H.A. , Smeenk R.J.T. , Swaak A.J.G.
Published: November 2007

One of the rare examples of the transfer of autoimmune disease from mother to (unborn) child is the neonatal lupus syndrome. This syndrome comprises the development of fetal heart disease (congenital heart block) or neonatal skin rash and is specifically associated with maternal anti-Ro/SS-A autoantibodies. Previous studies have suggested that especially maternal autoantibody reactivity against the 52 kDa protein of the Ro/SS-A antigen and/or against the La/SS-B antigen is responsible for the development of congenital heart block (CHB).

To determine the CHB-associated antibody response in more detail, we analysed the presence of autoantibodies in sera from mothers of children with isolated heart block. All 14 mothers of children with congenital heart block were positive for anti-Ro/SS-A antibodies. Remarkably, their antibody profile, including recognition of different Ro/SS-A proteins and autoantibody levels against these proteins, did not differ from anti-Ro/SS-A positive mothers of healthy children. In contrast, all 8 anti-Ro/SS-A negative mothers had children with acquired heart block.

We conclude from our data that maternal anti-Ro/SS-A antibodies are essential for CHB but that fine analysis of this autoantibody response does not predict the occurrence of CHB.
Standardized Ultraviolet-A Exposure Provokes Skin Reaction in Systemic Lupus Erythematosus
Authors: Nived Ola , Johansen Per B. , Sturfelt Gunnar
Published: November 2007

The immediate, 1 day and 14 days skin reaction was determined in 23 female SLE patients and 23 age-matched controls after standardized exposure to ultraviolet light of the UV-A wavelengths (320-440 nm). Eighteen of the patients and 12 of the controls were photosensitive by history. Eight separate sites on the buttocks were exposed to UV-A light, four sites at doses between 42 and 252 kJ/m2 and four sites with longpass filters (320, 345, 360 and 375 nm). The reactions on test sites were graded by two independent observers unaware of given doses or filter location. All patients and controls reacted with immediate erythema irrespectively of the presence or absence of photosensitivity. After 1 day, 39% of controls and 78% of patients had erythema and the reactions were more pronounced to longwave UV-A light (>320nm) in the patients (P < 0.001). After 14 days, six patients, but no control, had persistent erythema ( P = 0.04). Interestingly, three of the four patients without anamnestic photosensitivity did not react on days 1 or 14, while the pattern seen in the controls on day 1 was totally unpredictable with regard to reported photosensitivity. These findings strongly suggest that a considerable proportion of SLE patients show pathological skin reactions to physiological doses of longwave UV-A and not only the far more studied shortwave UV-B (290-320 nm). The responsible chromatophore for the UV-A reaction is not known.
Interstitial Inflammatory and Chronic Tubulointerstitial Lesions in Lupus Nephritis: Comparison with Those in IgA Nephropathy
Authors: Yamamoto Tatsuo , Nagase Mitsumasa , Hishida Akira , Honda Nishio
Published: November 2007

The significance of interstitial inflammatory and chronic tubulointerstitial lesions was studied in relation to the severity of glomerular lesions in 62 patients with lupus nephritis and 88 with IgA nephropathy. Severe interstitial inflammatory and chronic tubulointerstitial lesions were found in patients with severe glomerular lesions in both lupus nephritis and IgA nephropathy. In such cases, the serum creatinine levels at biopsy were high and the renal prognosis was poor regardless of the underlying disease (lupus nephritis or IgA nephropathy). No IgA nephropathy patients with nil or mild glomerular lesions had moderate or severe interstitial inflammatory and/or chronic tubulointerstitial lesions. In contrast, predominantly severe interstitial inflammatory lesions were found in 36% of lupus nephritis patients with nil or mild glomerular lesions. The prevalence of interstitial immune complexes deposition was markedly high in those with predominant interstitial inflammatory lesions. However, the severity of chronic tubulointerstitial lesions was mild and renal function did not deteriorate in the mean follow-up periods of 68.6 months. It is suggested that, besides the tubulointerstitial lesions attributable to the severe concomitant glomerular damage, the interstitial deposition of immune complexes per se plays a pathogenic role in the interstitial inflammatory lesions in lupus nephritis. Its prognostic significance, however, was considered to be minor.
Systemic Lupus Erythematosus: Sex Hormones in Male Patients
Authors: Sequeira João F. , Keser Gokhan , Greenstein Ben , Wheeler Michael J. , Duarte Paulo C. , Khamashta Munther A. , Hughes Raham R.V.
Published: November 2007

Systemic lupus erythematosus (SLE) is a rare disease in males. There is evidence that a functional state of hypoandrogenism is important in the pathogenesis of the disease. We analysed the levels of several hormones (follicle stimulating hormone (FSH), luteinizing hormone (LH), testosterone (T), estradiol (E2), free estradiol (FE2) and prolactin (PRL)) in 17 male SLE patients and 17 male healthy controls with similar age distribution. Three lupus patients were excluded from the analysis due to previous cyclophosphamide therapy or pre-puberty. Thus 14 male lupus patients were eligible for the study. Six of the 14 SLE patients (43%) showed at least one abnormal level of FSH, LH or T. There were no abnormalities in these hormones in the 17 controls. This difference was significant (P < 0.01). In five of these 6 male patients (36% of all lupus patients) the hormonal profile was compatible with a functional state of hypoandrogenism (high LH and/or low T). The ratio E2/T (estradiol/testosterone:pmol/nmol) was also significantly higher in the SLE group (average = 6.5; SD 4.3) when compared with that of the control group (average 4.2; SD 1.2; Mann-Whitney rank sum test: P < 0.03). There were no significant differences in E 2, FE2 or PRL between lupus patients and controls. We did not confirm the notion that left-handedness is frequent in male lupus as all our patients were right-handed. We found a significantly higher prevalence of sex hormone abnormalities in male lupus patients when compared with healthy controls with a similar age distribution. These abnormalities were consistent with a functional state of hypoandrogenism although the levels of FE 2 were not different between male lupus and controls.
Fish Oil Derivatives as a Prophylaxis of Recurrent Miscarriage Associated with Antiphospholipid Antibodies (APL) : a Pilot Study
Authors: Rossi Edoardo , Costa Mauro
Published: November 2007

Since 1989, 22 patients with persistent antiphospholipid syndrome (PAPS) associated with recurrent miscarriage (defined as three or more miscarriages) were treated with fish oil, equivalent to 5.1 g eicosapetaenoic acid (EPA) and docosahexaenoic acid (DHA) at a ratio of 1.5 EPA to DHA. Twenty-two patients had 23 pregnancies (one patient had two pregnancies) over a period of 3 years. There was only one intrauterine fetal death at the 27th week associated with pre-eclampsia. Twenty-one pregnancies, 19 of which ended after the 37th week, produced a baby. Two pregnancies ended with cesarean section for pre-eclampsia at 30th and 35th week of gestation and one is ongoing at 32nd week. All babies are well. The weight at birth of babies delivered at term was always >2500 g. These encouraging results favour a therapeutic role, without any adverse reaction, of fish oil to prevent recurrent miscarriage in PAPS.
Hydroxychloroquine, Dosage Parameters and Retinopathy
Authors: Spalton D.J. , Verdon Roe G.M. , Hughes G.R.V.
Published: November 2007

In a study designed to determine the presence of retinopathy in patients taking hydroxychloroquine we compared 82 patients attending a rheumatology clinic who had taken hydroxychloroquine for over 1 year with controls. Outcome measurements included fundoscopy, fundus photography and automated computerized visual fields of the macular area. No patient was found to have retinopathy. No correlation could be found between indices of visual field function and total drug usage, average daily dose, dosage in mg/kg body weight or duration of treatment. Retinopathy is unlikely to occur at dosages of hydroxychloroquine of less than 6.5 mg/kg body weight with under 10 years of treatment. Present guidelines for ophthalmic screening of patients on antimalarial therapy are too rigorous and visual field testing if not of benefit. Further recommendations are made for ophthalmic supervision.
Malignancy in Systemic Lupus Erythematosus: a Report of Five Cases in a Series of 96 Patients
Authors: Lopez Dupla Miguel , Khamashta Munther , Garcia Vicente Pintado , Uriol Paz Lavilla , Ortega Eulalia Valencia , Gil Aguado Antonio
Published: November 2007

The association of systemic lupus erythematosus (SLE) and malignancy has been reported previously and suggests an increased risk of cancer in this disease. Lymphomas are the most frequent neoplasias reported in these patients and carcinoma of the cervix and breast are also seen. Several factors probably play a role in the aetiology of malignancies associated with SLE including intrinsic disturbances of immunity and concomitant immunosuppressive therapy. We report five solid tumors (one breast carcinoma, one squamous cell carcinoma of the anus, one adenocarcinoma of the rectum, one carcinoma of the cervix and one carcinoma of the gallbladder) among 96 patients with SLE. The most striking finding in this study was the absence of haematological malignancies. The incidence of malignancy in the series, the age of diagnosis of SLE and neoplasia and the time delay between SLE and malignancy diagnosis was similar to other series. We did not find any clinical or immunological feature that predicted the development of neoplasia.

In conclusion, patients with SLE may have the same malignancies as the general population after adjustment for age and sex. There are no predictive indicators for malignancy and immunosuppressive therapy may be a contributing factor.
Safety of Hormone Replacement Therapy (HRT) in Systemic Lupus Erythematosus (SLE)
Authors: Arden N.K. , Lloyd M.E. , Spector T.D. , Hughes G.R.V.
Published: November 2007

Despite its obvious benefits, many physicians are reluctant to prescribe HRT to patients with SLE. We have performed a retrospective study in 60 postmenopausal women with SLE including 30 HRT users and 30 never users. The patients were studied for 12 months after the initiation of HRT. The two groups were well matched for disease characteristics. The HRT users experienced significant improvements in general well being, libido and depression. There was no significant difference in any other parameter measured. There was no increase in the number of thrombo-embolic events in the user group despite 7 patients having a positive thrombophilia screen. In conclusion, in stable postmenopausal SLE HRT appears well tolerated and safe.
Thrombosis and Antiphospholipid Syndrome: a Preliminary Assessment of Three Antithrombotic Treatments
Authors: Rivier Gilles , Herranz M. Teresa , Khamashta Munther A. , Hughes Graham R.V.
Published: November 2007

To assess retrospectively three antithrombotic treatments in the secondary prevention of thrombosis in the antiphospholipid syndrome (APS), 23 patients (six systemic lupus erythematosus, seven lupus-like disease and 10 primary antiphospholipid syndrome) were included in this study. Treatments assessed were: (1) aspirin 75 mg daily, (2) warfarin (international normalised ratios (INRs) 2.0-2.9) ± aspirin 75 mg daily, and (3) warfarin (INRs > 2.9) ± aspirin 75 mg daily. Where patients had received two or three of these treatments successively, the periods of time on each treatment were added and the number of patients with recurrence(s) on each treatment were compared by Fisher's exact probability test.

'High' anticoagulation (INRs > 2.9) ± aspirin 75 mg daily was more effective than aspirin 75 mg daily in preventing further thromboembolic events (P = 0.0053). In addition, when comparing 'high' anticoagulation ± aspirin 75 mg daily versus 'low' anticoagulation (INRs 2.0-2.9) ± aspirin 75 mg daily, there was a trend in favour of 'high' anticoagulation (P = 0.066). No statistically significant difference could be demonstrated when comparing 'low' anticoagulation ± aspirin 75 mg daily with aspirin 75 mg daily (P = 0.092).

These results suggest that aggressive anticoagulation with or without low-dose aspirin is effective in preventing further thromboembolic events in APS.
Cross-Sectional Analysis of the Differences Between Patients with Systemic Lupus Erythematosus in England, Brazil and Sweden
Authors: Johnson A.E. , Cavalcanti F.S. , Gordon C. , Nived O. , Palmer R.G. , Sturfelt G. , Viner N.J. , Bacon P.A.
Published: November 2007

This study is a cross-sectional analysis of the differences between SLE outpatients seen in Rheumatology departments at University centres in England, Brazil and Sweden, using a standard protocol. The demographic characteristics, extent and activity of disease of 209 patients with SLE were studied; 112 patients were seen in England, 33 in Brazil and 64 in Sweden. The median age of disease onset of Brazilian and English patients was 25 years and of Swedish patients 31.5 years.

Disease activity was measured by the BILAG index. In most systems Brazilian patients experienced more activity than English patients and English patients more activity than Swedish patients. Non-Caucasians experienced more active disease than Caucasians. No sex or occupational differences were observed in disease activity.

English patients were the most likely to have experienced photosensitivity, oral ulcers and haematological disorders, Brazilian patients renal disorders and Swedish patients discoid rashes. Brazilian patients were the most likely to be prescribed only one drug for treatment of SLE and to be taking steroids and the highest dose of steroids, in contrast to the European patients who were often prescribed steroids and an antimalarial agent or azathioprine.

The results of this cross-sectional assessment of disease activity using a standardized instrument indicate that there are real differences in the extent and degree of activity of SLE in different national groups. This reflects a combination of genetic, environmental and social influences on disease expression and has implications for treatment and monitoring of SLE patients.
Primary systemic Sjögren syndrome (SS) preceding systemic lupus erythematosus: a retrospective study of 4 cases in a cohort of 55 SS patients
Authors: Zufferey Pascal , Meyer Olivier C , Bourgeois Pierre , Vayssairat Michel , Kahn Marcel-Francis
Published: November 2007

That SS and SLE can coexist is well established. The time relationship between the two diseases is however not well known. To determine whether SS can precede SLE, we reviewed the charts of 55 patients with SS diagnosed in our department between 1975 and 1985. All 55 patients fulfilled at least four of the six preliminary criteria for SS developed by the European Study Group for SS. At entry, none met four ARA criteria for SLE and none had anti-double-stranded DNA or anti-Sm antibodies. The mean follow-up was 12 years (range 8-18 years). Four of the 55 patients developed SLE (7.5%). High titres of anti-native DNA antibodies (> 35 IU RIA, normal < 7 IU) appeared in four patients (2-11 years after the diagnosis of SS). Two of these patients fulfilled at least four ARA criteria for SLE; two others had only three criteria. Two additional patients developed anti-Sm antibodies without anti-native DNA antibodies 1 and 10 years after the initial diagnosis of SS, respectively. Both patients fulfilled six ARA criteria for SLE. At entry, anti-SSA and/or anti-SSB antibodies were present in all six SS patients and persisted subsequently. The main clinical events suggesting progression to SLE were pleuropericarditis, glomerulonephritis and focal central nervous system disease.

In conclusion, SS with extraglandular components can antedate SLE by many years. Anti-native- DNA and anti-Sm antibodies should be measured when atypical clinical manifestations occur in a patient with systemic SS.
Heparan sulphate-ELISA gives false positive results for anti-DNA-DNA/histone immune complexes in sera of patients with SLE
Authors: Suenaga Ronsuke , Abdou Nabih I
Published: November 2007

Heparan sulphate-reactive antibodies in lupus sera have been suggested to be anti-DNA-DNA/ histone immune complexes and to be associated with lupus nephritis. In this study, 23 anti-DNA- positive lupus sera including 13 active nephritis sera were tested for the presence of circulating anti- DNA-DNA/histone immune complexes by solid phase heparan sulphate-ELISA. Because of high background binding to protamine chloride-linked heparan sulphate plates, poly-L-lysine (PLL) was used as a linker and the remaining active sites of PLL were blocked with poly-L-glutamic acid. The ELISA was capable of detecting small amounts of anti-DNA IgG-DNA/histone immune complexes formed in vitro. However, only three active nephritis sera of the 23 sera tested showed significant binding to heparan sulphate plates. This binding was found to be non-specific, the result of high background binding of IgG to PLL. Anti-heparan sulphate ELISA using positively charged linkers detects non-specific binding when lupus sera are tested. Specific assays need to be developed for DNA/histone-related immune complexes present in lupus sera.
Anti-neuronal antibodies in antiphospholipid syndrome with central nervous system involvement: the difference from systemic lupus erythematosus
Authors: Tishler Moshe , Alosachie Iyad , Chapman Yoav , Korcyn Amos , Lorber Margalit , Mevorach Dror , Tane David , Barka Noori , Lin Hun-Chi , Alarcon-Segovia Donato , Youinou Pierre , Peter James B , Shoenfeld Yehuda
Published: November 2007

The presence of antineuronal antibodies was compared in 43 patients with primary aPLS and 57 patients with neuropsychiatric SLE. Fifty-eight patients with Guillain-Barré syndrome and 72 normal healthy donors served as control groups. Seventeen patients in the study group had aPLS associated with CNS involvement. Antineuronal antibodies were studied in the sera employing a novel flow cytometric assay. The frequency of antineuronal antibodies in patients with aPLS and CNS involvement was not significantly different from that of patients with aPLS without CNS disease or from that found in the control groups (12%, 19% and 7%, respectively). However, it was significantly different from that found in SLE patients with CNS involvement (60%) (P < 0.001). Our results provide further evidence that unlike CNS-SLE, the major mechanism of CNS involvement in patients with primary aPLS might not be autoantibody (antineuronal) mediated, but rather 'thrombotic' in origin, or due to yet unknown factors.