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Objectives: With an increasing prevalence of musculoskeletal conditions within the UK, specialty bodies are concerned that graduating medical students may lack appropriate knowledge in this system. We investigated the knowledge base of final year Sheffield medical students in the musculoskeletal system, compared with other major body systems., Methods: A computer-based assessment was designed covering core topics that a pre-registration house officer should know about in musculoskeletal medicine, cardiology and neurology, using a predesigned testing format. The test was blueprinted against internal and external guidelines. It comprised 24 extended matching questions, each with three stems. A sample of 74 volunteer students from the final year (year 5) of the medical course at the University of Sheffield took part in the assessment., Results: Overall scores of students on the test ranged from a baseline of 45% to a maximum of 85%. Test reliability was 0.75 (Cronbach's alpha). On stratifying the overall percentages into marks for individual systems, it was found that there were no significant differences between scores in musculoskeletal medicine, cardiovascular medicine or neurology., Conclusions: Despite the disparity of teaching between musculoskeletal medicine and other major organ systems within Sheffield's integrated medical curriculum, the knowledge base of medical students in the basic and clinical musculoskeletal sciences appears to be similar to that for cardiovascular medicine and neurology by the time of graduation. Nevertheless, several important issues must be addressed before these findings can be generalized.

Background: Rheumatology training has undergone significant changes in the last decade with Calmanization, implementation of the New Deal for junior doctors and newer educational strategies for improving musculoskeletal training, like a core curriculum. However, concerns have been expressed about the quality of postgraduate training programmes in the UK., Objectives: First, to assess current trainees' perceptions of the quality of core and subspecialty training, the impact of workload on training, and to explore demographic variations in training experience. Secondly, to identify educational strategies that trainees felt would enhance their training., Methods: The questionnaire was initially distributed to all specialist registrars attending the BSR Annual Meeting in Brighton in April 2002. Subsequently, the questionnaire was posted to all registrars on the Joint Committee for Higher Medical Training list with a reminder after 4 weeks., Results: Trainees rated positively training in routine patient care, musculoskeletal examination and injection skills while training in primary care rheumatology, epidemiology, paediatric rheumatology and sports medicine was rated negatively. There is agreement that the reduction in junior doctors' hours has adversely affected training, and issues relating to workload have overtaken training issues. Trainees undertaking dual accreditation are more likely to feel this. Educational strategies deemed to enhance training included training workshops focused on specific topics, such as musculoskeletal radiology (89.2%), and an adequate debriefing session after an out-patient clinic (81.6%). An independently administered, reliable and valid scale for quality of training could be used to assess regional variations in training and monitoring quality., Conclusions: The changes to junior doctors' hours, the working patterns of doctors and service commitments have all affected the quality and time available for certain aspects of rheumatological training. A major effort to enhance quality is necessary to ensure that the objectives of training are met within the intended training budget.

The training of junior doctors has undergone major changes in recent years. There is now more structure, with defined assessment time points leading to a Certificate of Specialist Training. This certificate provides documentation indicating that the trainee has undergone a satisfactory period of training and that they are sufficiently competent to practise as a specialist, unsupervised. The changes have led to re-examination of the role of, and educational provision for, research training as well as clinical training. In this article we review these issues and argue that the development of masters educational programmes may help to address several concerns.

Trauma is the commonest cause of acute monoarticular joint pain and swelling in patients attending an accident and emergency (A&E) department. However, in a significant minority of patients there will be no history of trauma and consequently a different approach to assessment and investigation is required. Our aim is to offer an outline of how to assess, investigate, and manage a patient with monoarthritis. Despite advances in antibiotic treatment diagnostic delay partly explains why septic arthritis is still associated with considerable morbidity and mortality. It is therefore imperative that joint infection is considered above all other diagnoses. Arthrocentesis is a relatively safe procedure and doctors in A&E medicine are encouraged to develop the skills required to aspirate large joints. In the same way that the A&E department is often portrayed as the shop window of a hospital, the joint can reflect a wide variety of internal diseases. Connective tissue disease, inflammatory bowel disease, sarcoidosis, and vasculitis can all present with a monoarthritis. A non-specific reactive monoarthritis may be a feature of a wide variety of common and uncommon infections including, brucellosis, Lyme disease, and leptospirosis. Drugs are also associated with acute arthritis either through their metabolic consequences or as idiosyncratic drug reactions. The ability for the joint to reflect multisystem disease necessitates close liaison with specialists from other fields. A multidisciplinary approach to the management of these patients is strongly encouraged as some will have unusual diseases that require specialist advice. It is not difficult to appreciate how the patient with monoarthritis can present the clinician with a fascinating diagnostic and therapeutic challenge, which we hope this article will help to unravel.

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

Both rheumatoid arthritis (RA) and ankylosing spondylitis (AS) have an increased familial occurrence and each disease is associated with the inheritance of specific HLA antigens. We report a pair of identical twin brothers with discordant disease phenotypes: one developed AS at the age of 26, and the other developed RA at the age of 55. The twins possessed both of the disease susceptibility antigens HLA B27 and DR4. Differences in the twins' environmental exposure are discussed.

We describe seven patients who developed malignancy before, after or at the onset of systemic lupus erythematosus (SLE). They comprise three cases of breast adenocarcinoma, two cases of Hodgkin's lymphoma, and one each of cholangiocarcinoma and thymoma. Only one of this group had been treated with cytotoxic agents, and five have subsequently died. They belonged to a group of 150 SLE patients, many of whom had been treated with steroids and cytotoxic agents, under long-term follow-up at a specialized lupus clinic. We discuss the reported association of malignancy in patients with SLE.

Using a detailed questionnaire, the cumulative historical and current demographic, clinical and serological data on 704 SLE patients from 29 European centres and 14 countries have been assessed. Ninety-three percent of the patients were Caucasian and the female/male ratio was 10:1. Analysis of the cumulative incidence showed that arthralgia/arthritis (94%), rash (69%), Raynaud's phenomenon (49%), serositis (44%) and renal disease (38%) were the most frequent clinical manifestations. Virtually all the patients (98%) were antinuclear antibody positive, while anti-ds-DNA antibodies (76%), hypocomplementaemia (71%) and anti-Ro(SSA) antibodies (35%) were frequent serological abnormalities. Whilst much of this data is in line with previous reports, it is notable that renal, lung, and central nervous system involvement and the frequency of rheumatoid factor, anti-Sm and anti-RNP antibodies were much lower than in most comparable series in the United States. We assume that ethnic differences and the greater present awareness of lupus could explain this variations. Low dose corticosteroids, non-steroidal anti-inflammatory drugs and anti-malarials were used to treat over half of the patients, 75% of whom were between 15 and 55 years of age. This report offers a useful overview of lupus both clinically and serologically in Europe in the 1990's.

In the first phase of this study, a data-base containing clinical and laboratory findings of 704 patients with systemic lupus erythematosus (SLE), originating from 29 centres and 14 countries, was used to assess the validity of 4 common indices of disease activity, SLAM, BILAG, SLEDAI and SIS. The physician's judgement of activity was assumed as the unique reference criterion (gold standard). Computer programmes were developed to calculate automatically the 4 activity indices; this computation appeared to correspond with manual computations in a sample of 60 appropriately selected cases. All 4 indices were closely correlated with each other (r in the range of 0.716 to 0.872), and with the physician's score (r in the range of 0.620 to 0.719). In the second phase of the study the activity index developed in part I (ECLAM) was prospectively validated, and its performance compared to that of the other scales, both as a single state index and as a transition index (i.e., its ability to assess disease activity at a single point in time and to detect variations in consecutive readings). A computer-assisted clinical chart was prepared for this purpose. This chart allowed us to calculate automatically all the indices. Two consecutive observation times (time 0, and time 1 three months later) were included in the study protocol. Data on 75 patients from 19 centres were collected, and each patient was observed twice. All the computed indices were closely correlated, both at time 0 (r ranging from 0.725 to 0.884), and at time 1 (r ranging from 0.607 to 0.833).(ABSTRACT TRUNCATED AT 250 WORDS)

A European Consensus Group study, involving 29 centres from 14 countries, was performed in order to reach agreement on the definition of disease activity in systemic lupus erythematosus (SLE) and to construct a new disease index. Data on 704 lupus patients were collected and analysed, using univariate and multivariate statistical procedures, to select those clinical and laboratory features of the disorder which best correlate with the global assessment of disease activity assigned to the patients by the physician of each participating centre. A combination of 15 clinical and laboratory variables was shown to be the best predictor of disease activity in SLE. A European Consensus Lupus Activity Measurement (ECLAM) was then formulated. This index included the 15 selected variables, weighted (with some adjustments) according to their respective regression coefficients in the multivariate model. ECLAM appears to be an effective instrument for scoring patients with different degrees of disease activity. This is the first SLE disease activity index based on data from a very large number of lupus patients followed at a large number of lupus centres in different countries. It might therefore very well serve as a standardised measure for future European clinical studies. Final assessment of the validity, reliability and sensitivity of this index is now underway.

A patient with systemic lupus erythematosus (SLE)/erosive arthritis overlap is described who was also shown to have late latent treponemal infection. The possibility of serological reactivation is discussed. Since antiphospholipid antibodies are the basis of the reagin tests for syphilis as well as being present in patients with SLE, difficulties with diagnosis may ensue.

Twenty five patients with idiopathic myositis attended this department for long term follow up from 1980 to 1989. Twelve patients had primary polymyositis (four men, eight women) and six had primary dermatomyositis (three men, three women); five women had an overlap syndrome. Two patients had a malignant condition associated with the myositis. The mean age at diagnosis was 40 years. All of the patients had proximal muscle weakness, 18/25 had a raised creatine kinase value (mean 2325 IU/l), 19/20 had an abnormal electromyogram, and 19/24 had positive muscle biopsy samples. Of the disease specific antibodies, anti-Jo-1 was detected in only 1/21 patients tested (three patients with fibrosing alveolitis were negative for this antibody), but the 56 kDa antibody was detected in 12/17 patients. The HLA data analysed in the white patients (17/25) showed that 6/8 of those tested were HLA-DR3 positive. All patients were treated with prednisolone and azathioprine was used for 14/25 patients. Only three deaths occurred during the eight year follow up, but there was a substantial morbidity, which may reflect the referral pattern. Muscle strength tests and creatine kinase levels were useful in recording the response to treatment in some patients. These data emphasise that careful long term follow up of patients with myositis is mandatory and that although the present treatment strategy has substantially reduced the death rate, morbidity associated with the disease remains a major problem.

A prospective, double blind, cross over study assessing the effects of a low fat, high marine oil diet in 27 patients with active systemic lupus erythematosus has been performed. The patients were given 20 g daily of MaxEPA (eicosapentaenoic acid) or 20 g of olive oil (placebo) in matching capsules added to a standardised isoenergetic low fat diet. When individual outcome measures of the 17 patients who completed the full 34 week study were considered 14 who were receiving MaxEPA achieved useful or ideal status, whereas 13 receiving placebo were rated as worse or no change. The difference between the two types of capsule was statistically significant. No major side effects were noted, and it is suggested that dietary modification with additional marine oil may be a useful way of modifying disease activity in systemic lupus erythematosus.

Dual energy x ray absorptiometry and a wide range of blood and urine tests were used to assess the propensity of patients with systemic lupus erythematosus to develop an impairment of bone mineral density. Surprisingly, in this preliminary study no significant differences in bone mineral density were found when patients taking 10 mg or more of prednisolone for six months or longer were compared with those who had never taken prednisolone.

Clinical features and immunogenetics were assessed in 100 SLE patients attending a rheumatology clinic for periods ranging from six months to 11 years (mean five years). Five-year survival was 88 per cent. Joint problems (94 per cent), rash (90 per cent) and haematological abnormalities (89 per cent) were the most common clinical features; neuropsychiatric disturbance (45 per cent) and renal disease (29 per cent) were seen less frequently. A range of serological abnormalities was found, including antinuclear antibodies (98 per cent) and antibodies to phospholipids (38 per cent). Anti-Sm antibodies (7 per cent) showed a marked ethnic bias. Tissue typing confirmed the importance of genetic factors by demonstrating significant increases in A1, B8 and DR3 in white Caucasians. The composite phenotype A1,B8,DR3 was present in 35 per cent of white Caucasian patients with SLE. The A1,B8 phenotype was associated with a relative risk of 8.0 and B8,DR3 with a relative risk of 8.32.

A detailed retrospective study of 105 patients with Sjögren's syndrome (50 primary and 55 secondary cases), showed that 31 had vasculitis and 18 had neurological abnormalities which after full investigation were not attributable to other causes. Most of the neurological symptoms were mild and when found in patients with secondary Sjögren's syndrome were more characteristic of the underlying autoimmune rheumatic disease. We found no significant association between the frequency of either vasculitis or any autoantibodies and the presence of neurological disease, but did confirm a significant association between vasculitis and the presence of antibodies to extractable nuclear antigens. We therefore question whether severe and relapsing neurological disease is common in patients with Sjögren's syndrome.

Septic arthritis is a well recognized complication of rheumatoid arthritis (British Medical Journal, 1976; Mitchell et al., 1976), particularly after joint replacement (Freeman, 1976). We report here infection with an unusual organism--Salmonella typhimurium.

Specific antibody production and the proliferative response of peripheral blood lymphocytes (PBLs) to a variety of viruses, including herpes simplex virus-type-1 (HSV-1) and varicella zoster (VZ), were studied in 7 patients with Behçet's syndrome. None of the patients produced an antibody response against HSV-1 or VZ. Furthermore, none of the patients showed a proliferative response to VZ, and three of them also failed to mount a response to HSV-1. These results suggest that the PBLs of patients with Behçet's syndrome make an inappropriately poor antibody and proliferative response when stimulated by HSV-1 and VZ.

Clinical and enzymatic studies on two brothers with severe deficiencies of erythrocyte hypoxanthineguanine phosphoribosyltransferase (HGPRTase) are described, and are compared with similar studies of a classical case of the Lesch-Nyhan syndrome from another family. The two brothers have no neurological abnormalities, only traces of erythrocyte HGPRTase, erythrocyte adenine phosphoribosyltransferase activities approaching the high levels found in the Lesch-Nyhan patient, and similarly raised plasma and urinary concentrations of uric acid. Despite these strong biochemical similarities between the three patients, there were wide differences in the clinical case histories. In both families the enzyme deficiency appeared to be inherited as an X-linked character through asymptomatic carrier females. The relationship of HGPRTase deficiencies to the Lesch-Nyhan syndrome is discussed. Some observations relating to techniques are reported. Cellulose acetate has been found to give much better separations of labelled reaction products in low-level phosphoribosyltransferase assays than filter paper, when used as a supporting medium for electrophoresis. The analysis of hair follicles gives indications of individuals heterozygous for the enzyme deficiency, but the proportion of enzyme-deficient follicles was very small, and the test needs support from studies of other cell types. Using haemolysates, there were signs of a slow indirect conversion of hypoxanthine to inosinic acid, via inosine. Inosine appears to be labelled by a ribosyl-transfer reaction.

Thirteen haematological parameters were measured in 44 patients with rheumatoid arthritis (RA) and 39 disease control patients with ankylosing spondylitis (AS). Using a 10 000 cells per sample automated differential counter the most frequent abnormalities found were monocytopenia, low numbers of large unstained cells, basophilia and increased numbers of cells with high peroxidase activity (HPX). The total white cell count, lymphocyte and monocyte counts, the HPX count, platelet distribution width and erythrocyte sedimentation rate were able to distinguish RA from AS at a statistically significant level. Discriminant function analysis showed that a maximum of 55% of RA patients could be correctly classified into disease state when combinations of six out of seven laboratory tests were used.

Following influenza immunization, in vitro anti-influenza antibody production by peripheral blood lymphocytes (PBM) taken from some patients with systemic lupus erythematosus is shown to be impaired despite apparently normal serum antibody responses. One explanation for this finding could be the sequestration of antibody-producing cells in the lymphoid tissue. In this study, antibody production in vitro by lymphocytes from peripheral blood and lymph nodes was measured in parallel following influenza immunization of SLE patients and normal controls. Antibody production by lymph node cells was confirmed in the presence of an impaired PBM antibody response, suggesting redistribution of antibody-producing cells to the lymphoid tissue. This finding was not however, confined to SLE patients, and the relatively low serum antibody response in these individuals suggests a more generalised impairment of the immune response.

Circulating phagocytes play a major role in the defence of the host against microbial infection. In an attempt to identify the reason for the unusual susceptibility to infection of patients with systemic lupus erythematosus (SLE) various parameters of phagocytic cell function were assessed kinetically in whole blood, and the accumulation of cells in areas of inflammation was studied in vivo with the skin window technique. The effect on these parameters of conventional therapy with glucocorticoids and pulse therapy with large doses of methylprednisolone were examined. Patients on conventional doses of steroids had no abnormality of phagocyte function that might have predisposed to infection, apart from a reduced accumulation of monocytes in areas of inflammation and decreased lactoferrin secretion. Pulse therapy with methylprednisolone considerably delayed the secretion of lactoferrin and the adherence of neutrophils in most of the patients, as well as impairing bacterial killing and digestion.

A disturbance in endothelial cell (EC) function may be pathogenetic in the thrombotic tendency of patients with the lupus anticoagulant (LA). The ability of serum from normal subjects and patients with systemic lupus erythematosus (SLE), with and without the LA, to modulate the release of prostacyclin (PGI2) and the expression of procoagulant activity by cultured human EC was investigated. Only the 10% and 20% serum concentrations from patients with SLE-LA produced a significantly greater inhibition of 6-keto-prostaglandin F1 alpha (6-keto-PGF1 alpha) release (the stable metabolite of PGI2) than control serum. However, when patients with SLE-LA having Raynaud's phenomenon were excluded from this group, there was then no significant difference between the effect of the patient and control serum. Serum from patients with SLE +/- LA caused a significant increase in EC procoagulant activity compared to healthy controls. The two-stage partial thromboplastin time expressed in seconds decreased from 66 (normal) to 34 (SLE - LA) and 31 (SLE + LA), but there was no significant difference between the patients with and without the LA. The significantly increased EC procoagulant activity induced by serum from patients with SLE +/- LA may account for the observed increased incidence of thrombotic events in patients with SLE. Our data suggest that factors other than decreased prostacyclin release are responsible for the altered hemostasis observed in patients with SLE + LA.