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Poster Viewing Session 2
Conclusions: This CTC model has helped to shorten the referral lag time, as well
as management lag time. The self-administered questionnaire has proved to be
valid and comprehensible. Clinical tests for CTS are not reliable predictors of the
diagnosis. There is a room for conservative therapy in the management of CTS.
Miscellaneous Rheumatic Diseases
264. EARLIER MHC CLASS I OVER-EXPRESSION RESULTS IN A MORE
SEVERE PHENOTYPE IN TRANSGENIC MODEL OF MYOSITIS
Thursday 04 May 2006, 08:30–10:00
i111
Results: Two males and five females, age range 26 to 62 yrs (median age 47)
disease duration from 5 months to 8 yrs, took part. Interviews produced rich
descriptions. Issues related to bladder and bowel involvement emerged from the
preliminary data and these themes were explored in more depth in later interviews.
Most patients experienced frequency, urgency and pain similar to cystitis on
micturition. When asked to describe these organs, they expressed the bladder and
to a lesser extent the bowel, were enormous and felt a sense of the organs merging
with their affected leg. One patient reported no dysfunction or distortion.
Conclusions: Findings from this qualitative study indicate that CRPS patients do
suffer from disturbances of visceral sensation and perception including pain,
supporting our hypothesis. Some of whom had extensive urological investigations
which were negative but without a diagnostic label. This has implications for clinical
practice. Further research is required in this area.
C. K. Li, B. Singh and L. R. Wedderburn
1
Rheumatology Unit, Institute of Child Health, UCL, London, United Kingdom and
2
Department of Pathology and Infectious Diseases, The Royal Veterinary
College, London, United Kingdom
266.
Background: Over-expression of Major Histocompatibility Complex (MHC) Class I
on muscle fibres in inflammatory muscle diseases is a well-recognised
phenomenon. Expression of MHC on normal muscle fibres is low. We have
shown that in the most common paediatric form of myositis, juvenile dermatomyositis (JDM), this phenomenon is seen very early after presentation, and that it
may be present in the absence of infiltrating inflammatory cells (Li et al. 2004).
Methods: Using a double transgenic mouse model where MHC Class I expression
on muscle cells is controlled by the withdrawal of the antibiotic doxycycline
(Nagaraju et al. 2000), we modelled JDM by early over-expression of MHC class l at
3 weeks of age, simultaneous with weaning. Mating parents and pre-weaned female
pups were kept on doxycycline. Mice in group 1 were taken off doxycycline at time
of weaning, while those in group 2 were kept on doxycycline until 35 days. Age
matched double transgenics left on doxycycline and single transgenics were kept as
controls.
Mice were regularly weighed and observed for behavioural changes, in
particular for reduction of voluntary movement. Muscle strength was measured
by timing the ability to maintain running on an accelerating rotating device
(Rotarod). Skeletal muscles were frozen at the time of sacrifice. All procedures
comply with United Kingdom Home Office guidelines and ethical approval. Mice
were sacrificed at pre-defined time-points, or when deemed to be significantly
disabled on a scoring system as advised by veterinary support.
Results: Double transgenic mice had statistically significant reduction of strength/
stamina as assessed by Rotarod running when analysed in a regression model
factoring in that double transgenic mice exhibit weight loss over time, compared to
age-matched controls. Controls kept on doxycycline did not show significant weight
loss or weakness (n ¼ 10). Mice from group 1 developed severe and fatal disease
compared to the late group. 69% (24/35) died spontaneously or were deemed to
have severe disease to be culled at 90 days of age (69 days after transgene
activation), compared to group 2 where the same severity was not reached by
250 days. Histological analysis revealed muscle necrosis, fibre size variation,
hyalinisation, loss of striations, attempted muscle regeneration and predominantly
macrophagic inflammatory infiltration in both groups. A scoring system encompassing these features was devised and comparison of mice from the two groups
(matched by transgene activation time) and age matched single transgenic
controls was performed: the differences in the scores were statistically significant
when comparing either double transgenic group to the control group (Kruskal
Wallis, P ¼ 0.03), but did not show significant difference between the two double
transgenic groups.
Conclusions: Our data suggest that pathological upregulation of expression of
MHC Class I at a time point when muscle fibres are growing in early life, may have
a different effect than the same process in adult mature fibres.
Background: Minocycline, a semi-synthetic tetracycline used predominantly as a
treatment for acne, has been recognised as a cause of a drug induced lupus since
the early 1990s, with over 100 cases now reported in world literature. For an illness
to be recognised as a drug induced lupus, symptoms should be induced only after
exposure to the drug, and recur on rechallenge. In a series reported by Lawson et
al. [1], oral rechallenge with minocycline induced an illness characterised by fever,
polyarthralgia and myalgia with raised CRP, within a few hours of a single oral
dose. This suggests a cytokine mediated process. The aim of our study was to
produce an in vitro rechallenge with minocycline, in patients previously diagnosed
with a likely minocycline induced lupus, and observe the response of TNF and
other pro-inflammatory cytokines. Idiopathic lupus patients not previously exposed
to minocycline were used as controls.
Methods: PBMCs were isolated from 3 patients diagnosed with minocycline
induced lupus, in whom minocycline had been withdrawn, and 3 patients with
idiopathic lupus, meeting ACR criteria for diagnosis but with quiescent disease.
PBMCs were then cultured with minocycline at 5 and 50 g/ml concentrations, with
and without LPS stimulation. TNF mRNA production was measured by Realtime
PCR, at 0, 4 and 18 hr.
Results: In 1 patient with a severe minocycline induced illness consisting of fever,
rash and a small joint polyarthritis starting within 3–4 weeks of commencing
minocycline, a marked elevation in TNF mRNA production was seen after 18 hr of
culture with 50 g/ml minocycline compared with the samples not cultured with
minocycline, and the lupus controls (Fig. 1). The remaining patients, with less
severe clinical presentations did not demonstrate a TNF response which differed
from those with idiopathic lupus.
Conclusions: This study has demonstrated a convincing TNF response to in
vitro rechallenge with minocycline in a patient presenting with an acute illness 3 to
4 weeks after commencing minocycline, which resolved on withdrawal of the drug.
This supports the hypothesis that a cytokine mediated mechanism underlies this
illness. The lack of a similar response in the remaining patients, whose symptoms
were milder, and occurred only after 12–18 months of minocycline use may reflect
a different disease mechanism, a sub-threshold minocycline dose, or that the
observed illness following in vivo rechallenge is the result of hepatic metabolism.
We believe that this area is worthy of further study in a larger series, as the
technique has the potential to be used as part of a diagnostic test for minocycline
induced lupus.
MINOCYCLINE INDUCED LUPUS – AN IN VITRO RECHALLENGE
J. R. Maxwell, D. Mewar, E. M. Heath, A. L. Coote, M. Akil and A. G. Wilson
Division of Genomic Medicine, The University of Sheffield, Sheffield,
United Kingdom
265. COMPLEX REGIONAL PAIN SYNDROME IS ASSOCIATED WITH
VISCERAL DISTURBANCE IN BLADDER AND BOWEL: A HYPOTHESIS
H. E. Cohen1,2, J. S. Lewis1,3 and D. R. Blake1,2
1
Rheumatology, The Royal National Hospital for Rheumatic Diseases, Bath,
Somerset, United Kingdom, 2School of Health, University of Bath, Bath,
Somerset, United Kingdom and 3School of Health Professions and Rehabilitation
Sciences, University of Southampton, Southampton, United Kingdom
Background: Peripheral and central sensitisation of visceral afferent nerve fibres
may occur in an analogous fashion to that demonstrated in sensory motor nerve
fibres with chronic pain states. Lower limb visceral and sensory afferent nerve fibres
travel together possibly generating sympathetic nociceptive expansion. This could
cause increased awareness of visceral sensations that would be interpreted as
painful. Similarly, neuroplastic cortical remapping may occur which could give rise to
disturbances of autonomic visceral sensation. On this basis we would hypothesise
that patients with complex regional pain syndrome (CRPS) would have disturbances
of visceral sensation and perception including pain.
Methods: Following informed consent, 7 consecutive patients with lower limb
involvement who met the International Association of the Study of Pain CRPS
classification were interviewed. Qualitative methods were used to explore
participant experience and perceptions regarding their bladder and bowels.
These semi-structured interviews were coded and analysed utilising a grounded
theory approach and themes emerged from the data.
FIG. 1.
Reference
1. Lawson et al., Rheumatology 2001;40:329–335.
i112 Thursday 04 May 2006, 08:30–10:00
267. ARTHROPATHY ASSOCIATED WITH HEPATITIS
C VIRUS INFECTION
A. A. Elbeialy1, S. M. Zaky2 and A. M. Bersy3
1
Rheumatology, Azhar Faculty of Medicine, Damietta, El Salvador, 2Enterology,
Azhar Faculty of Medicine, Damietta, Egypt and 3Clinical Pathology, Azhar
Faculty of Medicine, Damietta, Egypt
Background: Arthropathy associating HCV is common, affecting up to 20% of
HCV-infected individuals, and is commonly presented as rheumatoid-like, with
symmetrical inflammatory polyarthritis involving mainly small joints, or, less
commonly large joints, and is associated with a positive rheumatoid factor test. It
is typically non-deforming, and not associated with articular bony erosions. This
study was carried out to assess the incidence of rheumatoid like manifestations in
association with HCV infection and its characterization.
Methods: A cohort of 100 patients with clinically evident HCV infection, were
examined for the presence of articular or musculoskeletal problems. Another 100
rheumatoid arthritis patients were screened for the presence of concomitant HCV
infection. CBC, ESR, ALT and AST enzymes, serum uric acid, antinuclear, anti
smooth muscle, antimitochondrial, and extractable nuclear antigen autoantibodies,
serum rheumatoid factor, and mixed cryoglobulins were all measured. HCV
antibodies were tested for all patients. HCV RNA sequences were determined in
rheumatologically affected patients.
Results: Polyarthropathy was reported in 25 HCV patients (25%), 2 of them had
true RA. The other 23 patients had not fulfilled the ACR criteria, although they had
polyarthropathy of hands and/or feet, small and large joints, and a positive
rheumatoid factor. They had neither rheumatoid nodules, morning stiffness, nor
diagnostic X-ray findings. On the other hand, HCV infection was detected in only
4% of RA patients. Rheumatoid factor autoantibody was detected in 100% of HCV
patients with arthropathy vs 69% of those without arthropathy. ESR was
significantly raised in patients with arthropathy than those without arthropathy,
while haemoglobin was significantly lower in the arthropathy group. Arthropathy
group significantly had viraemia (88%), which was of the moderate and low levels
as referenced by the used kits. The arthropathy group had significantly longer
duration of HCV infection (10.6±4.2 yrs) prior to complaining of joint problems.
Conclusions: Rheumatological symptoms and autoimmune markers are common
in HCV infection and are usually overlooked. Patients with unexplained joint pains
or rheumatological symptoms of unknown origin should be screened for HCV,
because treatment would be different, and the outcome of proper treating HCV
arthritis would be excellent.
268. HUMAN IMMUNODEFICIENCY VIRUS-ASSOCIATED RHEUMATIC
MANIFESTATIONS IN A WELL-DEFINED HIV-POSITIVE COHORT IN THE UK
K. E. Walker-Bone1, M. Fisher2, D. Churchill2 and G. Dean2
1
Department of Rheumatology, Brighton & Sussex Medical School, Falmer,
Brighton, East Sussex, United Kingdom and 2HIV/GUM, Royal Sussex County
Hospital, Brighton, East Sussex, United Kingdom
Background: Since the discovery of human immunodeficiency virus (HIV) in 1984,
HIV has become a global pandemic. Soon after the virus was discovered, early
case reports suggested that untreated HIV infection was associated with rheumatic
manifestations such as spondyloarthropathies, psoriatic arthritis, Reiter’s syndrome, and a diverse range of connective tissue diseases and vasculitis. Now
however, highly active antiretroviral therapy (HAART) has dramatically improved
the morbidity associated with HIV infection and rates of survival have improved
considerably. Currently unknown however, is what effect, if any, the advent of
HAART has had on rheumatic syndromes associated with HIV infection.
In Brighton, East Sussex, there is a high prevalence of HIV infection and
patients have been studied prospectively for more than 10 yr. Currently, there is a
well-defined cohort of 1100 patients undergoing regular follow-up and assessment.
The objectives of this study was a prospective evaluation of rheumatic symptoms
among any member of the cohort.
Methods: In collaboration with the team of local HIV/GUM physicians, a dedicated
rapid response rheumatology clinic has been set up adjacent to the premises of the
HIV/GUM department. All HIV positive patients reporting symptoms or signs of
rheumatic disease are referred for assessment by one Consultant Rheumatologist.
Symptoms and signs are classified, where possible, according to recognised
classification criteria and investigation and treatment plans initiated in collaboration
with the HIV/GUM physician.
Results: To date, 42 patients with HIV infection and musculoskeletal symptoms/
signs have been referred to the clinic since January 2005 (equivalent to 5% annual
incidence): 34 men (81%) and 8 women (19%). The group was predominantly
homosexual (71%). Arthralgia was common (5/42, 12%). Two cases of chronic
Reiter’s syndrome (2/42, 5%) and three other cases of seronegative arthritis were
diagnosed (one enteropathic associated with ulcerative colitis), and one psoriatic. 2
patients have presented with SLE, according to ACR criteria and one patient with
Diffuse Idiopathic Lymphocytosis Sydrome (DILS). Two male patients have
presented with gout. Two female patients fulfilled the ACR criteria for the diagnosis
of fibromyalgia syndrome (5%). Soft tissue regional pain syndromes were also
common, including: carpal tunnel syndrome, lateral epicondylitis, pre-patellar
bursitis, shoulder pain and neck pain. Most patients had low viral loads and high
CD4 cell counts at the time of presentation and the majority were taking HAART.
To date, intra-articular injections have been undertaken in 5 patients under strict
sterile technique. Two patients have been commenced on corticosteroids.
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Conclusions: Rheumatic symptoms were common among this HIV positive
population. In comparison with the pre-HAART era, there appears to be a changing
spectrum of rheumatic conditions. HAART may be associated with autoimmune
rheumatic disease through the phenomenon of immune reconstitution.
269.
AUTOLOGOUS STEM CELL TRANSPLANT IN STILL’S DISEASE
H. V. Reddy1, V. V. Kaushik1, T. D. Kennedy1, P. J. Prouse2 and J. Thachil3
1
Rheumatology Department, Royal Liverpool University Hospital, Liverpool,
United Kingdom, 2Rheumatology Department, North Hampshire Hospital,
Basingstoke, Hants, United Kingdom and 3Haematology Department, Royal
Liverpool University Hospital, Liverpool, United Kingdom
Background: Still’s disease is a systemic disease characterised by polyarthritis,
intermittent fever and a typical rash [1]; other clinical features include
lymphadenopathy, hepatosplenomegaly, anaemia, leucocytosis and characteristically significantly raised inflammatory markers and ferritin. The first reported use of
autologous Hematopoietic Stem Cell Transplant (HSCT) in juvenile idiopathic
arthritis was in children with the most severe and longstanding systemic disease,
with much irreversible erosive joint destruction [2]. We present 2 patients with Still’s
disease successfully treated with HSCT.
Methods: A 24-year-old woman was diagnosed with Still’s disease at the age of
14. Her disease was characterised by frequent flares with no significant joint
damage and was steroid dependant. She failed to respond to traditional DMARDS,
biologic therapies and IVIG. Based on previous reports of successful HSCT [2, 3,
4], she underwent this procedure in May 05. The post transplant period was
complicated by persistent fever for 3 months, thought to be viral in origin. She also
had 2 episodes of severe autoimmune haemolysis with frank haematuria
associated with Kidd group antibodies. She recovered well and is currently in
remission, with normal inflammatory markers. She is being gradually weaned off
prednisolone (current dose 2.5 mg daily).
The second report is a 34-year-old woman with the diagnosis, based on
intermittent fever, rash, arthritis, leucocytosis, anaemia and raised inflammatory
markers. She was steroid dependant and had significant erosive disease. She
failed to respond to traditional DMARDS and biologic therapies. She underwent
HSCT in early 2003. She responded very well and was in remission soon after with
normalised inflammatory markers. She had a successful pregnancy in 2004 giving
birth to a healthy baby boy in October and to date remains in remission.
Results:
Conclusions: Still’s disease accounts for 10–20% of patients with JIA. The
pathogenesis is unknown and management includes NSAIDs, high dose
corticosteroids and intravenous immunoglobulin courses. There are a number of
reports showing moderate efficacy with the use of traditional DMARD therapy.
However DMARD therapies appear to help the articular symptoms more than the
systemic symptoms. With the advent of biologic therapies, these have been tried in
a few patients with varying degrees of success. Based on our two patients and
other published reports Hematopoietic Stem Cell Transplant (HSCT) appears to
offer a viable alternative and hope for patients with recalcitrant Still’s disease.
However the potential risks associated with the procedure should be weighed
against its benefits before being offered to patients. More research is required into
this before it can be recommended as a definitive management option.
References
1.
2.
3.
4.
Bull Rheum Dis 1972;23:712.
Lancet, 353,550–3.
Annals of the Rheumatic Diseases, 63;1318–26.
Bone Marrow Transplant 2000;25:1307–10.
270.
ADULT ONSET STILL’S DISEASE. CLINICAL COURSE AND
OUTCOME; SOUTHEND EXPERIENCE
D. Makkuni and B. Dasgupta
Rheumatology, Southend General Hospital, Southend, Essex, United Kingdom
Background: Adult onset Still’s disease(AOSD) is a rare systemic inflammatory
condition of unknown aetiology. Diagnosis and treatment of this condition can be
difficult due to the lack of diagnostic and management guidelines. The disease can
have a variable course and outcomes with some patients presenting with recurrent
systemic symptoms like fever and rash or others evolving into a chronic
arthropathy with serious functional disability. The treatment options have improved
with the advent of biological agents. Through this study we look into the local
experience of the management of AOSD.
Methods: Patient with an established diagnosis of AOSD, fulfilling the Yamaguchi
criteria (Major-fever, arthralgia, rash and leucocytosis Minor-sorethroat, liver
dysfunction, negative ANA and Rheumatoid factor, Lymphadenopathy) were
identified through the Rheumatology data base at the Southend hospital. The
clinical presentation, course of the disease and response to treatment were
studied.
Results: Eight cases of AOSD were identified, 5 female and 4 male patients. The
age of presentation varied between 28–80 yr (only 3 patients above the age of
40 yr). The joints most commonly affected were wrists, ankles and knees (Table 1).
Six patients had tenosynovitis and out of these 2 subsequently developed
stenosing tenosynovitis needing orthopaedic intervention. Systemic signs apart
from fever and rash were pleurisy, pericarditis, anemia, lymphadenopathy,
hepatosplenomegaly, mild transaminitis and positive urine sediments. All patients
initially received highdoses of steroids to control the disease (50–80 mg oral
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Thursday 04 May 2006, 08:30–10:00
prednisolone and 500–1 gm IV methylprednisolone) followed by methotrexate. Two
patients went into complete remission 2 and 4 yr of treatment but the rest
developed chronic polyarthropathy and these patients required a maintenance
dose of 10–15 mg prednisolone along with 15–20 mg methotrexate. Three patients
received biologic therapy (2 etanercept and 1 Anakinra) and showed very good
response to therapy. Of the eight patients only two patients showed progressive
articular damage. One patient underwent total knee replacement.
Conclusions: The natural history of AOSD from this study has shown a significant
tendency for chronicity. Six out of 8 patients developed chronic articular problems
with poor functional outcome. Tenosynovitis of flexor and extensor tendons were
seen at the time of presentation in majority of cases. All patients required high
doses of steroids for inducing as well as maintaining the disease in remission in
addition to therapeutic doses of methotrexate. Anti TNF drugs like etanercept and
IL1 blocker Anakinra were found to be extremely useful in controlling the disease in
a subset of patients with chronic active disease. In future an aggressive treatment
strategy including early introduction of biologic agents should be tried in these
patients.
Articular distribution in AOSD
Tenosynovitis
Wrists
Ankles
Knee
Others
6
6
3
3
2
i113
(DAS(4) ¼0.54*sqrt(RAI) þ 0.065*
(swollen44) þ 0.33*Ln(ESR) þ 0.0072*GH;
DAS(3) ¼ 0.54*sqrt (RAI) þ 0.065*(swollen44) þ 0.33*Ln(ESR) þ 0.22; DAS-4(crp)
¼ 0.54*sqrt(RAI)þ0.065*SJC44 þ 0.17*ln(CRP+1) þ 0.0072*GH þ 0.45;
DAS3(crp) ¼ 0.54*sqrt(RAI)þ0.065*SJC44 þ 0.17*ln (CRP+1) þ 0.65; DAS28(4) ¼
(0.56*sqrt(t28) þ 0.28*sqrt(sw28)þ 0.70*Ln(ESR) þ 0.014*GH; DAS28(3) ¼ [0.56*
sqrt(t28) þ 0.28*sqrt(sw28) þ 0.70*Ln(ESR)]*1.08 þ 0.16; DAS28-CRP(4) ¼ 0.56*
sqrt(TJC28) þ 0.28*sqrt(SJC28) þ 0.36*ln(CRP+1) þ 0.014* GH þ 0.96; DAS28CRP(3) ¼ [0.56*sqrt(TJC28) þ 0.28*sqrt (SJC28) þ 0.36*ln(CRP+1)]*1.10 þ 1.15).
All data are permanently stored and data retrieval for analysis in most format is
possible (excell, access, spss . . .), as well as download of pre-existing data set.
Results: I designed a software for easy DAS calculations however, with the unique
ability to download pre-existing data, permanently store and retrieve data in most
desired formats. The capture screen was designed to resemble the paper version
of DAS calculation used in most research departments. All procedures are
automatic and data entry is similar to what would be stored on paper records.
There is no need to know the database language and procedures are simplified to
the maximum. The computer and web-interface versions are available and the
mobile phone and pocket computer versions are currently under further
development.
Conclusions: This new tool provides the opportunity for research departments to
record DAS from any set up (computer, web-interface, pocket-PC or mobile phone)
in a unique format that can be stored in a centralised departmental database. The
software will be compatible with other clinical data storage systems currently under
development.
Others-MCPjoints.
271. AN EPIDEMIOLOGICAL STUDY OF CONNECTIVE TISSUE DISEASES
PRESENTING AS FEVER OF UNKNOWN ORIGIN (FUO) OVER 5
YEARS DURATION
F. I. Fahmy1, A. A. Elbeiay1, S. M. Zaky2 and A. M. Bersy3
1
Rheumatology, Azhar Faculty of Medicine, Damietta, Egypt, 2Tropical Medicine,
Azhar Faculty of Medicine, Damietta, Egypt and 3Clinical Pathology, Azhar
Faculty of Medicine, Damietta, Egypt
Background: The diagnostic spectrum of fever of unknown origin (FUO) has
changed since its original definition. The number of cases of FUO due to infectious
diseases has declined and there has been a corresponding increase in the number
of cases due to multisystem diseases, such as systemic lupus erythematosus,
rheumatoid arthritis, and Still’s disease. Unfortunately, many of these rheumatic
diseases presenting with FUO may remain undiagnosed for months, and reach the
rheumatologist very late. This prospective study was devoted to point out the
frequency of patients presenting with FUO associated with connective tissue
diseases, over 5 yrs’ duration in our country.
Methods: This study included a series of 578 patients, all had been attending
Tropical Medicine Departments, al-Azhar University, and Abbasseya Fever
Hospital, in Cairo, over 5 yrs duration, from 2000 to 2004. Each patient was
subjected to complete evaluation and meticulous medical examination, followed by
intensive laboratory studies, documented thoroughly with respect to past and
present medical history. Each patient had fulfilled Petersdorf and Beeson criteria
for FUO.
Results: The most common causes of FUO were infectious diseases (294,
50.86%), connective tissue diseases (CTDs) (107, 18.51%), neoplasms (105,
18.17%), and other causes (72, 12.46%). SLE was the most common cause of
CTDs associated with FUO (45.8%), followed by RA (19.6%), and Still’s disease
(14.0%). Other CTDs were polymyositis (5.6%), dermatomyositis (2.8%),
polyarteritis nodosa (1.9%), Behcet disease (1.9%), sclerodermia (2.8%), rheumatic fever (1.9%), mixed connective tissue disease (0.9%), inflammatory bowel
disease (0.9%), psoriasis (0.9%), and eosinophilic fasciitis (0.9%). The study
showed that most of the patients diagnosed as having CTD presented with fever
were in the middle age group (30–40 yrs old), most of them were females
(95.09%).
Conclusions: These results give us a strong impression that we have not to
neglect the increased incidence of CTDs from routine medical clerking of patients
with FUO, and it is valuable to include a rheumatologist in the consultation of all
patients with FUO. Together with a logical and disciplined approach, this might
help early detection and diagnosis of CTDs presented with FUO.
272.
AN ELECTRONIC TOOL TO CALCULATE AND STORE DAS SCORES
P. Gente
Software Designer, Paris, France
Background: The need for rapid disease activity score calculations has been
recognised in Rheumatology both clinically and for research purposes. However,
some of the current calculators do not allow data retrieval or even long term
storage. I have created a software with a single capture screen for DAS and
DAS28 calculations with automatic download and storage of all individual
component of the score into a database allowing data analysis in the long term.
Methods: A new database language allowing the design of applications for
computers, pocket-PC, mobile phones and a web-interface was used. A single
screen for the capture of all data set (patient ID, date, visit number for followup,
tender swollen joint count, ESR, CRP and patient’s global assessment of disease
activity) was designed using a combination of free data entry fields, pre-set
boxes and click-button. Different DAS calculations appear automatically
FIG. 1.
273.
RISK FACTORS FOR OSTEOPOROSIS IN SUBJECTS WITH BENIGN
JOINT HYPERMOBILITY SYNDROME (BJHS): COMPARISON TO
POPULATION BASED CONTROLS
A. Stewart1, A. J. Black2 and D. M. Reid1
1
Department of Medicine and Therapeutics, University of Aberdeen, Aberdeen,
United Kingdom and 2Department of Rheumatology, NHS Grampian, Aberdeen,
United Kingdom
Background: Previous studies have shown that those with joint hypermobility are
at greater risk of osteoporosis. These studies examined patients suffering from
Ehlers-Danlos syndrome or benign hypermobility. These studies involved a variety
of techniques to measure bone mass. The aim of our study is to examine
osteoporosis risk factors, bone mass and bone turnover markers in subjects with
BJHS and controls.
Methods: Individuals with diagnosed BJHS were identified using an electronic
diagnostic database, with a total of 54 subjects identified. Of these 50 were invited
to take part. Our response rate was 70% with 35 individuals responding positively
and of these 30 attended for their appointments. To date the bone mass has been
measured in BJHS cases only. Age-matched controls (n ¼ 120) are part of a
population-based study (OPUS) in whom data are available on risk factors for
osteoporosis and historical, but not current, bone mass. A medic examined the
joints to provide a Beighton score to test whether the Brighton criteria for BJHS
were met. A questionnaire used in the OPUS study was completed by both cases
and controls. 2 analysis was used to detect differences in prevalence of risk
factors.
Results: The average age of both cases and controls was 37 yrs, and largely
premenopausal (86.7% of cases, 74.2% of controls, P ¼ 148). Of the cases 25
(83.3%) fill the criteria for hypermobility syndrome as set by the Brighton criteria.
We assessed differences in osteoporosis risk factors. There were no significant
differences between cases and controls for age (P ¼ 0.818), height (P ¼ 0.376) or
weight (P ¼ 0.694). Differences were found for self-reported osteoporosis risk
factors. Significantly higher prevalence was found in cases compared to controls
for the following risk factors; previous fracture (P ¼ 0.007, cases 60%, 33.3%):
family history of osteoporosis (P ¼ 0.012, cases 33.3%, controls 13%): oral
corticosteroid use (P ¼ 0.012, cases 13.3%, controls 2.5%). We asked about
osteoporosis medications and the cases indicated a higher prevalence (P ¼ 0.011)
of ever taking calcium (16.7%) in comparison to the controls (3.3%), but
i114 Thursday 04 May 2006, 08:30–10:00
no differences in other forms of treatment. No differences were found for smoking,
alcohol intake or exercise, but the cases did record significantly reduced days of
current intake of yoghurt (P ¼ 0.004), milk (P ¼ 0.027), milk products (P ¼ 0.007).
Back pain was high in both groups, but significantly higher in the cases (P ¼ 0.009,
cases 86.7%, controls 61.7%). However cases graded their general health better
than controls (P ¼ 0.002).
Conclusions: Subjects with BJHS may be at higher risk of osteoporosis due to an
increased prevalence of previous fracture, family history of osteoporosis,
corticosteroid use, and a decreased intake of calcium based foods. Bone mass
has been measured in the BJHS subjects and will be compared to the controls in
whom bone mass will be reassessed in the next few months. This will indicate
whether the increased risk factors translates into lower bone mass.
274. MRI APPEARANCES IN LÖFGREN’S SYNDROME; IS ANKLE
SWELLING CAUSED BY ARTHRITIS, TENOSYNOVITIS OR
PERIARTHRITIS?
J. D. Rees, P. Peterson and B. E. Bourke
Department of Rheumatology, St George’s Hospital, London, United Kingdom
Background: The rheumatological presentations of sarcoidosis are diverse. In the
acute form of arthritis seen in Löfgren’s syndrome features include a variable
inflammatory picture, sometimes mimicking either a reactive arthritis or rheumatoid
arthritis. Features of periarticular soft tissue swelling, synovitis and tenosynovitis
may be present clinically and are often associated with severe disability.
Methods: We present MRI findings in two typical cases of acute sarcoidosis. Both
cases occurred in young adult Caucasians who presented with acute and disabling
symptoms. Both patients had bilateral hiliar lymphadenopathy on chest X-Ray,
elevated serum ACE levels and bilateral lower leg swelling.
Results: MRI findings in the two cases were very different as was clinical outcome.
Case One In the first case the MRI confirmed a periarthritis rather than a true
inflammatory arthritis. The main abnormalities demonstrated were of subcutaneous oedema, an effusion within the ankle joint and oedema within the sinus tarsi
(Fig. 1). The patient was treated with NSAIDs alone and there was a rapid
resolution of the symptoms and signs.
Poster Viewing Session 2
Conclusions: We describe the features of acute sarcoidosis of the ankle joints in
which have been investigated acutely with MRI scanning. The MRI has been able
to define accurately the periarticular vs articular nature of the acute manifestations
of the arthritis seen in Löfgren’s syndrome. We suggest that an early MRI may give
prognostic information regarding the course of the arthritis. Patients with a
periarthritis rather than a true inflammatory arthritis are likely to have a more
favourable clinical outcome.
Paediatric and Adolescent Rheumatology
275.
NEUROMUSCULAR IMPAIRMENTS IN CHILDREN DIAGNOSED
WITH HYPERMOBILITY SYNDROME: A PRELIMINARY STUDY
F. A. Fatoye1, F. Macmillan1, S. Palmer2, P. Rowe3 and
S. Wilkinson4
1
Physiotherapy Subject Area, Queen Margaret University College, Edinburgh,
United Kingdom, 2Faculty of Health & Social Care, University of the West of
England, Bristol, United Kingdom, 3Bioengineering Unit, University of Strathclyde,
Glasgow, United Kingdom and 4Physiotherapy Department, Royal Hospital for
Sick Children, Edinburgh, United Kingdom
Background: Impaired joint proprioception has been found in association with
hypermobility syndrome (HMS) (Mallik et al. 1994; Hall et al. 1995). However, it is
uncertain if other neuromuscular indices in children with HMS are impaired.
Therefore, this study investigated a range of neuromuscular impairments in healthy
children and those diagnosed with HMS.
Methods: Seventeen healthy girls (mean age 11.3±S.D. 2.5 yr) and 13 girls
diagnosed with HMS (mean age 11.8±S.D. 1.3 yr) participated in this investigation.
The study was approved by the Education Department, City of Edinburgh Council,
and the QMUC and NHS Lothian Local Research Ethics Committees. Informed
written consent was obtained from the participants and their parents. Knee joint
kinaesthesia (KI) was assessed at 60 degrees of knee flexion and joint position
sense (JPS) was examined at both 25 and 10 degrees of knee flexion using a
motorised proprioception measuring device. Absolute angular error (AAE) was
calculated as the difference between the target and perceived angle for JPS tests.
Quadriceps and hamstrings muscle torque was measured in high sitting with the
test knee in 90 degrees using a digital myometer. Muscle torque was normalised to
body weight. Mann-Whitney U tests were used to compare the variables between
the two groups.
Results: The results of this study are presented in table 1 below:
Conclusions: The knee joint proprioception outcome measures were significantly
different in children with HMS and they also showed weaker quadriceps muscles
than the healthy controls. However, there was no hamstring muscle strength deficit
between the two groups. Clinicians should be aware of these identified
impairments in children with HMS, and a programme of proprioception and
muscle strengthening exercises may be indicated.
TABLE 1. The mean (S.D.) for joint proprioception and muscle torque
Proprioception ( )
Group
FIG. 1A.
Case Two In the second case the MRI confirmed a true inflammatory arthritis with
a marked synovitis affecting the ankle joint. Additionally there were effusions of
numerous tendon sheaths (tibialis posterior, flexor digitorum longus and flexor
hallucis longus). The patient is still symptomatic two years following onset despite
treatment that has included NSAIDs, corticosteroids, hydroxychloroquine and most
recently methotrexate.
Healthy
HMS
P-values
Muscle torque (Nm/kg)
KI at 60
AAE at 25
AAE at 10
Quadriceps
Hamstrings
1.90 (0.83)
3.15 (1.91)
0.030*
4.56 (2.94)
7.39 (3.69)
0.045*
2.33 (2.89)
4.92 (2.53)
0.011*
1.49 (0.24)
1.16 (0.30)
0.002*
0.86 (0.48)
0.63 (0.21)
0.161
*Statistically significant at < 0.05.
References
1. Hall et al. British Journal of Rheumatology 1995;34:121–25.
2. Mallik et al. British Journal of Rheumatology 1994;33:631–37.
276.
GENERATION OF TOLEROGENIC DC FROM PATIENTS WITH
JUVENILE IDIOPATHIC ARTHRITIS
E. Sala Soriano, K. R. Newton, S. Burns and L. R. Wedderburn
Rheumatology Unit, Institute of Child Health, University College London, London,
United Kingdom
FIG. 1B.
Background: Juvenile Idiopathic Arthritis (JIA) is one of the most common
rheumatic diseases in children. There are various subtypes of JIA, with major
differences in both severity and outcome. Both T cells and Dendritic Cells (DC) are
key cell types contributing to the pathology of JIA. We have found CD4+CD25þ
regulatory T cells (Treg) in the synovial fluid (SF) of children with JIA, and DC in a
semi-mature state in the synovium. We have adapted a method previously
developed in our laboratory to investigate the interactions of DC and T cells in JIA.
Methods: Human monocyte derived DC grown from SF from children with JIA or
peripheral blood of healthy controls, were infected with adenoviral constructs (E1E3-deleted) and then matured. CFSE- labelled T cells were cultured with either
autologous DC (adenoviral infected or control) and stimulated with plate bound
anti-CD3 or PHA, or were seeded with allogeneic DC. T cell proliferation was
measured by CFSE dilution using flow cytometry.