Andrew Borg

Freedom of movement across the EU: legal and ethical issues for children with chronic disease

While freedom of movement has been one of the most highly respected human right across the EU, there are various aspects which come into play which still need to be resolved for this to be achieved in practice. One of these key issues is cross border health care. Indeed, there is an increasing awareness of standardisation of health service provision and cross border collaboration in the EU. However, certain groups particularly children may be at risk of suboptimal treatment as a result. We present the case of a child patient which highlights the complexity of this matter spanning family law, health law, social security law as well as ethical issues. EU legislation needs to ensure that children patients have access to high quality care across the EU borders.

EULAR recommendations underplay importance of severe anxiety and depression in fibromyalgia treatment

The European League Against Rheumatism recently published updated recommendations on the management of fibromyalgia (FM), including pharmacological and non-pharmacological measures.1 While non-pharmacological measures are now the first approach, there is no stratification according to severity of anxiety or depression prior to selecting treatment options. We are concerned that these recommendations seriously underplay the role of the need of expert psychological and psychiatric assessment prior to the selection of treatment strategies. Severe anxiety and/or depression may prevent the ability to comply with non-pharmacological therapy such as exercise or cognitive behavioural therapy, and the interactions with chronic pain and fatigue can become cyclical and self-perpetuating. Additionally it is known in FM that …

Characterisation of Patients with Systemic Lupus Erythematosus in Malta: A Population Based Cohort Cross-Sectional Study

Systemic Lupus Erythematosus (SLE) is a multisystemic autoimmune disorder. The aim of this study was to characterise the SLE patients living in Malta in order to estimate the prevalence and incidence of SLE and characterise the clinical presentation as well as identify any unmet needs. 107 SLE patients who fulfilled SLICC classification criteria were identified. These were invited to participate in the study by means of an interview, blood and urine tests, and filling of the following questionnaires: Fatigue Severity Scale (FSS), visual analogue scale (VAS) for fatigue, Hospital Anxiety and Depression Scale (HADS), VAS for pain, Pittsburgh Sleep Quality Index (PSQI), and modified Health Assessment Questionnaire (mHAQ). The estimated prevalence of SLE in Malta is 29.3 patients per 100,000 and the estimated incidence is 1.48 per 100,000 per year. 93.5% of SLE patients were female, and the mean age at diagnosis was 33.1 years. 60.8% were overweight or obese and body mass index (BMI) had a significant positive correlation with daily dose of prednisolone (R=0.177, p=0.046). 20.7% and 3.3% had a moderate and high disease activity, respectively, as measured by SLEDAI-2K. Disease activity had a significant positive correlation with functional disability measured by mHAQ (R=0.417, p<0.001). 56.5% had an abnormal level of fatigue (FSS >3.7) and 57.6% had a high level of anxiety (HADS ≥8). This study has identified a number of unmet needs of SLE patients, including obesity, uncontrolled disease activity, fatigue, and anxiety.

AB0622 Characteristics of systemic lupus erythematosus patients in malta; a population based cross-sectional cohort study

Background Systemic Lupus Erythematosus (SLE) is an autoimmune disorder that involves multiple systems including the skin, musculoskeletal, renal, neurological, haematologic, cardiovascular and respiratory systems. Objectives The aim of this study was to characterise the patients with systemic lupus erythematosus living in Malta, in terms of age of disease onset, BMI, co-morbidities, drug history, disease activity, damage and other factors including fatigue, sleep quality, depression, anxiety and vitamin D level. Methods The study consisted of a cross-sectional cohort study of all known SLE patients, over the age of 18, living in Malta. 92 patients who fulfilled the SLICC classification criteria for SLE, gave informed consent and an interview was carried out. Fatigue, anxiety, depression, sleep quality and disability were assessed respectively by filling in the following questionnaires: Fatigue Severity Scale (FSS), Hospital Anxiety and Depression Scale (HADS), Pittsburgh Sleep Quality Index (PSQI) and modified Health Assessment Questionnaire (mHAQ). Results 92.4% of SLE patients studied were female. Table 1 summarises the characteristics of the SLE patients. 23.9% of SLE patients were in remission (SLEDAI-2K 0), while 52.2% had a low disease activity (SLEDAI-2K 1–5) at the time of the interview. 20.7% and 3.3% had a moderate (SLEDAI-2K 6–10) and high (SLEDAI-2K 11–19) disease activity respectively. A significant positive correlation was noted between function measured by mHAQ and SLEDAI (R=0.417, p=0.000). 56.5% were noted to have an abnormally high level of fatigue (FSS >3.7). 6.5% were noted to have depression (HADS D>10) and 35.9% had anxiety (HADS A>10). 55.4% were noted to have poor sleep quality (PSQI >5) and 26.1% had an abnormal level of function (mHAQ >0.3). 15.2% were found to have vitamin D deficiency and 27.2% were vitamin D insufficient.Abstract AB0622 – Table 1 Clinical characteristics of the cohort Characteristics Values Age, mean (S.D.) years 46.9 (13.9) Caucasian race, n/N (%) 90/92 (97.8) Disease duration, median (range) years 13 (0–35) Age of SLE onset, mean (S.D.) years 33.8 (12.8) BMI, median (range) kg/m2 26.5 (17.7–53.5) Current smoker, n/N (%) 14/92 (15.2) Family history of SLE in first degree relative, n/N (%) 3/92 (3.3) Osteopaenia/osteoporosis, n/N (%) 30/92 (32.6) Hypertension, n/N (%) 22/92 (23.9) Diabetes mellitus, n/N (%) 7/92 (7.6) Fibromyalgia, n/N (%) 9/92 (9.8) Anti-phospholipid syndrome, n/N (%) 7/92 (7.6) Sjogren’s syndrome, n/N (%) 4/92 (4.3) Rheumatoid arthritis, n/N (%) 3/92 (3.3) Current prednisolone, n/N (%) 41/92 (44.6) Current hydroxychloroquine, n/N (%) 55/92 (59.8) Current azathioprine, n/N (%) 20/92 (21.7) Current methotrexate, n/N (%) 10/92 (10.9) Current mycophenolate, n/N (%) 6/92 (6.5) Conclusions This is the first population based study on SLE to be carried out in Malta. The prevalence of SLE in Malta is estimated to be 25.5 patients per 1 00 000 and the estimated incidence is 1.05 patients per 1 00 000 per year. A high frequency of obesity and vitamin D deficiency and insufficiency were noted in SLE patients. Other unmet needs include an uncontrolled disease activity, fatigue, poor sleep quality and anxiety. Disclosure of Interest None declared

Dyspnoea in lupus

A 32-year-old woman suffering from systemic lupus erythematosus presented with a 6-week history of progressive dyspnoea and pleuritic chest pain. Examination was normal apart from reduced air entry at the lung bases. Arterial blood gases showed hypoxaemia and chest X-ray revealed raised hemidiaphragms without any pleural effusions. Lung function showed a restrictive pathology while high-resolution chest CT and CT pulmonary angiogram were negative. Echocardiography showed normal ventricular diameters and no pericardial effusion. Reduced lung volumes and a positive fluoroscopic sniff test lead to a diagnosis of shrinking lung syndrome. Symptoms improved following treatment with glucocorticoids and non-invasive ventilation, but there was no change in lung function. A year later, our patient presented again with worsening dyspnoea. This time echocardiography revealed severe mitral stenosis with pulmonary hypertension. Mitral valve replacement was performed and dyspnoea resolved. Histology showed Libman-Sachs endocarditis.

SAT0729-HPR Psychological profile of patients with fibromyalgia syndrome

Background Fibromyalgia (FM) is a chronic non inflammatory condition characterized by pain and fatigue as well as physical and psychological symptoms. Management can be particularly challenging and a combination of pharmacological and non pharmacological treatments are recommended. Objectives The aim was to study the level of anxiety and depression in fibromyalgia patients and whether these were associated with symptom severity, functional status, social or demographic factors. Methods A cross sectional survey of 155 consecutive patients attending the nurse-led fibromyalgia clinic was carried out. Patients were diagnosed using the 2010 ACR diagnostic criteria. Demographic data, Widespread Pain Index (WPI), Symptom Severity Score (SSS), VAS pain and VAS fatigue were recorded. Patients filled in the self-administered questionnaires including the Revised fibromyalgia impact questionnaire (FIQR), Hospital Anxiety and Depression Scale (HADS). Patients were classified as suffering from high anxiety or depression if HADS was ≥11, moderate if 8–10 and low ≤7 for each scale respectively. Cross tabs and chi squared were used to study associations between anxiety and depression and social and demographic factors. Logistic regression analysis was performed to identify whether WPI, SSS, FIQR, VAS pain and VAS fatigue were predictors of severe anxiety and depression. Results One hundred fifty five patients (92% female) participated in the survey. The average age was 50.3 years (SD 11.5) and mean duration of symptoms 13.4 years (SD 11.3). The mean HADS-A (anxiety) was 11.8 (SD 4.13), HADS-D (depression) 9.1 (SD 3.8) and FIQR 60.3 (SD 17.6). High levels of depression were reported by 31.5%, moderate 32.1% and low 30.3%. High levels of anxiety were reported by 60%, moderate 21.2% and low 13.3%. Both high levels of anxiety and depression were found in 25.2% of patients. SSS was the single best predictor for anxiety (p=0.001) while disease duration (p=0.01), SSS (p=0.02) and FIQR (0.04) predicted depression. VAS pain and VAS fatigue were not good predictors of severe anxiety and depression. When patients with high levels of anxiety and depression were compared with those with moderate and lower levels no association was found with age or social factors including occupation, marital status, level of education and family support. Conclusions Both severe anxiety and depression were highly prevalent in our FM cohort, particularly anxiety. All FM patients need to be screened for anxiety and depression particularly those with high SSS and FIQR scores and longer disease duration. A psychiatrist and psychotherapist with a special interest in FM are essential members of the multidisciplinary team to ensure that anxiety and depression are addressed early prior to starting further interventions for FM. References Jensen KB, Fransson P, Marcus HM, Williams SCR, Choy E, Mainguy Y, Gracely R, Ingvar M, Kosek E (2010) anxiety and depression in fibromyalgia are related to poor perception of health but not to pain sensitivity or cerebral processing of pain. Arthritis & Rheumatology Journal 62(11). Ugar M, Sarp U, Karaaslan O, Tanik N, Arik HO (2015) Health anxiety and depression in patients with fibromyalgia syndrome. Journal of Inernational Medical Research 45(3):679–685. Acknowledgements NIL. Disclosure of Interest None declared

THU0108 Closing The Audit Cycle: Have Cardiovascular Risk Assessment and Management in Rheumatoid Arthritis Patients Improved?

Background Patients who suffer from rheumatoid arthritis have an increased risk of morbidity and mortality from cardiovascular disease. This is due to the high prevalence of traditional risk factors and the effect of systemic inflammation. Objectives The aim of the audit was to determine whether the cardiovascular risk assessment and management in rheumatoid arthritis patients at Mater Dei Hospital is in concordance with the recommendations by the European League Against Rheumatism (EULAR). Methods An audit was carried out retrospectively on 91 patients who suffer from rheumatoid arthritis by using the medical notes to collect data on demographics, co-morbidities, drug history and cardiovascular risk assessment and management over a two year period (August 2010 to July 2012). The results of the first audit were then disseminated through the rheumatology department and a form for cardiovascular risk assessment and management in rheumatoid arthritis patients was implemented. The audit was repeated on 107 patients and data was collected retrospectively over the two year period starting from January 2013 to December 2014. Results Documentation of cardiovascular risk factors over the two year period audited improved as follows from the first to the second audit: weight in 27.5% to 52.3%, height in 0% to 27.1%, BMI in 0% to 10.3%, smoking status in 72.5% to 93.5%, blood pressure in 72.5% to 92.5%, blood glucose in 72.5% to 97.1% and lipid profile in 54.9% to 96.3%. Documentation of smoking cessation advice improved from 15.8% to 41.1% and advice on other lifestyle changes improved from 14.3% to 18.7%. Moreover calculation of DAS28 over a one year period improved from 20.9% to 51.4%. In the first audit, 13.8% of patients who had complete data and in whom the ten year cardiovascular risk could be calculated (29 patients), would benefit from the use of a statin according to the guidelines (and were not currently receiving one). In the re-audit, this decreased to 8.6% of such patients (93 patients). In the second audit it was noted that the form that had been introduced for cardiovascular risk assessment was used in 15% (16 patients). The documentation of lifestyle advice was significantly higher (p<0.001) in the group of patients in whom the form was used. The same applies for documentation of weight (p<0.001), height (p<0.001), BMI (p<0.001) and calculation of DAS28 over a one year period (p<0.001). The prevalence of diagnosed hypertension in our cohort of rheumatoid arthritis patients was 49.0%; diabetes was 19.7%; hyperlipidaemia was 21.8%; and ischaemic heart disease was 8.8%. Conclusions Cardiovascular risk factors are highly prevalent in rheumatoid arthritis patients. This audit showed that cardiovascular risk assessment and management improved through raising awareness of its importance in the rheumatology department as well as the implementation of the cardiovascular risk assessment form. References Peters M JL, Symmons D PM, McCarey D, Dijkmans B AC, Nicola P, Kvien T K et al. EULAR evidence-based recommendations for cardiovascular risk management in patients with rheumatoid arthritis and other forms of inflammatory arthritis. Ann Rheum Dis, Feb 2010; 69: 325–33. Disclosure of Interest None declared

AB0536 Can Rheumatologists Diagnose and Manage Giant Cell Arteritis Better than Non Rheumatologists

Background Giant cell arteritis (GCA) is the commonest vasculitis affecting large and medium vessels. GCA presents with varying symptomatology which makes timely diagnosis and management challenging. A number of guidelines for the management of GCA have been published.1,2 In clinical practice, patients present to various specialists including general practitioners, general physicians, neurologists, rheumatologists and ophthalmologists which may result in wide variations in clinical care. Objectives To establish the incidence of GCA in the Maltese population and whether rheumatologists are able to diagnose and manage giant cells arteritis better than non rheumatologists. Methods This was a population observational study of Maltese patients between 2012 and mid 2015. All patients who underwent a temporal artery biopsy (TAB) were recruited and their case notes reviewed. Results There were 122 patients (80 females) who underwent a TAB over 3.5 years for suspected GCA and out of these, 29 were positive. The incidence of biopsy proven temporal arteritis in Malta was 6.4 per 100,000 inhabitants aged over 50 years. Mean age was 72.6 (SD 10.5) years. An additional 27 patients were treated for GCA on clinical grounds. Median CRP was 31 mg/L (IQR 6–87) and ESR 81 mm/hr (53.5–102.5). A total of 53 patients presented to the rheumatologists and 69 patients to non rheumatologists, and a positive TAB was obtained in 32% and 11.6% of patients respectively. Glucocorticoids were initiated before taking a TAB in 39.6% by rheumatologists and 34.7% and non rheumatologists. Overall (including TAB negative patients) 60.4% of patients presenting to the rheumatologists and 33% of patients presenting to non rheumatologists were treated as GCA. The starting dose of Prednisolone was between 40–60mg in 82% of rheumatologist treated patients compared to 65% by non rheumatologists. The tailoring regimes varied widely and documentation was poor for many patients treated by non rheumatologists. Rheumatologists treated 50% of patients with a Prednisolone dose of 40 mg or greater for at least a month, 37.5% were given lower doses while the treatment regimen was unclear in 12.5%. Non rheumatologists treated 20.8% with a Prednisolone dose of 40 mg or greater for at least a month, 33.3% were given lower doses and in 45.8% regimen was unclear. The main differential diagnoses were sepsis/infection (7 cases), PMR (4 cases), pyrexia of unknown origin (4 cases) and cluster headache (2 cases). Conclusions Malta seems to have a low incidence of GCA comparable to other Mediterranean countries but lower than northern European countries.3 GCA presents to a wide variety of specialists leading to a broad variation in treatment regimens. Non rheumatologists seem to have a lower threshold for TABs and possibly perform a number of unnecessary TABs. In order to ensure timely and appropriate management rheumatologists should take the lead and set up interspecialist pathways based on international guidelines that work in the local context. These pathways should include guidance about access, diagnosis, investigations and treatment and be disseminated to other clinical colleagues in both primary and secondary care. References Mukhtyar C, et al. Ann Rheum Dis. 2009;68:318–23. Dasgupta B, et al. Rheumatology. 2010;49:1594–7. Mohammad AJ, et al. Ann Rheum Dis. 2015;74:993–7. Disclosure of Interest None declared

Back pain in a young adult

A 32 year old man was referred to the emergency department with a one week history of worsening mid-thoracic back pain. The pain radiated bilaterally and he occasionally felt it anteriorly in the chest. It was constant in nature and there were no associated symptoms. He did not look unkempt and was accompanied by his girlfriend and brother. He had no medical history of note but was a known intravenous drug user. He admitted to injecting drugs through “a vein in his armpit” and claimed that the last time he had heroin it looked “thicker” than usual. His physical examination was unremarkable and the following blood test results were obtained: white blood cell count 11.0×109/L (reference range 4.3-11.4), haemoglobin (11.9 g/L (14.1-17.2), and troponin <0.01 µg/L (0.006-0.04). A chest radiograph was normal. He was sent home with advice about back pain and analgesia. The pain persisted and he re-presented to the emergency department but was discharged again after a normal physical examination and thoracic spine radiography, which was reported as normal. Two days later he returned with worsening back pain, which was now waking him up at night. On percussion of the spine he was tender over the mid-thoracic area. At this stage he was febrile and had a wide based gait, with his left lower limb being spastic when compared with the right. Tone was increased on the left, whereas his lower limb power was decreased on the left (4/5) compared with the right. Sensation to light touch and pin prick was decreased (but present) from the nipple downwards on both sides. Joint position sense was present at both ankles and vibratory sensation was decreased but present at both big toes and normal at the ankles. Knee and ankle jerks were increased on the left more than …

A man with generalised lymphadenopathy.

A 75 year old man with Paget’s disease, hypercholesterolaemia, and hypertension presented with a three year history of multiple neck lumps, which had never been investigated and had increased in size over the past two months. He felt generally well and had no history of fever or weight loss. His appetite was normal and there was no change in bowel habit. Clinical examination showed no abnormalities and he had no shortness of breath, chest pain, lower limb oedema, joint pains, or neurological symptoms. On examination, he had hepatomegaly (width of three fingers) and his cervical, axillary, and inguinal lymph nodes were enlarged, hard, and non-tender bilaterally. His blood count, renal function, and C reactive protein (<57 nmol/L; reference range 0-95) were normal. He had a raised erythrocyte sedimentation rate (102 mm in the first hour (28-32) and altered liver function tests (alkaline phosphatase 6.5 µkat/L (0.67-2.15), alanine aminotransferase 0.35 µkat/L (0.08-0.68), γ-glutamyl transferase 9.67 µkat/L (0.13-1.02), and total bilirubin 29.5 µmol/L (1.72-17.1). Proteinuria (0.25 g/L) was noted on urinalysis. Chest radiography showed mediastinal lymphadenopathy. Computed tomography of the thorax, abdomen, and pelvis showed enlarged mediastinal, axillary, and inguinal lymph nodes, which varied in size from 1 cm to 3 cm, with eggshell calcifications and an enlarged liver with hypodense texture. A lymph node biopsy was done and histological analysis performed (figure⇓). Fig 1 Section from a lymph node biopsy stained with Congo red and viewed under polarised light. Image courtesy of Ed Uthman, Houston, Texas, with permission1 ### 1. What are the causes of generalised lymphadenopathy? #### Short answer Generalised lymphadenopathy can be caused by infections (viruses, bacteria, fungi, or protozoans), cancer (including lymphoproliferative disorders), autoimmune disorders (including …