Cecilia Mercieca

Pulmonary, renal and neurological comorbidities in patients with ankylosing spondylitis; implications for clinical practice

Ankylosing spondylitis (AS) is associated with several comorbidities which contribute significantly to morbidity and mortality and add to the complexity of management. In addition to the well known extra-articular manifestations and increased cardiovascular risk, several pulmonary, renal, and neurological complications which have been associated with AS deserve equal attention. Whereas a clear link has been established for some manifestations, the evidence for other associations is less clear. Interstitial lung disease, apical fibrosis, secondary infection, and ventilatory restriction from reduced chest wall movement are well known pulmonary complications; more recently an association with sleep apnoea has been suggested. Renal amyloidosis and IgA nephropathy remain a treatment challenge which may respond to anti-TNF therapy. Atlanto axial subluxation and vertebral fractures can result in serious neurological complications and are notoriously difficult to diagnose unless a high level of suspicion is maintained. Despite several reports linking AS with demyelination a true link remains to be proved. This review discusses the prevalence, pathophysiology, and management of pulmonary, renal, and neurological complications, and implications for clinical practice.

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 …

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

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