Elena Elefante

Rate of serious infections in Behçet’s disease patients receiving biologic therapies: a prospective observational study

Behçet’s disease (BD) is a systemic, chronic relapsing vasculitis, typically characterized by recurrent oro-genital ulcers, ocular inflammation, and skin manifestations [1, 2]. The clinical profile of BD is extremely variable; while prevalent mucocutaneous involvement and arthritis represent the only clinical features in patients with a benign disease subset, there are other patients who develop potentially sightor lifethreatening manifestations, due to ocular, neurological, or major vascular involvement [3]. Literature shows growing data reporting effectiveness of tumor necrosis factor (TNF)alpha blockers in inducing remission for sightand lifethreatening involvement in BD [4–6]. Since TNF plays an important role in host defense and tumor growth control, antiTNF antibody therapies may increase the risk of serious infections.

Epidemiology and Management of Neuropsychiatric Disorders in Behçet’s Syndrome

Behçet’s syndrome (BS) is a systemic, chronic, relapsing vasculitis, typically characterized by recurrent orogenital ulcers, ocular inflammation and skin manifestations; articular, vascular, gastroenteric and neurological involvement may also occur. Besides the other clinical features of BS, it seems relatively frequent that patients with BS develop a neurobehavioural syndrome, characterized by euphoria, bipolar disorders and paranoid attitudes, loss of insight/disinhibition, and indifference to their disease, defined as ‘neuro-psycho-BS’. To date, the pathogenetic mechanism underlying neuro-psycho-BS has not been determined. It may be secondary to organic neurological involvement, or it may be related to poor quality of life and the relapsing course of the disease. Another engaging theory suggests that it could be related to the frequent observation of psychiatric symptoms during relapses or, in some cases, in the phases preceding reactivation of the disease; these elements suggest that psychiatric disorders in BS could represent a crucial element, whether a psychiatric subset or a distinct clinical feature of the disease. Moreover, it has been reported that cognitive impairment in BS can be seen with or without central nervous system involvement. Globally, psychiatric symptoms have been described as being multifaceted, ranging from anxiety disorders to depressive–bipolar disorders or to psychotic ones. In addition, some psychological characteristics of BS patients seem to predispose them to maladaptive stress management, which may lead to stress-related disorders, including anxiety and depression. Therefore, the aims of this review are to explore the epidemiology of neuro-psycho-BS by evaluating the relationship between the stress system and the multifaceted psychiatric manifestations in BS, and to summarize the therapeutic strategy used.

Systemic vasculitis and the lung.

Purpose of review The purpose of this review is to provide a critical analysis of the recent literature on this topic, with particular focus on the most relevant studies published over the last year. Recent findings Many studies are published every year on the diagnosis, pathogenesis and treatment of pulmonary involvement in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV). The main subjects covered by this article are the pathogenesis, diagnosis and clinical aspects of lung involvement in ANCA-associated vasculitis and non-ANCA-associated vasculitis. Summary Lung involvement is a common feature in systemic vasculitis. The lungs are one of the most frequently involved organs in systemic vasculitis. In order to provide an update on the recent advances in the pathogenesis, clinical features and novel treatments of lung involvement in systemic vasculitis, a systematic MedLine search has been performed. Most of the data analyzed have confirmed that lung involvement seems to develop more frequently in patients with myeloperoxidase-ANCA-positive AAV, mainly in those with a diagnosis of microscopic polyangiitis (MPA), compared with patients with proteinase 3 ANCA-positive AAV. Moreover, among non-ANCA-associated vasculitis lung involvement may represent a worrying complication of the disease, mainly when associated with vascular involvement.

FRI0326 Peripheral Lymphadenopathy in Primary Sjögren's Syndrome: Frequency, Pattern of Distribution and Clinical Significance - A Single Center Experience

Objectives To analyze frequency and clinical determinants of SLN-US prescription in clinical practice; to explore patterns of nodal size/location and their clinical significance in Sjögrens syndrome (pSS) Methods In this retrospective, single center study we included patients with a diagnosis of pSS, regularly followed at our University Hospital in the time frame 2010–2015. Demographic, clinical and serological data were recalled from clinical charts. Patients with lymphadenopathy secondary to infections, malignancy, NHLs were excluded from the study. Variables analyzed included: clinical determinants of SLN-US, number of US evaluation/time, frequency, size and nodal locations of the SLN (axillary, inguinal, cervical, supraclavicular, other). Results We enrolled in this study 229 (224 F: 5M) patients with pSS (AECG 2002), mean age at diagnosis (DS) 49 yrs (14) and mean follow-up 5.6 yrs (6.6). All the patients included in the study underwent a SLN-US at least once in the time frame 2010–2015. Peripheral lymphoadenopathy was documented in 136/229 (59.4%) patients. The most common site for peripheral lymphadenopathy was the cervical region with the most affected LN groups at this level being the lateral – cervical lymph groups (55.5%). Inguinal LN were involved in the 11.4% of the cases and axillary LN in the 10.9%. In 29 (12.7%) patients, pSS affected simultaneously the LN groups located in several segments. On average, pSS patients underwent an US of SLN every 18 (12) months (mean (SD)) with a negative correlation between the frequency of SLN-US and the duration of follow-up (r=-0.742 p=0.000). Patients underwent a SLN-US either to characterize peripheral LN, palpable at physical examination, or as a routine screening test during the assessment of the disease activity in asymptomatic patients. On the basis of the SLN-US results we distinguished different patient groups: a) 26 patients with palpable LN and pathological SLN-US, b) 110 patients without palpable LN and subcentimer lymphadenopathy at SLN-US and c) 93 patients without palpable LN and normal SLN-US. Patients included in group a) were significantly younger at the diagnosis (p=0.003), more frequently male (p=0.001), had a significantly higher disease activity (p=0.001) and presented more often salivary gland swelling (p=0.007), low C3 levels (p=0.008), hypergammaglobulinemia (p=0.006), anti-Ro/SSA (p=0.000), anti-La/SSB (0.02), Rheumatoid Factor positivity (p=0.003) and cryoglobulins (p=0.02). No significant differences were detected when patients included in the group b) and c) were compared one to each other. No specific association was observed between LN location and disease related clinical and serological manifestations. However, patients with pathological LN located in several segments presented a significantly higher disease activity and a more complex serological profile (p<0.01). Conclusions Peripheral lymphadenopathy is quite common in pSS patients. However, only patients with palpable lymphadenopathy at clinical examinations or LN located simultaneously in several segments presented a distinguished more severe phenotype. Subcentimer lymphadenopathy tends to remain stable over the time and does not seem to represent a poor prognosis factor in pSS patients. Disclosure of Interest None declared

AB0593 Disease Activity and Quality of Life in BehÇEt's Disease: the Role of Patients Reported Outcomes

Background Behçets disease (BD) is a systemic vasculitis, typically characterised by recurrent oro-genital ulcers, ocular inflammation and skin manifestations; articular, vascular, gastro-enteric and neurological involvement may also occur. Since BD has a chronic-relapsing course and it can be very severe, debilitating and potentially life-threatening, it may without any doubt affect the quality of life of the patients. Moreover, it is well known that patients perception of own disease represents an useful tool to help physicians to improve the understanding and management of the disease itself. Objectives The primary aim of this study was to explore the role of quality of life patients reported outcome (PRO) in better identifying the global status of BD. Methods The study enrolled 120 patients, all fulfilling the International Study Group (ISG) criteria for BD. The male/female ratio was 1.6:1, with a mean disease duration of 11±6 years. Their mean age was 42±8 years (min:18, max:77), while the mean age at disease onset was 24±5 years. The primary end-point was to study any potential correlation between quality of life and disease activity. Disease activity was evaluated by means of the Behçets Disease Current Activity Form (BDCAF), while the Italian version of the Short-form-36 (SF-36) was used to evaluate quality of life. Disease activity was compared with the global SF-36 score and with each dimension, that includes: physical functioning, physical disability, body pain, general health, vitality, social functioning, emotional disability, mental health. The statistical analysis was performed using Student t-test, Mann-Whitney-U test, ANOVA and Pearson correlation Results At time of evaluation, according BDCAF, 47 BD patients (39%) had clinically active disease (18 ocular involvement, 8 joint involvement, 4 neurological involvement, 2 gastro-enteric, 15 muco-cutaneous involvement). As expected, the overall SF-36 scores were significantly lower in patients with clinically active disease. Moreover, female BD patients had statistically significant lower scores in all SF-36 domains compared with male patients. When each domain of SF-36 was evaluated, we found that physical disability (p=0.004), body pain (p=0.006), general health (p=0.001), and vitality (p=0.001) were significantly lower in patients with disease activity. Notably, vitality (p=0.001), physical disability (p=0.004), social functioning (p=0.001), emotional disability (p=0.003) and mental health (p=0.001) were significantly lower in patients with muco-cutaneous active disease, compared with the other patients with active disease. Conclusions The clinicians who take care of any chronic disease would like to correctly know the current status of a patient to manage him properly. In this regard, the combination data of PRO measures and disease activity have been demonstrated to add more information compared to the evaluation of disease activity alone. These consideration suggest that the correct assessment of BD need a multi-dimensional approach, that fairly includes disease activity, disease damage and quality of life Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.5715

AB0925 Liver Involvement in Adult Onset Still's Disease: Retrospective Analysis of 18 Cases

Background Adult-onset Stills disease (AOSD) is a multisystem inflammatory disease of unknown etiology typically characterized by high fever, arthralgias/arthritis and transient cutaneous rash. Liver involvement has been reported in AOSD, however only few studies have described it comprehensively. Objectives To describe the frequency and clinical presentation of liver involvement in a single center cohort of AOSD. Methods Retrospective observational study including unselected patients with a diagnosis of AOSD made according to the Yamaguchi criteria and admitted at our University Hospital between 2009 and 2013. Demographic, clinical and serological data were retrieved from patients files including: fever, evanescent rash, sore throat, arthritis, myalgias, pleuritis, pericarditis, pneumonitis, lymphadenopathy, splenomegaly, hepatomegaly leucocytosis >15,000/μl and high serum ferritin levels. The severity of liver-associated enzymes was based on the degree of elevation and was stratified as mild (<2 times normal), moderate (2-5 times normal), and severe (>5 times normal). Severe liver dysfunction was defined as having all of the following criteria: total bilirubin >3 mg/dL; albumin <3.2 g/dL; and prothrombin time >3 seconds prolonged. Medications used, response to treatment and long-term outcomes were also recorded. Comparison in terms of continuous data were determined using independent sample t tests or Mann–Whitney tests, and in terms of proportions using contingency table analysis and chi square test. Results Eighteen patients (9 F: 9M; mean age (SD) =37 (12) years, mean follow-up=31 (15) months) were included in the study. Twelve patients out of 18 (66.6%) presented liver test abnormalities whereas hepatomegaly occurred in 4/18 (22.2%) cases. No correlation was found between liver involvement and gender or age at the diagnosis. A mild elevation of liver enzymes was found in 4/12 cases, a moderate alteration in other 4 patients, and a severe cytolysis in the other 4. A liver biopsy was performed in 3/12 patients revealing acute hepatitis with necrosis and accompanying non specific inflammatory infiltration. Three patients presented a liver acute failure concurrently with a diffuse intravascular coagulation (DIC) and, in one case, with a fatal macrophagic activation syndrome (MAS). Prednisone therapy induced a fast improvement of liver function in all the cases but one. Hydroxychloroquine, methotrexate and cyclosporine were the most common steroid sparing agents adopted during the follow-up. Conclusions Our findings outlined the high frequency of liver involvement in AOSD. Despite generally mild or moderate, severe acute hepatitis can occur during the disease course especially in those patients with DIC or MAS features. Treatment with systemic corticosteroid therapy is generally able to control liver function during AOSD, however steroid sparing agents are crucial in maintaining a long- term remission. Larger prospective studies could clarify the pathogenetic role of macrophagic activation in AOSD severe liver involvement, leading to develop new concepts for treatment modalities. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.4541