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Rheumatoid meningitis, a very rare complication, is not well-recognised, and there are few reports describing its treatment. We report the case of a 74-year-old Japanese woman who was diagnosed with rheumatoid meningitis by characteristic brain magnetic resonance imaging (MRI) and was successfully treated with glucocorticoids. We observed fluid-attenuated inversion recovery and diffusion-weighted imaging hyperintensity, which had a meningeal gadolinium-enhancing characteristic of rheumatoid meningitis. We suggest that it is possible to diagnose this disease based on characteristic MRI findings and treat patients early using glucocorticoids.

Objectives To describe the long-term clinical course of each manifestation of Behçet’s disease (BD) and clarify factors involved in oral ulcer (OU) remission using clinical information of BD patients. Methods We retrospectively studied 155 BD patients visiting our hospital (1989–2020). We defined remission criteria for each manifestation and examined long-term clinical changes. Classification and regression trees and multivariable analyses were performed to investigate OU prognostic factors; hazard ratios were used to assign scores to prognostic factors deemed significant [OU prognosis score (OuP score)]. Risk stratification was examined by dividing the OuP scores into four stages. Results OUs appeared earliest, with the slowest decline in prevalence observed post-BD diagnosis. OU presence was the most common factor inhibiting complete remission. Young age at OU onset, never smoker, presence of genital ulcers, positive pathergy test, no usage of tumour necrosis factor inhibitors or of immunosuppressants, and long-term non-treatment or symptomatic treatment for OUs were poor OU prognostic factors. Based on multivariable analysis, the area under the curve of the OuP score to predict OU prognosis was 0.678. Conclusions Remission criteria for each symptom clarified that OU had the greatest impact on complete BD remission. Faster OU remission was associated with earlier OU therapeutic intervention other than symptomatic treatment.

Background Pneumocystis jirovecii pneumonia (PCP) is an opportunistic infection in patients on steroid therapy for connective tissue diseases. The standard agent for primary PCP prophylaxis is trimethoprim/sulfamethoxazole (TMP-SMX), although this agent can cause common adverse reactions, including myelosuppression and renal toxicity, that result in cessation. Aerosolized pentamidine and oral atovaquone are alternatives for PCP prophylaxis. The efficacies of atovaquone, pentamidine, and TMP-SMX to prevent PCP in patients with connective tissue diseases have never been compared. Methods Hospitalized patients with connective tissue diseases who started steroid therapy and PCP prophylaxis were enrolled. PCP prophylaxis regimens were oral TMP-SMX, aerosolized pentamidine, or oral atovaquone. Information was retrospectively collected from medical records about laboratory findings, duration of PCP prophylaxis, and reasons for terminating PCP prophylaxis. Results Ninety-six patients received PCP prophylaxis. All of them were initially treated with TMP-SMX, but this was replaced during the study period with pentamidine in 33 patients and with atovaquone in 7. Forty-one (43%) patients discontinued TMP-SMX because of adverse events, and 5 (15%) also discontinued pentamidine. None of the patients discontinued atovaquone. The most frequent causes of TMP-SMX and pentamidine cessation were cytopenia (N = 15) and asthma (N = 2). The rates of continuing treatment with TMP-SMX, pentamidine, and atovaquone at one year after starting PCP prophylaxis were 55.3%, 68.6%, and 100%, respectively (P = 0.01). None of the patients developed PCP. Conclusion Although TMP-SMX for PCP prophylaxis had to be discontinued in 43% of patients with connective tissue diseases, pentamidine and atovaquone were well tolerated.

Both juvenile temporal arteritis (JTA) and Kimura's disease are eosinophilic inflammatory conditions but exhibit different clinical manifestations. Here, we describe a case involving a 40-year-old man who developed JTA secondary to Kimura's disease. Approximately 3 years before admission, masses appeared on both posterior auricles. A biopsy of the right posterior auricle mass led to a diagnosis of Kimura's disease. Approximately 4 months before admission, both masses increased in size, and almost simultaneously, the left temporal artery became distended. Histopathology of a biopsy of the left temporal artery revealed inflammatory findings with marked eosinophil infiltration and significant intimal hyperplasia with stenosis of the vascular lumen, indicating JTA. An analysis of the 48 reported cases of JTA, identified in a literature review, and the present case, revealed that Kimura's disease was detected in 6 cases, all of which involved Asians. In conclusion, this case and the literature review suggest that JTA can be accompanied by another eosinophilic inflammation-based disorder, Kimura's disease, particularly in Asians. This newly highlighted relationship between JTA and Kimura's disease could lead to a better understanding of JTA, which is an extremely rare disease.

Background Non-tuberculous mycobacterial (NTM) infection is currently a growing health concern due to the increasing incidence and the need for prolonged therapy. In patients with connective tissue diseases, use of immunosuppressants may lead to an increased risk of NTM infection. However, few studies have examined the recent incidence of NTM infection among connective tissue diseases patients. This study investigated recent trends in NTM infection among connective tissue diseases patients. Methods We included adult patients from whose cultures NTM were isolated between January 2009 and October 2017 in our hospital. By reviewing their medical records, connective tissue diseases patients were identified. Types of connective tissue disease, NTM species, and treatment of NTM infection were extracted. Results NTM was isolated from 657 patients during the period. Among these, 24 patients had connective tissue diseases. The number and rate of NTM isolates from connective tissue diseases patients increased during the period, with 4 patients 2009 to 2012 (1.9%), and 20 patients from 2013 to 2017 (3.3%; P = 0.04). The proportion of Mycobacterium avium complex (MAC) to total NTM tended to be lower among connective tissue diseases patients (58.3%) than among non-connective tissue disease-patients (72.8%), but the difference was not significant (P = 0.20). Mycobacterium xenopi was significantly more frequent in connective tissue disease patients than in non-connective tissue diseases patients (P Conclusion The recent increase in the incidence of NTM infections in connective tissue diseases patients was larger than that in the total population. NTM species other than MAC were isolated from connective tissue diseases patients.

A 67-year-old woman presented with hematuria and proteinuria 16 and 11 months ago, respectively. She had been followed up as mixed connective tissue disease and Sjögren's syndrome for over 19 years. Blood chemistry showed no elevated serum creatinine or C-reactive protein but did reveal myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) of 300 U/dL. A kidney biopsy showed pauci-immune focal necrotizing glomerulonephritis. She was treated with prednisolone and rituximab, resulting in normal urinalysis and decreased MPO-ANCA. The complication of ANCA-associated glomerulonephritis should not be overlooked when abnormal urinalysis findings appear in the course of connective tissue disease, irrespective of the presence of rapidly progressive glomerulonephritis.

To identify the specific characteristics of patients with refractory intestinal Behçet's disease (BD) who required more than glucocorticoid (GC) and/or 5-aminosalicylic acid (5-ASA) treatment.A retrospective review of the patient records in a university hospital identified 34 patients with intestinal BD. The patients treated only with glucocorticoid and/or 5-ASA (n = 8) were compared with refractory cases which required additional immunosuppressants, anti-TNFα antibodies, or surgery (n = 12).In the refractory group, ulcers were found outside the ileocecal region more often, and more active intestinal bleeding or melena was observed, than in the GC/5ASA-controlled group (75% vs 0%, p = 0.001), (58% vs 0%, p = 0.015). The refractory group also showed higher positivity for HLA-B51 (45% vs 0%, p = 0.044), higher blood C-reactive protein (CRP) levels (14.7 ± 8.74 vs 3.93 ± 6.33 mg/dL, p = 0.046), and a higher white blood cell or WBC count (14750 ± 6760 vs 7210 ± 1830/μl, p = 0.025) at onset. The existence of either HLA-B51, melena, or elevated CRP of more than 4 mg/dL predicted the refractory form of BD with 100% sensitivity and 85.7% specificity.Refractory intestinal BD was distinguished from the non-refractory form by distinct clinical and laboratory findings. These findings will be useful in identifying patients who require intensive therapy (e.g., anti-TNFα antibodies) in addition to GC/5ASA.

Background Central nervous system involvement is one of the most serious complications in Behcet9s disease (BD). This condition is referred to as neuro-Behcet9s disease (NB) and can be classified into acute type (ANB) and chronic progressive type (CPNB) based upon differences in the clinical course and responses to corticosteroid treatment. Cerebellar ataxia, such as gait disturbances and dysarthria, is one of the representative manifestations in CPNB. Accordingly, brainstem atrophy is frequently observed in CPNB, but not in ANB. Notably, progressive neurobehavioral changes mimicking those in Alzheimer9s disease (AD) are also frequently observed in CPNB, but these changes cannot be accounted for by brainstem atrophy. Objectives In the present study, volumes of the hippocampus were examined to identify the responsible lesions for neurobehavioral changes in CPNB, and patterns of atrophy in the hippocampus and brainstem were compared between patients with CPNB and AD. Methods The subjects were 32 patients, including 13 with CPNB (11 males and 2 females, age 51.2±12.1 years old [mean ± SD]), 13 with Behcet9s disease without NB (non-NB) (10 males and 3 females, age 54.4±11.4 years old), and 6 with AD (5 males and 1 female, age 78.8±7.5 years old). All patients with BD satisfied the international classification criteria for BD. CPNB was defined as intractable, slowly progressive neurobehavioral changes and/or cerebellar ataxia accompanied by persistent elevation of interleukin-6 of >20 pg/mL in cerebrospinal fluid on two different occasions at an interval of at least 2 weeks. All patients with AD satisfied Diagnostic and Statistical Manual of Mental Disorders (DSM)-IV criteria. Brain magnetic resonance imaging (MRI) was obtained from each subject. The areas of the midbrain tegmentum and pons were measured on mid-sagittal sections of T1-weighted images using image analysis software (Image J ver.1.45: NIH, USA). Severity of gray matter loss in the hippocampal region and whole brain were investigated using Voxel-Based Specific Regional Analysis System for Alzheimer9s Disease (VSRAD) software (Eisai Co., Ltd) to determine the degrees of hippocampal region atrophy (Z score) and whole-brain atrophy (WBAI). Thus, the 1/Z score is positively correlated with the hippocampus volume. The ratio of the degree of brainstem atrophy to that of hippocampal atrophy was evaluated by the brainstem area value divided by the 1/Z score (BA/H score) in each patient. Results The brainstem area was significantly decreased in CPNB (461.8±87.3 [mean ± SD]) compared with that in AD (661.9±56.1) and non-NB (666.1±50.6) (Figure A). VSRAD analysis showed that Z score was significantly increased in CPNB (1.46±0.70) and AD (3.13±1.21) compared with that in non-NB (0.77±0.40) (Figure B). Of note, the BA/H score, reflecting the brainstem/hippocampus volume ratio, was much lower in CPNB than in AD [663.5±311.8 vs 2018±667.6, p=0.001] (Figure C). Conclusions These results indicate that the hippocampus, in addition to the brainstem, is a common target lesion in CPNB, and this accounts for the progressive neurobehavioral dysfunction in this disease. Moreover, the data emphasize that brainstem atrophy is disproportionately greater than hippocampal atrophy in CPNB, in contrast to AD. Disclosure of Interest None declared

Optic perineuritis is an uncommon inflammatory disorder of the optic sheath that causes visual loss or eye pain. There are few case reports of optic perineuritis associated with granulomatosis with polyangiitis. Herein we report the case of a 37-year-old male with granulomatosis with polyangiitis and who presented with headache, blurred vision in the right eye, diplopia, and numbness in the right forehead. Brain magnetic resonance images (MRI) findings revealed hypertrophic pachymeningitis and refractory optic perineuritis. These were manageable only by means of weekly methotrexate and mycophenolate mofetil combination therapy but not with methotrexate, mycophenolate mofetil, intravenous cyclophosphamide, rituximab, azathioprine, or cyclosporine individually.

A 78-year-old man presented with bilateral auricular and nasal chondritis and an inner ear disorder. Relapsing polychondritis (RPC) was diagnosed and corticosteroid therapy was initiated. Two years later, he developed abdominal pain and a fever. A contrast-enhanced computed tomography scan showed enhancement of the mesentery and massive ascites. The patient underwent emergency laparotomy, which revealed inflammation and thickening of the omentum. A microscopic examination of the omentum disclosed vasculitis, and corticosteroid and cyclophosphamide pulse therapies were administered. We herein report the first case of RPC complicated by pathologically proven vasculitis of the omentum, clearly indicating an association between the pathogenesis of these two conditions.

A 31-year-old woman who had developed systemic lupus erythematosus at 17 years of age was admitted to the hospital for suspected cellulitis in the lower extremities. A blood culture performed upon admission to the hospital detected Helicobacter cinaedi (H. cinaedi), which was also isolated in blood and fecal cultures obtained on the 42nd hospital day. Bacterial translocation of H. cinaedi present in the intestines may have led to the development of recurrent bacteremia and cellulitis. In cases such as this, appropriate antibiotics therapy might be needed for more than one month. Moreover, H. cinaedi, a cause of emerging infections, requires a long period of time to grow; therefore it is important to extend the culture duration when the presence of this bacterium is suspected.

A 68-year-old man was admitted with rapid visual loss. Churg–Strauss syndrome (CSS) was diagnosed, based upon the symptoms of asthma, eosinophilia, interstitial pneumonitis, and positive myeloperoxidase-anti neutrophil cytoplasmic antibody (MPO-ANCA). Light reflexes were absent and vision was completely lost in both eyes. Bilateral central retinal artery occlusion (CRAO) was observed by fluorescence angiography. Steroid pulse along with an anticoagulant improved the visual acuity to light perception and hand motion. CSS-associated CRAO should be considered when acute visual loss occurs.

A 41-year-old woman presented with continuous fever, and her laboratory data suggested the recrudescence of systemic lupus erythematosus. She was treated with 60 mg/day prednisolone. With a dose reduction of prednisolone, high fever and pancytopenia were observed again. A bone marrow biopsy revealed hemophagocytosis. The effects of steroid pulse therapy, high-dose intravenous immunoglobulin, cyclosporine A, and methotrexate were insufficient. However, after four injections of etanercept (25 mg, twice a week) subcutaneously, her symptoms had completely resolved. In such cases, therapy with etanercept may be effective.

A 31-year-old woman who had developed systemic lupus erythematosus at 17 years of age was admitted to the hospital for suspected cellulitis in the lower extremities. A blood culture performed upon admission to the hospital detected Helicobacter cinaedi (H. cinaedi), which was also isolated in blood and fecal cultures obtained on the 42nd hospital day. Bacterial translocation of H. cinaedi present in the intestines may have led to the development of recurrent bacteremia and cellulitis. In cases such as this, appropriate antibiotics therapy might be needed for more than one month. Moreover, H. cinaedi, a cause of emerging infections, requires a long period of time to grow; therefore it is important to extend the culture duration when the presence of this bacterium is suspected.

A 62-year-old woman presented to a primary care doctor on January 2006 due to a sore throat and high fever, and had received medication for a common cold. She was referred to our hospital in February 2006 because of additional manifestations such as painful rashes on the lower limb similar to erythema nodosum and polyarthralgia on her feet, shoulder and finger joints. She was initially treated with an anti-inflammatory drug (NSAID) for polyarthritis but the symptoms did not improved. In addition, the serum level of anti-streptolysin O antibody (ASO) was elevated at the second visit more than that at the first visit. She was diagnosed to have rheumatic fever (RF) based on the polyarthritis, inflammatory data and an increase of the ASO level. She was treated with 10 mg a day of prednisolone (PSL) and sultamicillin tosilate. However, a systolic murmur that had been never noticed by previous auscultation appeared after the third hospital day and the mitral regurgitation was also detected on echocardiogram. She was then treated with 40 mg a day of PSL because of an appearance of the carditis due to RF. The increased PSL dose promptly improved the systolic murmur as well as the arthritis. This report presented an RF case with carditis detected by an development of the systolic murmur in an adult female.

We examined the capacities of bone marrow (BM) plasmacytoid dendritic cells (pDC) to differentiate into type B synoviocyte-like cells. BM aspiration samples were obtained from 24 rheumatoid arthritis (RA) patients and 19 osteoarthritis (OA) patients during joint operations from the iliac crest. CD34+ cells and pDC purified from BM mononuclear cells were cultured with or without SCF, GM-CSF, and TNF-α for 2–4 weeks. RA BM pDC as well as OA BM pDC comparably differentiated into fibroblast-like cells (FLC), expressing cadherin-11 and producing MMP-1, especially in the presence of TNF-α. Of note, depletion of BDCA4+ pDC from RA BM CD34+ cells significantly diminished their capacities to differentiate into FLC, which were restored by addition of BDCA4 + cells in a dose–response manner. These results indicate that pDC is one of the progenitors of type B synoviocytes, suggesting that BM pDC might be involved in the pathogenesis of RA and OA.

Systemic immune tolerance is induced for orally administered antigen, and this phenomenon is called oral tolerance. However, the mechanism of oral tolerance has not been completely elucidated. It has been suggested that antigen presentation and generation of regulatory T cells in Peyer's patches (PPs) are important for induction of oral tolerance. Hence, we orally administered fluorescence-labelled antigen to mice and examined kinetics of the antigen and interaction between antigen-loaded dendritic cells and T cells. It was visualized that dendritic cells in PP rapidly take up antigen. We next transferred antigen-specific naive T cells from T cell receptor transgenic mice and administered the antigen orally. Antigen-specific T cells accumulated in IFR in PP and DCs that have ingested antigen come in contact with antigen-specific T cells in IFR. The accumulated T cells were then collected and analyzed for the pattern of gene expression by real-time PCR, which revealed a gene expression pattern similar to that of FoxP3-positive regulatory T (T reg ) cells. CCR9, an intestinal homing marker, was also strongly expressed. These results suggest that DCs that have captured oral antigens in PPs locally induce antigen-specific naive T cells to differentiate into T reg cells with the intestinal homing phenotype.

Background Central nervous system (CNS) involvement is a serious complication of Behcet9s disease and is referred to as neuro-Behcet9s disease (NB). NB is classified into acute type (ANB) and chronic progressive type (CPNB), depending on the clinical characteristics. CPNB is characterized by intractable, slowly progressive neurobehavioral changes, ataxia and dysarthria, which lead to severe disability and deterioration (1). Recently, we have found that brainstem atrophy is significantly more prevalent in CPNB than in ANB or non-NB, indicating that brainstem atrophy is a frequent and specific finding in CPNB (2). Objectives The goal of the study was to examine whether the integrated cerebrospinal fluid (CSF) interleukin-6 level in the first 6 months after diagnosis is correlated with brainstem atrophy by quantitative analysis of magnetic resonance imaging (MRI) scans. Methods The subjects were 10 patients (9 males and 1 female; all HLA-B51 positive; age at first MRI: 35.1±11.6 years old [mean ± SD]) who had been diagnosed with CPNB. The integrated CSF IL-6 level was calculated from the area under the curve (AUC) in a kinetic analysis of CSF IL-6 from the time of diagnosis until 6 months after diagnosis. The areas of midbrain tegmentum and pons were measured on mid-sagittal sections of T1-weighted images using NIH Image J ver.1.45 software (ver.1.46, National Institutes of Health: NIH, U.S. [http://rsbweb.nih.gov/ij/index.html]). Results The kinetics analysis showed that brainstem atrophy progressed most markedly in the first 2 years after diagnosis of CPNB. As shown in the Figure, integrated CSF IL-6 (0-0.5 years) was significantly correlated with brainstem atrophy rate in the first 2 years (0-2 years). Conclusions These results indicate that higher levels of CSF IL-6 in the first 6 months after diagnosis are strongly associated with greater progression of brainstem atrophy in CPNB. This suggests that an appropriate therapeutic intervention to reduce CSF IL-6 is required as early as possible upon diagnosis of CPNB. References Hirohata S. et al., Behcet9s disease. Arthritis Res Ther 2003; 5:139-46. Hirohata S. et al., Clinical characteristics of neuro-Behcet9s disease in Japan: a multicenter retrospective analysis. Mod Rheumatol 2012; 22: 405-13. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.1610

Background Behcet’s disease (BD) is a chronic multisystemic vasculitis of unknown etiology with recurrent oral aphthous ulcers, genital ulcers, skin and ocular lesions. Serious complications may also occur in the intestinal tract, central nervous system (CNS) and vascular lesions, including ruptured aneurysms that can be a cause of death. The European League Against Rheumatism (EULAR) recommends immunosuppressive drugs for treatment of VBD to control vasculitis, but not use of antithrombotic drugs. The incidence of complication with vascular lesions is about 10% of BD in Japan, which is lower than that in the Middle East, Europe and the United States; however, antithrombotic drugs are frequently used in addition to immunosuppressive drugs because complication with deep venous thrombosis (DVT) is often found. Objectives Clinical manifestations of vascular Behcet’s disease (VBD) complicated with pulmonary thromboembolism (PTE) and treatment before and after onset were investigated, with the goal of establishment of strategies for prevention of development and recurrence of PTE. Methods A cohort study was conducted in 43 patients with VBD who were treated in Teikyo University Hospital from 1989 to 2012. All patients fulfilling the International Study Group Criteria were evaluated. Clinical features and treatment were compared between patients with a complication of PTE and those without PTE. Results The patients with PTE consisted of 5 males and 2 females, and had average ages of 35.0 ± 15.3 years old at the onset of BD and 38.4 ± 13.6 years old at the onset of VBD. The incidences of recurrent oral aphthous ulcers, genital ulcers and skin lesions were all 100% and those of eye involvement and epididymitis were 42.9% and 40%, respectively. The incidences of complications of intestinal, CNS and arterial lesions were all 14%. The HLA-B51 positive rates were 20% and the smoking rate was 57.1%. Three patients were treated with warfarin as anticoagulant therapy to prevent recurrence after onset of PTE. No hemorrhagic adverse reactions occurred, but the therapy was ineffective in one patient who died from recurrence (recurrence rate (RR): 33.3%). Three of the 4 patients who were not treated with warfarin had recurrence (RR: 75%) and one of these patients died due to development of a ruptured aneurysm while under immunosuppressive therapy with corticosteroids. Clinical features and treatment were compared between patients with (n=7) and without PTE (n=36). There were no significant differences in the incidence of symptoms, particular disease types, and complications. Administration of colchicine was significantly lower in the patients with PTE prior to onset of the incident. Administration of antithrombotic drugs was also significantly lower in these patients, although there was no significant difference in administration of warfarin or aspirin alone. Conclusions Warfarin might be beneficial for secondary prevention of PTE and VBD with DVT who were not treated with warfarin had a life-threatening risk of development of this complication. PTE is a fatal complication of VBD, similarly to an arterial lesion, and further accumulation of cases and analysis in prospective studies are required to establish effective antithrombotic therapy. References Hatemi G, et al. EULAR recommendations for the management of Behcet disease. Ann Rheum Dis. 2008;67:1656–62. Disclosure of Interest None Declared

Background Atherosclerosis is a chronic metabolic disease of inflammatory processes. Immune cells including monocytes are recruited to the subintimal lesion of the vascular wall and store lipids to transform themselves to the foam cells. The lipid profiles in the serum are shown to be risk factors for developing atherosclerosis, such as high LDL (low density lipoprotein) or low HDL (high density lipoprotein) concentration. In addition to deliver cholesterol from vascular wall to liver, HDL is supposed to exert antiinflammatory properties which are not well characterized. We recently indentified that HDL exert an antiinflammatory property which is not ascribed to it’s capacity to lower the cholesterol level of the peripheral tissue. Objectives To investigate the antiinflammatory function of reconstituted HDL (rHDL) in the Kawasaki arteritis murine model in vivo. Methods Coronary arteritis mimicking Kawasaki disease was induced by administrating 20 microgram of lipopolysaccharide followed by weekly injection of 500 microgram of the nucleotide-binding oligomerization domain 1 (NOD1) ligand FK-565 for 4 times. 2 mg of rHDL or control PBS was injected 4 times along with FK-565 administration. After a week after the last injection of FK-565 or control PBS, the severity of coronary arteritis was quantified by measuring the inflammation area surrounding the coronary arteries on the microscopic images. The cytokine levels of the serum were determined by multiplex. Results The rHDL treatment reduced the inflammatory area of coronary arteries compared with the control PBS treated groups, showing statistically significance (P=0.008 with Student t-test, 0.29±0.24 mm 2 (PBS control group) and 0.13±0.07 mm 2 (rHDL group)). Serum level of IL-12p40 was decreased in mice treated with rHDL. Conclusions The data indicate that rHDL exert an antiinflammatory activity in the NOD1 ligand induced Kawasaki arteritis model in vivo. References Nishio H et al. Arterioscler Thromb Vasc Biol . 2011, 31: 1093-9 Disclosure of Interest None Declared

Background Atherosclerosis is a chronic metabolic disease of inflammatory processes. Immune cells including monocytes are recruited to the subintimal lesion of the vascular wall and store lipids to transform themselves to the foam cells. The cholesterol crystal formed inside of the foam cells stimulates NLRP3 inflammaosme to secrete IL-1 and further inflammation. The lipid profiles in the serum are shown to be risk factors for developing atherosclerosis, such as high LDL (low density lipoprotein) or low HDL (high density lipoprotein) concentration. In addition to deliver cholesterol from vascular wall to liver, HDL is supposed to exert antiinflammatory properties which are not well characterized. Objectives To investigate the antiinflammatory function of reconstituted HDL (rHDL) in the Kawasaki arteritis murine model in vivo. Methods Coronary arteritis mimicking Kawasaki disease was induced by administrating 20 microgram of lipopolysaccharide followed by weekly injection of 500 microgram of the nucleotide-binding oligomerization domain 1 (NOD1) ligand FK-565 for 4 times. 2 mg of rHDL or control PBS was injected 4 times along with FK-565 administration. After a week of the last injection of FK-565 or control PBS, the severity of coronary arteritis was quantified by measuring the inflammation area surrounding the coronary arteries. Results The rHDL treatment reduced the inflammatory area of coronary arteries compared with the control PBS treated groups, showing statistically significance (P t- test, 0.31±0.29 mm 2 (PBS control group) and 0.14±0.08 mm 2 (rHDL group)). Conclusions The data indicate that rHDL exerts an antiinflammatory activity in the NOD1 ligand induced Kawasaki arteritis model in vivo. References Nishio H et al. Arterioscler Thromb Vasc Biol. 2011, 31: 1093-9 Duewell P, Kono H, et al. Nature 2010, 464: 1357-61. Disclosure of Interest None Declared

Background Central nervous system involvement in Behcet’s disease (neuro- Behcet’s disease [NB]) includes acute type and chronic progressive type, which may ultimately lead to the deterioration of the patients’ personality (1). Previous studies showed that infliximab (IFX) prevented the progression of chronic progressive NB along with marked reduction of cerebrospinal fluid (CSF) IL-6 levels in a 14-week open trial (2). However, the long-term efficacy and safety of IFX therapy for chronic progressive NB remains unclear. Objectives We performed a further follow-up survey of the 5 patients who had completed the 14-week trial of IFX therapy, for as long as 6 years. Methods Five patients (all males who were HLA-B51 positive; mean age at enrollment: 35.8±7.2 years [±SD]) with any of psychosis, dementia, urinary incontinence, or cerebellar ataxia who had been inadequately responded to methotrexate (MTX) and enrolled in the previous 14-week open trial of IFX therapy, were followed-up with regard to the clinical responses, brain magnetic resonance imaging (MRI) scans, CSF IL-6 levels and adverse events for up to 6 years. Results In 1 of the 5 patients, IFX was withdrawn without any increase in the dose of MTX after the 14-week trial, in whom CSF IL-6 levels remained below 20 pg/ml throughout the course without exacerbation of the manifestation and brainstem atrophy on MRI scans. In another patient complicated by refractory posterior uveitis, IFX was continued every 8 weeks with CSF IL-6 levels below 20 pg/ml throughout the course without exacerbation of the neurological manifestations. In the other 3 patients, IFX was stopped after 4-9 additional infusions following the 14-week trial. However, after 3-6 months IFX was resumed along with increasing the dose of MTX in all the 3 patients due to marked elevation of CSF IL-6 levels became elevated. IFX could be discontinued after 9 infusions in 1 of the 3 patients, showing no exacerbation of the neurological manifestation nor elevation of CSF IL-6 over 20 pg/ml until now, whereas IFX was continued every 6 weeks in the remaining 2 of the 3 patients, in whom CSF IL-6 levels were not decreased below 20 pg/ml with significant progression of the neurological manifestations and brainstem atrophy on MRI scans. Mild adverse effects occurred in 2 patients during the follow-up study, including transient headache and subclinical pneumocystis pneumonia, which was resolved by using trimethoprim-sulfamethoxazole. Conclusions These results indicate that IFX has a beneficial effect in the treatment of chronic progressive NB by reducing CSF IL-6 levels. However, there is a subset of patients who do not respond adequately to IFX. Further study is needed in order to establish an effective treatment regimen for such intractable chronic progressive NB. References Hirohata S. et al., Behcet’s disease. Arthritis Res Ther 2003, 5:139-146 Kikuchi H. et al., Effect of infliximab in progressive neuro-Behcet’s syndrome. J Neurol Sci 2008, 272:99-105 Disclosure of Interest None Declared