Background Giant cell arteritis (GCA) is often a disease refractory to corticosteroids and, besides, the efficacy of immunosuppressive agents is not well established. In recent years, several case reports and small case series have shown efficacy with the use of tocilizumab (TCZ). Objectives Our aim was to assess in a clinical practise setting the short and long-term efficacy of TCZ in GCA patients with refractory disease and/or with unacceptable side effects due to corticosteroids. Methods Retrospective multicenter open-label study on 31 GCA patients treated with TCZ [intravenously at standard dose of 8 mg/kg/monthly (n=29), and subcutaneously at a dose of 162 mg/week (n=2)]. We assessed the efficacy on clinical and laboratory parameters, the reduction of the dose of corticosteroids, as well as the short and long-term side effects and the possibility of discontinuation or reduction of the dose of TCZ. Wilcoxon test was used to compare laboratory parameters across time. Results We included 31 patients (24 women/ 7 men), with a mean age of 73±9 years. The main clinical features at TCZ onset were: polymyalgia rheumatica (n=21), asthenia (n=8), headache (n=8), constitutional syndrome (n=11), jaw claudication (n=3), and visual loss (n=4). Besides corticosteroids and before TCZ onset, 26 patients had also received several conventional immunosuppressive and/or biologic drugs. Twenty-seven of 31 patients achieved a rapid and maintained clinical improvement after TCZ therapy (Table). After a median follow-up of 18 [interquartile range, 6–30] months we observe a reduction of the median of: a) CRP from 1.9 [1.1–3.7] to 0.1 [0.1–0.7] mg/dL; b) ESR from 44 [17–74] to 12 [4–16] mm/1st hour; and c) the dose of prednisone from 20 [10–45] to 2.5 [0–7.5] mg/day. In this follow-up period, the outcome of patients was as follows: a) discontinuation of TCZ (n=8) due to sustained remission; b) dose reduction due to improvement (n=5) or side effects (n=2); c) withdrawal of TCZ because of side effects (n=7); and d) the same dose that at onset (n=8). The reasons why TCZ had to be discontinued were: severe neutropenia; recurrent pneumonia; colon adenocarcinoma; cytomegalovirus infection; hypertensive crisis during infusion; myelodysplastic syndrome; and overall health deterioration. The latter patient died because of stroke. Another patient also died after the second TCZ infusion due to stroke in the context of an infective endocarditis. Conclusions TCZ therapy leads to a rapid and maintained improvement in patients with refractory GCA and/or with unacceptable side effects related to corticosteroids. However, the risk of neutropenia and infection should be kept in mind when using this biologic agent in patients with GCA. Disclosure of Interest None declared

Background In patients with rheumatoid arthritis (RA), we previously described that adherence to the subcutaneous (SC) biological treatment is better with monthly administration. Objectives We further assessed if there are differences in patients expectations and satisfaction with efficacy and tolerance that could contribute to explain such finding. Methods ARCO was a retrospective study on RA patients who had been prescribed a SC biological 11–18 months prior to the study. Adherence was calculated with the medication possession ratio (MPR). Satisfaction and expectations were assessed with the Spanish validated Carbonell questionnaire [1]. Results We included 364 patients (age 54.9 years [SD 12.5], 77.5% women, median duration of RA 7.8 years, period studied for the SC biological 14.8 months). Non-adherence (MPR ≤80%) was lower in patients with monthly (6.4%) than with weekly (17.4%, p=0.034) or every 2 weeks administration (14.4%, p=0.102). The % of satisfied patients (quite/very satisfied) was 86.2% for efficacy and 64.4% for side effects or tolerance. Non-adherence was similar in satisfied with efficacy and in neutral/unsatisfied patients (14.7% vs. 8.3%, p=0.399), or in patients satisfied/not satisfied with side effects (13.1% vs. 15.4%, p=0.504). The fulfillment of expectations is shown in the table. With regard to expectations on the effect, non-adherence was 15.5% (higher than expected), 12.6% (as expected) and 10.7% (lower than expected) (p=0.677), and with regard to discomfort/side effects, it was 15.6% (greater than expected), 18.5% (as expected) and 11.1% (lower than expected, or no side effect) (p=0.189). Fulfilment of expectation on efficacy was similar for the 3 dosing schemes, but the % reporting lower than expected discomfort or no discomfort was greater with fewer SC injections (table). In particular, the % reporting no discomfort/side effects with the administration were 17.8% (weekly), 29.3% (every 2 weeks), and 35.0% (monthly) (p=0.013). Conclusions RA patients on SC biological therapy show high satisfaction and fulfillment of expectations on efficacy, although both aspects lower for tolerance. Dosing regimen with lower number of SC injections seems to be associated with better fulfillment of expectations of tolerance. This finding, added to the lower number of injections itself, might explain the better adherence observed with monthly administration. References Carbonell J, Badia X; Grupo Expresar. Development and validation of a satisfaction questionnaire in patients with rheumatoid arthritis. Reumatol Clin 2006;2:137–45. Acknowledgements Funded by MSD, Spain. Disclosure of Interest None declared

Background Non-infectious aortitis is often refractory to standard immunosuppressive therapy. In these cases, the use of inhibitors of TNF-α (iTNF-α) had been reported. Objectives Our aim was to assess the efficacy of iTNF-α in a series of patients with refractory non-infectious aortitis. Methods Retrospective multicenter study of patients diagnosed with aortitis refractory to traditional immunosuppressive agents and who received iTNFα. The diagnosis of aortitis was based on imaging techniques (MRI-angiography, computed tomography, PET scan, ultrasonography and/or arteriography). Results We studied 19 patients (15 women/4 men) with a mean age of 42±13 years. The iTNFα used were: infliximab (IFX) (n=14), adalimumab (ADA) (n=3) and etanercept (ETN) (n=2). The underlying conditions were: Takayasu arteritis (TA) (n=11), giant cell arteritis (GCA) (n=2), relapsing polychondritis (RP) (n=1), ulcerative colitis (n=1), Crohn9s disease (n=1), Behçet9s disease (n=1), sarcoidosis (n=1) and psoriatic arthritis (n=1). Seventeen patients were previously treated with traditional immunosuppressive agents [methotrexate (MTX) (n=15), azathioprine (AZA) (n=7), cyclophosphamide (CPM) (n=5), mycophenolate mofetil (MM) (n=3), chlorambucil (n=1), cyclosporine A (n=1), tacrolimus (n=1)]. Two patients needed a switching between biologic agents (a female with TA who switched from IFX to ETN; and a male with psoriatic arthritis who switched from ETN to ADA; in both cases switching was due to inefficacy of the first biologic drug). After a median follow-up of 16 [12–36] months, most patients experienced clinical improvement, showing a reduction of erythrocyte sedimentation rate from 37.5 [30–56] mm/1st hour at baseline, to 20.5 [10–24] mm/1st hour at the last visit. Besides, C-reactive protein decreased from 1.5 [0.1- 2.6] mg/dL to 0.3 [0.1–0.8] mg/dL. After 3 months of treatment, 55% of patients had experienced clinical improvement (p<0.01); and at 12 months, clinical improvement was attained by 94% of the patients (p<0.01). A corticosteroid sparing effect was also achieved (from 25.7±22.0 mg/day of prednisone at iTNFα onset, to 6.6±5.4 mg/day at the last visit). Improvement on imaging techniques was also observed in 10 out of 13 patients (77%) who underwent a follow-up imaging procedure. Three patients had to discontinue the iTNFα agent (IFX in the 3 cases) due to inefficacy (n=1), recurrent pneumonia (n=1) and severe infusional reaction (n=1). Conclusions iTNFα therapy appears effective and relatively safe in patients with non-infectious aortitis refractory to traditional immunosuppressive drugs. Acknowledgement This study was supported by a grant from “Fondo de Investigaciones Sanitarias” PI12/00193 (Spain). This work was also partially supported by RETICS Programs, RD08/0075 (RIER) and RD12/0009/0013 from “Instituto de Salud Carlos III” (ISCIII) (Spain). Disclosure of Interest None declared

Background Non-infectious aortitis is often an underrecognised condition. Early diagnosis and treatment is crucial to prevent serious complications. Corticosteroids represent the first-line therapy of non-infectious aortitis. However, disease-modifying antirheumatic drugs (DMARDS) and/or biologic therapy are usually required. Objectives Our aim was to assess the treatment and outcome of patients diagnosed with non-infectious aortitis from a single centre. Methods Retrospective study of 32 patients (22 women/10 men) diagnosed with non-infectious aortitis based on imaging techniques. We have considered 3 groups: group a) patients who only received corticosteroids; group b) patients treated with corticosteroids and DMARDs; and group c) those who required biologic therapy. Results were expressed as mean±standard deviation (SD) or as median and interquartile range (IQR) as appropriate. Wilcoxon test was used to compare the study parameters at baseline and at the last visit. Results The mean age±SD of the patients was 68±11 years and the mean±SD follow-up was 24.7±22.7 months. Group a) included 11 patients; group b) 12 patients treated with methotrexate (MTX) and 2 hydroxychloroquine (in one of them associated to MTX); and group c) 9 patients on biologic therapy: adalimumab (ADA) (n=3), tocilizumab (TCZ) (n=5) and rituximab (RTX) (n=1). In this group, 8 patients were previously treated with DMARDS: MTX (n=7) and azathioprine (n=1). The results are shown in the TABLE. Two patients receiving MTX had to withdraw it due to raised liver function test, and two patients who were on TCZ had to discontinue it because of neutropenia. Moreover, one of them had angina pectoris related to a hypertensive episode during the third TCZ infusion. Conclusions Corticosteroids are the cornerstone of treatment of noninfectious aortitis, although frequently DMARDS (usually MTX) and sometimes biologic therapy are required. The treatment seems effective and relatively safe. Acknowledgement This study was supported by a grant from “Fondo de Investigaciones Sanitarias” PI12/00193 (Spain). This work was also partially supported by RETICS Programs, RD08/0075 (RIER) and RD12/0009/0013 from “Instituto de Salud Carlos III” (ISCIII) (Spain). Disclosure of Interest None declared

Background Aortitis is often refractory to conventional immunosuppressive (IS) therapy. The use of biological therapy, such as tocilizumab (TCZ) and anti-TNFα agents, had been reported. Objectives Our aim was to compare the efficacy of TCZ with anti-TNFα therapy in patients with aortitis. Methods Retrospective multicenter study of patients with aortitis refractory to traditional IS agents. Results We studied 44 patients (36 W/8 M; 51±19 years); 25 with TCZ and 19 with anti-TNFα agents (IFX=14, ADA=3, and ETN=2). Baseline features of patients on TCZ compared to the antiTNFα group (always in this order) showed a) mean age: 58±20 vs 42±13 years (p=0.003), b) women: 84% vs 79%, (p=0.97), c) underlying conditions: Takayasu arteritis, 8 vs 11 cases (p=0.08); giant cell arteritis, 15 vs 2 (p=0.0025); relapsing polychondritis, 1 vs 1 (p=0.59); ulcerative colitis, 0 vs 1 (p=0.88); Crohn9s disease, 0 vs 1 (p=0.88); Behcet9s disease, 0 vs 1 (p=0.88); sarcoidosis, 0 vs 1 (p=0.88); psoriatic arthritis, 0 vs 1 (p=0.88); and idiopathic aortitis, 1 vs 0 (p=0.88) d) mean of previous traditional IS agents (1.1 vs 1.7, p=0.069) and biological therapies (0.3 vs 0.1, p=0.23). After 3 months of treatment, most patients in both groups had experienced a clinical and acute phase reactants improvement, as well as a reduction of the corticosteroid dose. This favorable response was maintained over time (Table). The improvement observed by imaging techniques was similar in both groups. After a median follow-up of 12 [9-17] vs 16 [12-36] months (p=0.014), TCZ was withdrawn due to severe neutropenia (n=1); recurrent pneumonia (n=1); cytomegalovirus infection (n=1) and systemic lupus erythematosus (n=1). Other adverse effects were thrombocytopenia (n=1) and infusional hypotension (n=1). One patient died due to a stroke in the setting of an infective endocarditis, and another one discontinued TCZ because of inefficacy. In the anti-TNFα group, 3 patients on IFX discontinued due to inefficacy (n=1), recurrent pneumonia (n=1) and severe infusional reaction (n=1). Conclusions Biological therapy appears effective and relatively safe in patients with aortitis refractory to traditional IS drugs. In this series, TCZ seems to be slightly more effective than antiTNFα agents. Disclosure of Interest None declared

OBJECTIVES:Non-infectious aortitis often presents with non-specific symptoms leading to inappropriate diagnostic delay. We intend to describe the clinical spectrum and outcome of patients with aortitis diagnosed at a single centre.METHODS:We reviewed the clinical charts of patients diagnosed with non-infectious aortitis between January 2010 and December 2013 at the Rheumatology Division from a 1.000-bed tertiary teaching hospital from Northern Spain. The diagnosis of aortitis was usually based on FDG-PET-CT scan, and also occasionally on CT or MRI angiography or helical CT-scan.RESULTS:During the period of assessment 32 patients (22 women and 10 men; mean age 68 years [range, 45-87]) were diagnosed with aortitis. The median interval from the onset of symptoms to the diagnosis was 21 months. FDG-PET CT scan was the most common tool used for the diagnosis of aortitis. The underlying conditions were the following: giant cell arteritis (n=13 cases); isolated polymyalgia rheumatica (PMR) (n=11); Sjögren's syndrome (n=2), Takayasu arteritis (n= 1); sarcoidosis (n=1), ulcerative colitis (n=1), psoriatic arthritis (n=1), and large-vessel vasculitis that also involved the aorta (n=2). The most common clinical manifestations at diagnosis were: PMR features, often with atypical clinical presentation (n=23 patients, 72%); diffuse lower limb pain (n=16 patients, 50%); constitutional symptoms (n=12 patients, 37%), inflammatory low back pain (n=9 patients, 28%) and fever (n=7 patients, 22%). Acute phase reactants were increased in most cases (median erythrocyte sedimentation rate 46 mm/1st hour, and a median serum C-reactive protein 1.5 mg/dL).CONCLUSIONS:Aortitis is not an uncommon condition. The diagnosis is often delayed. Atypical PMR features, unexplained low back or limb pain, constitutional symptoms along with increased acute phase reactants should be considered 'red flags' to suspect the presence of aortitis.

Background Aortitis is often refractory to conventional immunosuppressive (IS) therapy. The use of biological therapy, such as tocilizumab (TCZ) and anti-TNFα agents, had been reported. Objectives Our aim was to compare the efficacy of TCZ with anti-TNFα therapy in patients with aortitis. Methods Retrospective multicenter study of patients with aortitis refractory to traditional IS agents. Results We studied 44 patients (36 W/8 M; 51±19 years); 25 with TCZ and 19 with anti-TNFα agents (IFX=14, ADA=3, and ETN=2). Baseline features of patients on TCZ compared to the antiTNFα group (always in this order) showed a) mean age: 58±20 vs 42±13 years (p=0.003), b) women: 84% vs 79%, (p=0.97), c) underlying conditions: Takayasu arteritis, 8 vs 11 cases (p=0.08); giant cell arteritis, 15 vs 2 (p=0.0025); relapsing polychondritis, 1 vs 1 (p=0.59); ulcerative colitis, 0 vs 1 (p=0.88); Crohn9s disease, 0 vs 1 (p=0.88); Behçet9s disease, 0 vs 1 (p=0.88); sarcoidosis, 0 vs 1 (p=0.88); psoriatic arthritis, 0 vs 1 (p=0.88); and idiopathic aortitis, 1 vs 0 (p=0.88) d) mean of previous traditional IS agents (1.1 vs 1.7, p=0.069) and biological therapies (0.3 vs 0.1, p=0.23). After 3 months of treatment, most patients in both groups had experienced a clinical and acute phase reactants improvement, as well as a reduction of the corticosteroid dose. This favorable response was maintained over time (Table). The improvement observed by imaging techniques was similar in both groups. After a median follow-up of 12 [9-17] vs 16 [12-36] months (p=0.014), TCZ was withdrawn due to severe neutropenia (n=1); recurrent pneumonia (n=1); cytomegalovirus infection (n=1) and systemic lupus erythematosus (n=1). Other adverse effects were thrombocytopenia (n=1) and infusional hypotension (n=1). One patient died due to a stroke in the setting of an infective endocarditis, and another one discontinued TCZ because of inefficacy. In the anti-TNFα group, 3 patients on IFX discontinued due to inefficacy (n=1), recurrent pneumonia (n=1) and severe infusional reaction (n=1). Conclusions Biological therapy appears effective and relatively safe in patients with aortitis refractory to traditional IS drugs. In this series, TCZ seems to be slightly more effective than antiTNFα agents. Disclosure of Interest None declared

Background Non infectious aortitis may present as an idiopathic isolated condition or associated with a wide spectrum of diseases. Aortitis often presents with nonspecific symptoms leading in many cases to an inappropriate diagnostic delay. Objectives Our aim was to analyze the clinical features and outcome of patients with aortitis in order to improve the diagnosis of this entity. Methods We studied 32 patients (22 women and 10 men) with a mean age of 68 years (range, 45-87 years) at the time of diagnosis. The median interval from the clinical onset to the diagnosis was 21 months. F18-FDG PET scan was the usual radiological method for diagnosing aortitis. Results The underlying conditions were: giant cell arteritis (n=13 cases); isolated polymyalgia rheumatica (PMR) (n=11); Sjögren syndrome (n=2), Takayasu arteritis (TakA) (n=1); sarcoidosis (n=1), ulcerative colitis (n=1), psoriatic arthritis (n=1), and idiopathic aortitis (n=2). The most common clinical manifestations at diagnosis were: PMR features, often atypical in the clinical presentation (n=23 patients, 72%); diffuse lower limb pain (n=16 patients, 50%); constitutional symptoms (n=12 patients, 37%), inflammatory back pain (n=9 patients, 28%) and fever (n=7 patients, 22%). In most of the cases, serum acute phase reactants were increased, with a median erythrocyte sedimentation rate of 46 mm/1st hour and a median serum C-reactive protein of 1.5 mg/dL. Conclusions In conclusion, aortitis is not an uncommon disease. The diagnosis is often a challenge for the clinician. The presence of PMR features, in particular when they are atypical, unexplained low back or limb pain, constitutional symptoms along with increased acute phase reactants should be considered “red flags” to suspect the presence of an underlying aortitis. Acknowledgements This study was supported by a grant from “Fondo de Investigaciones Sanitarias” PI12/00193 (Spain). This work was also partially supported by RETICS Programs, RD08/0075 (RIER) and RD12/0009/0013 from “Instituto de Salud Carlos III” (ISCIII) (Spain). Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.2978

AbstractThe severity of clinical features and the outcomes in previous series of patients reported with Henoch-Schönlein purpura (HSP) vary greatly, probably due to selection bias. To establish the actual clinical spectrum of HSP in all age groups using an unselected and wide series of patients diagnosed at a single center, we performed a retrospective review of 417 patients classified as having HSP according to the criteria proposed by Michel et al. Of 417 patients, 240 were male and 177 female, with a median age at the time of disease diagnosis of 7.5 years (interquartile range [IQR], 5.3–20.1 yr). Three-quarters of the patients were children or young people aged 20 years or younger (n = 315), and one-quarter were adults (n = 102). The most frequent precipitating events were a previous infection (38%), usually an upper respiratory tract infection, and/or drug intake (18.5%) shortly before the onset of the vasculitis. At disease onset the most common manifestations were skin lesions (55.9%), nephropathy (24%), gastrointestinal involvement (13.7%), joint symptoms (9.1%), and fever (6.2%). Cutaneous involvement occurring in all patients, mainly purpuric skin lesion, was the most common manifestation when the vasculitis was fully established, followed by gastrointestinal (64.5%), joint (63.1%), and renal involvement (41.2%). The main laboratory findings were leukocytosis (36.7%), anemia (8.9%), and increased serum IgA levels (31.7%). The most frequent therapies used were corticosteroids (35%), nonsteroidal antiinflammatory drugs (14%), and cytotoxic agents (5%). After a median follow-up of 12 months (IQR, 2–38 mo), complete recovery was observed in most cases (n = 346; 83.2%), while persistent, usually mild, nephropathy was observed in only 32 (7.7%) cases. Relapses were observed in almost a third of patients (n = 133; 31.9%).In conclusion, although HSP is a typical vasculitis affecting children and young people, it is not uncommon in adults. The prognosis is favorable in most cases, depending largely on renal involvement.

Background Patients with spondyloarthritis (SpA) frequently show other immune-mediated inflammatory diseases (IMID) Objectives To describe the 2-year incidence of new diagnoses of IMID in patients with SpA recruited for the AQUILES study Methods AQUILES was a prospective observational study on 3 independent cohorts of patients aged ≥18 years-old with SpA, inflammatory bowel disease (IBD) or psoriasis recruited from rheumatology, IBD and dermatology offices from 15 hospitals in Spain. Patients were followed for 2 years. In the SpA cohort, the diagnosis of new IMID was based on reports from gastroenterologists (IBD), dermatologists (psoriasis) or ophthalmologists (uveitis) Results A total of 513 patients with SpA completed the 2-year follow-up (mean age: 48 years-old; 37.5% females, 62.5% males). Median duration of disease was 7.8 years. The diagnoses at baseline were: ankylosing spondylitis (AS) (n=285), psoriatic arthritis (PsA) (n=130), enteropathic arthritis (n=13), undifferentiated arthritis (n=83) and others (n=2). At baseline, 45% had other IMID (4.9% IBD; 28.7% psoriasis; 14.6% uveitis). During the 2-year period, 22 new diagnoses of incident IMID were recorded (cumulative incidence: 4.3%, 95% CI: 2.4-6.1; incidence rate: 17 cases per 10,000 patients-year). The predominant incident IMID was uveitis (16 cases, cumulative incidence 3.1%, 95% CI: 1.5-4.7). In the subgroup with AS, there were 16 new cases of IMID (5.6%; 13 of them uveitis [4.6%], 1 IBD [0.4%] and 2 psoriasis [0.8%]). In patients with PsA only 2 patients developed a new IMID (2 new diagnoses of psoriasis [1.5%]). In those with undifferentiated SpA, there were 2 new diagnoses of uveitis (2.4%) and 1 psoriasis (1.2%), and 1 patient with enteropathic arthritis developed uveitis. There were no differences in the incidence by patient9s age, gender, duration of disease, extra-articular manifestations or family history of SpA, and the multivariate analysis identified the diagnosis of AS as the only predictor of development of new IMID (OR: 3.5 [1.0-15.3, p=0.049] versus psoriatic arthritis) Conclusions In SpA patients recruited for the AQUILES study, the 2-year cumulative incidence of new IMID was 4.3%, mostly due to new patients with diagnosis of uveitis in patients with AS. Diagnosis of AS at baseline was the only predictor for the development of a new IMID Acknowledgements The AQUILES study was funded by Merck Sharp & Dohme of Spain. The authors thank the investigators for the recruitment and follow-up of patients Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.3090

Background The severity of clinical features and the outcome in the different series of patients with Henoch-Schönlein Purpura (HSP) shows great variability, probably due to selection-bias. Objectives Our aim was to establish the actual clinical spectrum of HSP in all age groups using an unselected and wide series of patients diagnosed at a single center. Methods For this purpose, we performed a retrospective review of 417 patients classified as having HSP according to the criteria proposed by Michel et al.1 Results From this series of 417 patients, 240 were men and 177 women with a median age at the time of disease diagnosis of 7.5 years (interquartile range-IQR: 5.3-20.1). Three-quarters of them were children or young people 20 years old and younger (n=315) and a quarter (n=102) were adults. The most frequent precipitating events were a previous infection (38%), usually an upper respiratory tract infection, and/or drug intake (18.5%) shortly before the onset of the vasculitis. At disease onset the most common manifestations were skin lesions (55.9%), nephropathy (24%) gastrointestinal involvement (13.7%), joint symptoms (9.1%), and fever (6.2%). Cutaneous involvement occurring in all the cases, mainly purpuric skin lesion, was the most common manifestations when the vasculitis was fully established, followed by gastrointestinal (64.5%), joint (63.1%), and renal involvement (41.2%).The main laboratory data were leukocytosis (36.7%), anemia (8.9%) and increased of serum IgA levels (31.7%). The most frequent therapies used were corticosteroids (35%), nonsteroidal anti-inflammatory drugs (14%), and cytotoxic agents (5%). After a median follow-up of 12 (IQR: 2-38) months, complete recovery was observed in most cases (n=346; 83.2%) while persistent, usually mild, nephropathy was observed in 32 (7.7%) cases. Relapses were observed in almost a third of patients (n=133; 31.9%). Conclusions In conclusion, although HSP is a typical vasculitis affecting children and young people, it is not uncommon in adults. The prognosis is favorable in most cases depending mostly on renal involvement. References Michel BA, Hunder GG, Bloch DA, Calabrese LH. Hypersensiviti vasculitis and Henoch-Schönlein purpura: a comparison between the two disorders. J Rheumatol. 1992; 19:721-728. Acknowledgements This study was supported by a grant from “Fondo de Investigaciones Sanitarias” PI12/00193 (Spain). This work was also partially supported by RETICS Programs, RD08/0075 (RIER) and RD12/0009/0013 from “Instituto de Salud Carlos III” (ISCIII) (Spain). Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.4358

Background Aortitis is the inflammation of the aortic wall, regardless of the underlying condition. It can occur alone or associated with other causes. Aortitis is often refractory to standard immunosuppressive therapy. Objectives To assess the efficacy and side-effects of biological therapy in patients with inflammatory aortitis. Methods Multicenter study of 30 patients diagnosed of inflammatory aortitis due to different underlying conditions. The diagnosis of aortitis was based on imaging (CT angiography, MR angiography, PET or echocardiogram). Results We studied 30 patients (27women/3men); mean age±SD, 47.07±18.62 years. The underlying etiologies were: Takayasu arteritis (TKY) (16 cases), giant cell arteritis (GCA) (7), relapsing polychondritis (2), sarcoidosis (1), ulcerative colitis (1), Sjögren’s syndrome (1), Behcet’s syndrome (1) and idiopathic aortitis (1) (Table). In 3 of 30 patients, biological therapy had to be discontinued: 1 GCA with tocilizumab (TCZ) because of neutropenia, 1 GCA with infliximab (IFX) for infusional reactions and 1 TKY with IFX for recurrent pneumonia. Of the remaining 27 patients undergoing biologic therapy, 13 were receiving TCZ (6 TKY, 5 GCA, 1 relapsing polychondritis and 1 idiopathic aortitis), 11 IFX (7 TKY, 2 GCA, 1 Behcet’s syndrome, and 1 relapsing polychondritis), 2 were receiving etanercept (2 TKY), 2 rituximab (1 TKY and 1 Sjögren’s syndrome) and 2 adalimumab therapy (1 ulcerative colitis and 1 sarcoidosis). Switching from a biologic therapy to another occurred in 8 cases. It was due to inefficacy in 7 cases and allergic reaction in 1 case. After a median [interquartile 25-75] follow-up of 16 [11-24] months most patients experienced clinical improvement and a reduction of ESR levels (from 42.6±29.4 mm/1st h to 14.8±14.6 mm/1st h). This fact made possible a reduction in the dose of corticosteroids (prednisone: 25.8±20.5 mg/day to 4.6±3.9 mg/day). Conclusions Our results indicate that biological therapy seems to be an effective and safe therapeutic option in inflammatory aortitis refractory to conventional immunosuppressive therapy. Disclosure of Interest None Declared

Background Henoch-Schonlein Purpura Nephritis (HSPN) and IgA Nephropathy (IgAN) have been traditionally considered related diseases Objectives To establish the possible differences and similarities between both entities. Methods Study of an unselected population of 141 patients with HSPN and 55 with IgAN from a teaching hospital. HSP was diagnosed according to the criteria proposed by Michel et al (J Rheumatol 1992). IgAN was diagnosed according to renal biopsy in 49 patients, in the remaining 6 cases biopsy was not performed due to typical clinical features. The statistical analysis was performed with the STATISTICA software (Statsoft Inc.). Continuous variables (normally and not normally distributed) were compared with the 2-tailed Student’s t-test or the Mann-Whitney U test, respectively. The chi-square test was used for the dichotomous variables. Results The mean age at onset was of 28.7±26.6 years (range; 2-84) in HSPN and 35.5±13.2 (range; 12-68) in IgAN (p=NS). Most patients in both conditions were male; 56.7% in HSPN and 63.6% in IgAN (p=NS). Precipitating events were found in 58 (41.2%) of patients with HSPN and 5 (27.3%) cases with IgAN (p= NS). The most frequent precipitating events in HSPN and IgAN were respectively drug intake (22% vs 22.5%; p NS) and an upper respiratory tract infection (34.5% vs 19.5%; p=NS). Extra-renal manifestations were more common in HSPN [skin (97.1% vs 18.6%; p<0.001), gastrointestinal (68.8% vs 14.6%; p<0.001), and joint involvement (64.5% vs 7%; p<0.001)]. Renal involvement was more severe in IgAN [renal insufficiency (15.5% vs 54.5%; p<0.001); nephrotic syndrome (11% vs 52.8%; p<0.001), and nephritic syndrome (11% vs 18.2%; pNS)]. Anemia was more common in IgAN (11.4% vs 31.2%, p=0.001). Increased serum levels of IgA were similar (58% in HSPN vs 71.4% in IgAN; p= NS). C4 was decreased in 10.7% (10 of 93 tested) patients with PSHN and in 0 of 39 patients with IgAN (p=0.03). Renal biopsies were performed in 16 patients with PSHN and in 49 with IgAN. The main histological findings were similar in both groups, in most cases mesangial hypercellularity with deposits of IgA and C3 in the mesangium. Drug therapy requirement was similar (57.1% in HSPN vs 69.2% in IgAN; p=NS). However, the initial corticosteroid dose was higher in IgAN (mean prednisone in mg/day; 35.5±15.5 vs 57.5±14.7, p=0.002). The disease relapsed in 36.4% of PSHN and in 58.8% of the IgAN (p=NS). After a median follow-up of 48 months; interquartile range (5.5, 60) and 48 months (12, 139), persisting mild renal insufficiency was observed in 4 and 6 cases and mild hematuria in 26 and 2 cases with PSHN and IgAN, respectively. Conclusions Our study shows that IgAN patients exhibit more severe renal involvement, with a higher frequency of renal insufficiency and nephrotic syndrome. As expected, extra-renal involvement was more frequent in HSPN. However, the final outcome was equally good in most patients with both diseases. Disclosure of Interest None Declared

OBJECTIVES:Henoch-Schönlein purpura nephritis (HSPN) and IgA nephropathy (IgAN) are related syndromes. In the present study we aimed to compare the clinical characteristics and outcome of a large and unselected series of patients diagnosed as having HSPN and IgAN.METHODS:Comparative study of a wide and unselected population of HSPN (142 patient) and IgAN (61 patients) from a teaching hospital of Northern Spain.RESULTS:All of the following comparisons were expressed between HSPN vs. IgAN, respectively. HSPN patients were younger (30.6±26.4 vs. 37.1±16.5 years, p<0.001). Precipitating events, usually an upper respiratory tract infection and/or drug intake, were more frequently observed in HSPN (38% vs. 23%, p=0.03). Extra-renal manifestations were also more common in HSPN than in IgAN; skin lesions (100% vs. 1.8%; p<0.001), gastrointestinal (62% vs. 7.4%; p<0.001), and joint involvement (61.3% vs. 3.6%; p<0.001). However, nephritis was less severe in HSPN, renal insufficiency (25% in HSPN vs. 63.4% in IgAN; p<0.001), nephrotic syndrome (12.5%, vs. 43.7%; p<0.001), and nephritic syndrome (6.8% vs. 10.7%; NS). Leukocytosis was more frequent in HSPN (22.5% vs. 8.2%; p=0.015) and anaemia in IgAN (12.7% in HSPN vs. 36% in IgAN, p<0.001). The frequency of corticosteroid (79.6% vs. 69%; NS) and cytotoxic drug (19% vs. 16.5%, NS) use was similar. The frequency of relapses was similar (38.6% in HSPN vs. 36.3% in IgAN). After a median follow-up of 120.8 (IQR; 110-132) months in HSPN and 138.6 (IQR; 117-156) in IgAN, requirement for dialysis (2.9% vs. 43.5%; p<0.001), renal transplant (0% vs. 36%, p<0.001) and residual chronic renal insufficiency (4.9% vs. 63.8%; p<0.001) was more frequently observed in patients with in IgAN.CONCLUSIONS:HSPN and IgAN represent different syndromes. IgAN has more severe renal involvement while HSPN is associated with more extra-renal manifestations.

Background AQUILES is a 2-year follow-up observational prospective study in patients with spondyloarthritis (SpA), inflammatory bowel disease [IBD] or psoriasis, to assess: 1) the coexistence of immune-mediated inflammatory diseases (IMID); 2) the prevalence of comorbidities and 3) the incidence of new clinical manifestations of IMID over a 2-year follow-up period Objectives To describe the baseline characteristics and the prevalence of concomitant IMIDs in the population with diagnosis of SpA recruited in Rheumatology offices Methods From March 2008 to December 2010, patients with known or newly diagnosed SpA, IBD or psoriasis were recruited from Rheumatology, IBD and Dermatology offices (15 centers). For Rheumatologists, inclusion criteria were age ≥18 years-old and previous or new diagnosis of SpA. Clinical data were collected through direct interview and patient’s clinical record review following a protocol agreed by the 3 specialties Results In Rheumatology offices, 563 patients were recruited (mean age: 48.5 years-old [SD: 12.7]; 62.5% males, 37.5% females; mean disease duration 7 years [IQR: 2-14]). A history of familial SpA was present in 19.2%, and 7.5% had newly diagnosed SpA. The diagnoses at baseline were: ankylosing spondylitis (AS) (52.9%), psoriatic arthritis (PsA) (25.2%), undifferentiated SpA (7.3%), enteropathic arthritis (2.3%), and others (12.3%). BASDAI activity index was 3.4 (SD: 2.6). The proportion of SpA patients with IBD at baseline was 5.0% (Crohn disease: 3.0%, ulcerative colitis: 1.1%, indeterminate colitis: 0.9%). The proportion was similar in men and women (4.4% vs. 5.9%, p=NS), in patients with familial vs. sporadic SpA (1.9% vs. 6.4%, p=0.064), or new vs. known SpA (5.0% vs. 4.8%, p=NS). Excluding those with enteropathic arthritis, the proportion with IBD was 2.7% (3.0% in AS, 1.4% in PsA, 2.4% in undifferentiated SpA, p=NS). The proportion of patients with psoriasis was 27.5% (4.5% excluding PsA; 4.4% in AS, 95.8% in PsA, 4.9% in undifferentiated SpA; p<0.05 for PsA vs. the others), was higher in women (33.3% vs. 25.3% in men, p=0.044) and similar in familial vs. sporadic (21.3% vs. 30.0%, p=0.075) or new vs. known disease (21.4% vs. 28.0%, p=NS). Finally, the prevalence of uveitis was 13.3% (19.6% in AS, 0.7% in PsA, 12.2% in undifferentiated SpA; p<0.05 for PsA compared to the others), and was similar in men and women (14.4% vs. 12.3%, p=NS) and in new vs. known disease (9.5% vs. 13.6%, p=NS), but higher in those with familial disease (21.3% vs. 10.7% in sporadic SpA, p=0.004) Conclusions In patients with SpA recruited in Rheumatology offices for the AQUILES study in Spain, the proportion with other IMIDs was similar to that described in the literature, and higher than the known prevalence in general population of Spain. We did not observe relevant differences between those with newly diagnosed and previously known SpA Disclosure of Interest R. García-Vicuña: None Declared, P. Zarco: None Declared, A. Rodríguez de la Serna: None Declared, E. Peirό: None Declared, E. Collantes: None Declared, L. Cea-Calvo Employee of: Merck Sharp & Dohme, J. Nadal Employee of: Merck Sharp & Dohme, F. Vanaclocha: None Declared, I. Marín-Jiménez: None Declared, C. González: None Declared

Background Chloroquine and Hydroxichloroquine are well-known anti-malarial drugs used in the management of several rheumatologic diseases, in particular in patients with Systemic Lupus Erythematosus (SLE) and Rheumatoid Arthritis (RA). Apart from the ophthalmologic complication, these drugs are considered to be relatively safe drugs. However, their prolonged use may be associated with other complications, including heart rhythm problems such as heart block. Methods We reviewed the medical records of a series of patients seen at the outpatient Rheumatology Clinic of a University Hospital, who required prolonged anti-malarial drug therapy. In the present study we aimed to identify the alterations in the heart conduction system as well as the conditions associated to these abnormalities. Results From a series of 212 patients treated with antimalarials, 154 (72.64%) were diagnosed with RA, 57 (26.88%) with SLE and 1 with Still disease. We disclosed 9 patients (6 females/3 males) who experienced heart conduction disturbances. The mean age at time of the diagnosis of the cardiac conduction disturbances was 61.3±17.2 years (range 20-81). The mean duration of the disease before the onset of these heart complications was 90±52 (18- 204) months. SLE was the disease more frequently associated with these complications (5 patients) followed by RA (3 cases) and Still disease (1 case). Two of 9 who suffered the heart complication had abnormal kidney function with a clearance ≤40mg/dl. Chloroquine and hydroxichloroquine were administrated in a single dose of 250 mg/daily and 200 mg/twice daily respectively. The mean weight of the patients was 66, 33 kg ± 7,28 (51-75).The weight-adjusted dose for the chloroquine were 3.87±0.5 mg/kg (3.47-4.9) and 5.9±0.55 (5.3-6.3) for the hydroxichloroquine. Before the onset of antimalarial therapy only 2 of the patients had abnormal ECG (atrial fibrillation (AF) and right bundle-branch block respectively). Following anti-malarial drug therapy the following heart conduction disturbances were observed: 5 atrioventricular blocks (2 associated with right bundle-branch block and other one with left bundle-branch), 2 right bundle-branch block, 1 left bundle-branch block, 1 blocked AF associated with right bundle-branch. Seven of these patients required pacemaker. Conclusions We must be aware of the potential risk of having heart conduction disturbances following prolonged used of anti-malarial drugs. Disclosure of Interest None Declared

Background Most Henoch-Schonlein Purpura (HSP) studies came from selected series and used different classification criteria Objectives Our aim was to assess the epidemiological, clinical features, treatment and prognosis in an unselected and well-defined seriesof HSP. Methods Retrospective study of a wide series of patients from two hospitals diagnosed as having HSP. Patients with vasculitis secondary to connective-tissue diseases, malignancies, severe infections, primary systemic necrotizing vasculitides, and those with other well-defined clinical conditions such as hipersensitivity vasculitis and essential myxed cryoglobulinemia were excluded from this analysis. Patients were classified as having HSP according to the criteria proposed by Michel et al [1]. Results According to the above mentioned criteria 340 patients (194 men/146 women), with a mean ±SD age of 18.7±22.6 years (range; 1 to 87) were classified as having HSP. Most of the patients (256) were young people (subset established if the disease occurred in individuals younger than 20 years) (6.9±3.1 years) and 84 were adults (54.6±17.5 years). Precipitating events were found in 41.2% of patients. A history of drug intake before the onset of vasculitis was found in 19.7% and a previous upper respiratory tract infection in 35.3%. At disease onset, the most common manifestations were skin lesions (62%), abdominal pain (19.5%), joint symptoms (11%) and fever (8.8%). When the HSP disease was diagnosed the most frequent manifestations were cutaneous (99.7%), gastrointestinal (65.6%), joint (63%), and renal involvement (41.5%). The main laboratory data were elevated ESR (31.9%), leukocytosis (32.6%), anemia (8.5%), positive rheumatoid factor (6 of 139 cases tested), positive ANA (17 of 148), C3 and/or C4 decreased (14 of 285), negative cryoglobulins (60 of 78) and negative ANCAs (35 of 35). The more frequent treatments were: NSADs (11.2%), corticosteroids (33.5%), and cytotoxic agents (5.3%) (azathioprine in 6 cases, cyclophosphamide in 6 and methotrexate in 1). After a mean follow-up of 32.9±51.2 (median, 12) months, relapses were observed in 28.5% of patients, complete recovery in 84.1%, persisted mild renal insufficiency in 4 cases and mild hematuria in 26 cases. Conclusions HSP as defined by the criteria proposed by Michel et al, represents a relatively benign syndrome that affects predominantly to young population. References Michel BA, Hunder GG, Bloch DA, Calabrese LH. Hypersensitivity vasculitis and Henoch-Schönlein purpura: a comparison between the 2 disorders. J Rheumatol. 1992 May;19(5):721-8. Disclosure of Interest None Declared

Background Non-infectious aortitis is often an underdiagnosed and potentially serious clinical entity. Although giant cell arteritis (GCA) is considered to be the most common cause of aortitis, it may also be idiopathic or associated with other conditions. Clinical manfiestations are frequently nonspecifc leading to inapropriate delay to the diagnosis. Methods Multicenter study of three hospitals of patients diagnosed as having aortitis by F18-FDG PET. Results Twelve patients (8 women/4 men) with a mean age of 68.4±12.7 years were diagnosed with aortitis by F18-FDG PET (TABLE). The median [interquartile range] of delay to the diagnosis was 12 [2-20] months. Aortitis was idiopathic (5 cases), associated with biopsy-proven GCA (n=4), Sarcoidosis (n=1), Ulcerative Colitis (n=1), and Takayasu arteritis (n=1). The most frequent symptoms were: A) Polymyalgia rheumatica (PMR) in 8 cases (67%), that was atypical in 5 of them. B) Fever and/or general syndrome in 6 cases (50%). And C) Severe low back pain in 4 cases (33%). Laboratory data disclosed anemia in all cases and high ESR in most cases (78.1±38.3 mm/1st hour). PET showed involvement of A) the whole aorta, thoracic aorta and/or supra-aortic vessels in 91% and B) involvement of arteries of the lower extremities in 50%. Conclusions F18 FDG-PET is a useful tool to confirm the diagnosis of aortitis. Atypical PMR, non-specific general syndrome, and/or unexplained severe low back pain along with anemia and elevated ESR may be red flags to suspect this condition. Disclosure of Interest None Declared