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Objective: This paper describes the baseline demographics, clinical characteristics, and patient-reported outcomes (PROs) according to clinical phenotype of patients with early psoriatic arthritis (PsA) for the purpose of creating a decision support system for daily clinical practice. Method: Patients with newly diagnosed PsA were included in the Dutch south west Early Psoriatic ARthritis (DEPAR) study. No classification criteria were applied, to ensure collection of real-world data on demographics, medication, clinical characteristics, and PROs. An IT infrastructure facilitated data collection. Results: We described 527 patients, categorized according to the clinical phenotype stated by the rheumatologist at the time of diagnosis, namely monoarthritis (15%), oligoarthritis (40%), polyarthritis (23%), enthesitis (10%), axial disease (2%), and dactylitis (10%). Overall psoriasis severity was mild and 83 patients (16%) had no psoriasis. Short-term sick leave (> 1 day per 4 weeks) was 17% and long-term sick leave (> 4 weeks) was 4%. The group with phenotype enthesitis reported the longest duration of complaints, had the highest fatigue scores, and contained the highest percentage of patients with a Hospital Anxiety and Depression Scale (HADS) anxiety score ≥ 8 and depression score ≥ 8. Conclusion: PsA patients presenting at outpatient clinics in the Netherlands had a mild degree of psoriasis, with impairment of quality of life and work productivity. Most patients presented with phenotype oligoarthritis. Those presenting with phenotype enthesitis more often reported scores suggestive of an anxiety or depression disorder and fatigue. It is important for attending rheumatologists to be aware of these differences when assessing patients with PsA.

Intussusception is a rare diagnosis in adults. Gastrointestinal involvement is not unusual in systemic lupus erythematosus (SLE).(1) A case of intussusception as first presenting symptom of SLE is described. A 50-year-old woman of Chinese origin came to the emergency room with symptoms of high fever, nausea, vomiting and weight loss of 10 kg. Three months before, an episode with abdominal pain due to ileal invagination in the colon occurred. Laboratory analysis showed: thrombocytopaenia, lymphopaenia, positive antinuclear antibody, antidouble-stranded DNA, Coombs tests and low C3 in combination with an active urine sediment and proteinuria. CT and positron emission tomography scan showed lymphadenopathy, but no other abnormalities. Axillar lymph node biopsy showed no abnormalities. The diagnosis new-onset SLE was made. Prednisone treatment was started and soon thereafter tapered, azathioprine and hydroxychloroquine were added. During follow-up, the SLE remained in remission. SLE is a rare cause of ileocolic intussusception and ileocolic intussusception may be the first presenting symptom.

Background The Psoriatic Arthritis Impact of Disease 12-item questionnaire (PsAID12) has been developed to measure impact of Psoriatic Arthritis (PsA) for purposes of monitoring and clinical management. Although validated in patients with longstanding disease, data on validity and sensitivity to change in early PsA is lacking. Objectives We aim to relate change in disease activity to change in PsAID12 score in early PsA and evaluate which PsAID domains are more likely to change. Methods Patients with a new diagnosis of PsA were included in the Dutch southwest Early Psoriatic Arthritis cohoRt (DEPAR). For this analysis, patients that have PsAID12 (range 0–10) and Composite Psoriatic Disease Activity Index (CPDAI, range 0–15) data at two consecutive visits (i.e. 3 months apart) within the first year were included. In case multiple periods per patients were available, the first time period was chosen. The change in PsAID is compared to the change in disease activity over this period, measured with the CPDAI using Spearman9s correlation coefficient. Change in score on individual domains of the PsAID was analysed in subgroups of patients that perceived improvement in health and those that perceived worsening. The SF-36 question on self-perceived change in health was used to determine these subgroups. Results 143 unique patients had at least one period with two PsAID and CPDAI measurements (67 from baseline-3 months, 26 3–6 months, 25 6- 9 months and 25 9–12 months). Mean age was 51 (SD 13.7) and 70 (49%) were male. The initial median PsAID was 3.35 (IQR 1.4–5.1) and the subsequent score was 2.25 (0.95–4.8 with a mean delta of 0.52 (P Conclusions Improvement in CPDAI disease activity is significantly but moderately associated with improvement in PsAID score, with the biggest improvement in the pain domain in patients with a self-reported improvement of health. Disclosure of Interest None declared

Background Psoriatic arthritis (PsA) is a multifaceted disease. Objectives We aimed to evaluate change in medication over time guided by joints, skin, enthesis, low back pain and dactylitis in newly diagnosed PsA patients Methods Newly diagnosed PsA patients were included in the Dutch Early south-west Psoriatic Arthritis cohoRt (DEPAR) study between August 2013 and March 2016. Initial drug treatment and escalation of therapy were described for all patients. Drivers of treatment changes in the first year were evaluated by mixed ordered ordinal regression with outcome treatment change and variables joints (66/68 count), skin (PASI), entheses (LEI and MASES), dactylitis (LDI) and axial disease (BASDAI) Results 323 patients had had baseline assessment in March 2016. Their average age was 50.0 years (SD 13.8) and 49% were male. 80% patients had arthritis (19% monoarthritis, 39% oligoarthritis and 23%polyarthritis), 9% had an enthesitis subtype, 2% axial disease and 9% dactylitis. Initial treatment consisted of methotrexate (MTX) (52%), in 7% of other synthetic disease modifying antirheumatic drugs (sDMARDs) and due to treatment of psoriasis 3% biologicals. Within the different phenotypes MTX was most frequently started in polyarthritis (84%) followed by oligoarthritis (63%), monoarthritis (33%) and other phenotypes (5%). At 12 months 70% (n=148) stayed on the initial drug. Of those switched, 9 started MTX, within the initial MTX users (n=74) almost equal percentages stopped, switched to metoject or biological (4%). A smaller percentage (2%) switch to leflunomide. Changes in medication were driven by swollen joint count and the presence of dactylitis (Table 1) Conclusions MTX was initiated in about half of the early PsA patients. The majority of patients were kept on the initial treatment strategy in first year. Failure on initial drug led to variation in subsequent drugs with additional start of other sdmards, switch subcutaneuous MTX, to other sdmards or to biological dmards. Treatment change was driven by Swollen Joint Count and presence of Dactylitis. Skin, Enthesis and Axial disease did not play a role in escalating treatment. Disclosure of Interest None declared

Background In Psoriatic arthritis (PsA) the pattern of joint involvement seems to be more heterogenic than Rheumatoid Arthritis (RA). Therefor the 66/68 joint count is frequently used in PsA scores. In this study we would like to investigate whether using less extensive joint counts influences PsA disease activity measures Objectives To evaluate the effect of using a 28, 44 or 66/68 joint count on Minimal Disease Activity (MDA). Methods Newly diagnosed PsA patients were included in the Dutch Early south-west Psoriatic Arthritis Registry (DEPAR) study between August 2013 and January 2017. Joint scores at baseline and 6 months were calculated using a 28, 44 and 66/68 joint count. Consecutively MDA was calculated using each of these joint counts. MDA is defined as minimal disease activity in 5 out of 7 domains (swollen joints, tender joints, PASI, patient vas pain, patient vas global, HAQ, enthesitis) Results In total, 413 patients were included into the study, of which 320 had reached 6 months follow-up at the time of this abstract. Half of the patients were male (49%), and mean age was 50 years (SD 13.8). The percentage of patients with at least one involved joint (swollen or tender) at baseline decreased from 91% using the 66/68 score to 88% with 44 joints and then to 80% with a 28 joint count (Table 1). At 6 month these scores were 66% 63% and finally 56% respectively for the 66/68, 44 and 28 joint count. After 6 months, 96 patients (30%) had achieved MDA using the original 66/68 joint count. Using a 28 joint count, 10 more patients (10% of the MDA population) were classified as having achieved MDA. Conclusions Using reduced joint count joints in PsA misclassifies around 10% of patients as having no joint involvement. It also misclassifies about 10% of the MDA population of having achieved MDA while they have not. Full 66/68 joint counts remain recommended for measuring disease activity in PsA. Disclosure of Interest None declared

Long-term bone mineral density (BMD) changes and the associated factors in systemic lupus erythematosus (SLE) patients were assessed. Despite the remarkably low overall bone loss, significant spine bone loss was associated with the use of glucocorticoids, use of antimalarials, and lower 25-hydroxyvitamin D levels, stressing the importance of prevention of osteoporosis and vitamin D deficiency in SLE patients. The aim of this study is to assess the BMD changes in patients with SLE and to identify the associated factors. Demographic and clinical data of 126 SLE patients were collected, and BMD measurements of the lumbar spine and the total hip were performed by dual-energy X-ray absorptiometry at baseline and follow-up. Statistical analyses were performed using independent Mann–Whitney U tests and linear regression analyses. At baseline, 39.7 % of the patients (90 % female, mean age 39 ± 12.2 years) had osteopenia, and 6.3 % had osteoporosis. The median follow-up duration was 6.7 years (range 1.9–9.3 years). Mean changes in BMD at the lumbar spine (−0.08 %/year) and the hip (−0.20 %/year) were not significant. During follow-up, 70 % of the patients used glucocorticoids. The mean ± SD daily glucocorticoid dose was 5.0 ± 5.0 mg. In multiple regression analysis, BMD loss at the spine was significantly associated with higher daily glucocorticoid dose and lower baseline 25-hydroxyvitamin D levels. BMD loss at the hip was associated with lower 25-hydroxyvitamin D levels at baseline, reduction of body mass index, and baseline use of antimalarials. In this 6-year follow-up study, bone loss was remarkably low. A dose-dependent relationship between glucocorticoid use and spinal bone loss was found. In addition, the use of antimalarials and lower 25-hydroxyvitamin D levels at baseline were associated with BMD loss. These findings underline the importance of prevention and treatment of vitamin D deficiency and osteoporosis in SLE, especially in patients using glucocorticoids or antimalarials.

Objective We observed 3 patients who developed severe venous and arterial thromboembolic events during treatment with adalimumab, 2 of whom had rheumatoid arthritis (RA) and 1 of whom had psoriatic arthritis. Antiadalimumab antibodies were detected in all 3 patients. We undertook this study to determine whether the development of antiadalimumab antibodies was associated with thromboembolic events during adalimumab treatment. Methods A retrospective search (with blinding with regard to antiadalimumab antibody status) for thromboembolic events was performed in a prospective cohort of 272 consecutively included adalimumab-treated RA patients. Incidence rates were calculated and hazard ratios (HRs) were estimated using Cox regression. None of the index patients were part of the cohort. Results Antiadalimumab antibodies were detected in 76 of 272 patients (28%). Eight thromboembolic events were found, 4 of which had occurred in patients with antiadalimumab antibodies. The incidence rate was 26.9/1,000 person-years for patients with antiadalimumab antibodies and 8.4/1,000 person-years for patients without those antibodies (HR 3.8 [95% confidence interval 0.9–15.3], P = 0.064). After adjustment for duration of followup, age, body mass index, erythrocyte sedimentation rate, and prior thromboembolic events, the HR was 7.6 (95% confidence interval 1.3–45.1) (P = 0.025). Conclusion These findings suggest that the occurrence of venous and arterial thromboembolic events during adalimumab treatment is higher in patients with antiadalimumab antibodies than in those without antiadalimumab antibodies. Patient numbers were relatively small; therefore, validation in other cohorts is mandatory.

Objectives To estimate the prevalence of depression in subjects with systemic lupus erythematosus (SLE) in relation to the general population and to unravel the relation between depression and SLE disease characteristics. Methods One hundred and two subjects with SLE (mean age 44.4 years) were studied using the Beck Depression Inventory (BDI) score to estimate the prevalence of depression. The BDI scores in subjects with SLE were compared with BDI scores from a pan-European population based study (Outcome in Depression International Network (ODIN) study, n = 7934), i.e. the general population. Results The mean BDI score was higher in SLE subjects (10.1 points) compared with the BDI scores derived from the general population (10.1 versus 5.6 points, respectively, p 0.1). Only 7% of SLE subjects with high BDI scores used antidepressants. Conclusion The mean BDI score and prevalence of depression are significantly higher in SLE subjects compared with the general population. No association was found between SLE disease characteristics and BDI scores. The number of depressed SLE subjects treated with antidepressants is low, suggesting inadequate recognition and treatment of depression in SLE.

A 49-year-old woman with a medical history of rheumatoid arthritis presented to the emergency room, with high fever and painful knees. In addition, she had had a mild headache for several days and some hearing loss over several months. We saw an ill patient with arthritis of both knees, from which purulent fluid was aspirated. Antibiotics were started for septic arthritis of both knees and her condition improved rapidly. However, the headache persisted and the hearing loss worsened. At the time, meningitis was suspected. Initial knee aspiration culture was positive for Neisseria meningitidis. PCR of the cerebrospinal fluid sample also was positive for N. meningitidis. The patient was finally diagnosed with bilateral septic gonarthritis secondary to a bacterial meningitis caused by N. meningitidis.

We describe a severe gastroenteritis with non-O1, non-O139 Vibrio cholerae in an immunocompromised patient returning from a holiday in Spain in July 2009. Predisposing factors and possible cholera enterotoxin production could explain the unusually grave symptomatology. The patient recovered after doxycyclin treatment.

Levamisole-contaminated cocaine can induce severe systemic vasculitis. The diagnosis can be challenging, especially when substance abuse is uncertain. We present the case of a 42-year-old woman suffering from vasculitis due to levamisole-contaminated cocaine, who persistently denied substance abuse. Symptoms included ulcerating skin lesions, arthralgia and myalgia, and the occurrence of an ileal intussusception. The definitive diagnosis was made using hair testing for toxins. She recovered through cocaine abstinence, but re-exposure resulted in a severe relapse with glomerulonephritis. Importantly, at time of the relapse, the patient became positive for both myeloperoxidase-antineutrophil cytoplasmic antibody (ANCA) and proteinase 3-ANCA. Cocaine-levamisole-induced vasculitis poses a great clinical challenge. The proper diagnostic strategy and therapy is still controversial. We highlight our diagnostic and therapeutic considerations, including hair testing for definitive proof of exposure.

Aim: To evaluate the frequency of the co-occurrence of a retinoma and another primary tumor. Method: Presentation of a case report and review of the literature. Results: A cutaneous melanoma was observed in a 52-year-old man, who was known to have two retinomas in the right eye. Five other cases were found in a review of the literature, suggesting the possibility of an increased risk of developing a second primary tumor in patients with retinoma. The occurrence of cutaneous melanoma as a second primary tumor after retinoma and/or retinoblastoma is discussed. Conclusion: There might be an increased risk for patients with retinoma to develop another primary tumor, necessitating regular follow-up of these patients.

Background Low bone mineral density (BMD) is highly prevalent in patients with systemic lupus erythematosus (SLE). In different studies, lower vitamin D levels were associated with BMD loss [1, 2]. Also, serum vitamin D levels are lower in patients carrying the FF genotype of FOK-I vitamin D receptor (VDR) polymorphism [3]. Therefore, the VDR FOK-I polymorphism could be a possible determinant of BMD loss in SLE. However, the relationship between FOK-I VDR polymorphisms and BMD has not yet been studied in SLE patients. Objectives To assess the relationship between FOK-I VDR polymorphisms and BMD in patients with SLE. Methods Demographic and clinical data of 123 SLE patients were collected. Genotyping was performed by restriction fragment length polymorphism analysis of polymerase chain reaction amplified fragments (PCR-RFLP) [3], BMD measurements of the total hip and the lumbar spine were performed using dual energy x-ray absorptiometry at baseline and at follow-up. Statistical analyses were performed using χ-square test, independent samples t-test and ANOVA test for parametric measurements. Results Of the 123 patients, (mean ± SD age 42.5±12.3 years, mean ± SD disease duration 8.9±7.8 years, 89% female, 72% Caucasian ethnicity), 47% carried the FF genotype, 39% carried the Ff genotype and 14% carried the ff genotype. Compared to patients with the FF and Ff genotypes, patients with the ff genotype had significantly higher mean BMD values ( p =0.042), T-scores ( p =0.007) and Z-scores ( p =0.033) in the lumbar spine, but not in the hip. Mean ± SD follow-up duration since inclusion was 5.3±2.9 years. Mean BMD changes during follow-up were not significantly different between the three genotypic groups. In the subgroup of patients not taking vitamin D supplements (n=56), 25(OH)D serum levels were higher in patients with the ff genotype (mean 84.83 nmol/l, SD 26.2) compared to patients with the FF/Ff genotype (mean 58.90 nmol/l, SD 29.16) ( p =0.043). Conclusions BMD of the lumbar spine was significantly higher in SLE patients carrying the FOK-I VDR ff genotype compared to the other two genotypes. This association could in part be mediated by higher mean 25(OH)D levels in the FOK-I ff patients. The possible involvement of VDR gene polymorphisms in the development of osteoporosis in SLE needs further elucidation. References Bultink IE, Lems WF, Kostense PJ, Dijkmans BA, Voskuyl AE (2005) Prevalence of and risk factors for low bone mineral density and vertebral fractures in patients with systemic lupus erythematosus. Arthritis Rheum 52:2044-50 Jacobs J, Korswagen LA, Schilder AM, van Tuyl LH, Dijkmans BA, Lems WF, Voskuyl AE, Bultink IE (2013) Six-year follow-up study of bone mineral density in patients with systemic lupus erythematosus. Osteoporos Int 24:1827-33 Smolders J, Damoiseaux J, Menheere P, Tervaert JW, Hupperts R (2009) Fok-I vitamin D receptor gene polymorphism (rs10735810) and vitamin D metabolism in multiple sclerosis. J Neuroimmunol 207:117-21 Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.3553

Background Depression is one of the most commonly reported neuropsychiatric symptoms in systemic lupus erythematosus (SLE) patients. However, the prevalence of depression in relation to the general population and its relationship with SLE disease characteristics are still largely unkown. Objectives To estimate the prevalence of depression in subjects with SLE in relation to the general population and to unravel the relation between depression and SLE disease characteristics. Methods We studied 102 patients with SLE (mean age 44.4 yrs, 88% female, mean disease duration 7.8 yrs), all fulfilling the ACR classification criteria for SLE. Severity of SLE was determined with the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and the Systemic Lupus International Collaborative Clinics Damage Index (SDI), representing disease activity and organ damage. We used the Beck Depression Inventory score (BDI), a validated screening instrument able to detect major depression and to estimate the prevalence of depression. A BDI score of more than 13 points was considered a high score. As a proxy for treatment of depression we assessed the number of patients using anti-depressants. Results The mean BDI score was higher in SLE patients (n=102) compared to the BDI scores derived from a European population based study (ODIN study, n=7934), (10.1 points in patients with SLE vs 5.6 points in subjects from ODIN, p There was no difference in disease characteristics, disease activity score (SLEDAI) and organ damage index (SDI) between SLE patients with and without depression (all p>0.1). The number of years of education was significantly lower in SLE patients with a depression compared to those without a depression (9.5 yrs. vs 12.2 yrs, p =0.004). Conclusions The mean BDI score, i.e. a proxy for prevalence of depression, is almost doubled in SLE patients compared to the mean BDI score from a large pan European population based study. Surprisingly, we found no association between disease characteristics or severity of SLE and presence of depression, determined with the BDI. Finally, we found that a small part of SLE patients with a high depression score were treated with anti-depressant medication, suggesting that depressed SLE patients might not be adequately recognized and treated for depression. For depressed SLE patients, this study provides clues that screening for depression in SLE patients should be done routinely, and that psychiatric counseling for these patients should be readily available. References Veerman JL, Dowrick C, Ayuso-Mateos JL, et al. Population prevalence of depression and mean Beck Depression Inventory score. Br J Psychiatry. 2009;195:516-9. Disclosure of Interest None Declared

Background The complement component C4 and its associated polymorphisms have a strong association with systemic lupus erythematosus (SLE). A low gene copy number (GCN) of C4 and its two isotypes (C4A and C4B) is a risk factor for SLE. At genetic level the C4 polymorphism has been studied in large groups of SLE patients of various ethnic origins. However, little is known about the relationship between these polymorphisms, serum protein levels, protein functional activity and clinical manifestations in SLE patients. Objectives The aim of this study was to assess C4 complement gene polymorphism, serum protein levels and protein functional activity in a cohort of SLE patients in relationship to clinical and serological manifestations. Methods Demographic data and ACR-criteria were collected in a cohort of 140 Dutch SLE patients (90% female, 69% Caucasian, mean age 42 yrs, mean disease duration 8.8 yrs). Measurements of total C4, C4A and C4B gene copy number, serum protein levels and protein functional activity were performed in SLE patients and in 104 healthy controls. Multiplex Ligation-dependent Probe Amplification (MLPA) assay, ELISA and functional C4 assay were used. [1,2] Statistical analyses were performed using Pearson’s chi square test, Mann-Whitney test and linear regression. Results A total C4 GCN of four was found in 35.7% of SLE patients, compared to 59.6% (p=0.0002) in controls. A GCN of less than four (i.e. a low GCN) was found in 55.7% (controls: 31.7%, p=0.0002); C4A and C4B deficiency (i.e. less than two copies) in 42.2% (controls: 25%, p=0.005) and 22.1% (controls: 33.6%, p=0.05) respectively. Median levels of serum protein and functional activity were significantly decreased in SLE patients versus controls, for both total C4 and the isotypes. As shown in table 1 presence of serositis was associated with lower C4A GCN as well as with lower C4A protein level and lower C4A functional activity. In addition associations were found between lower C4B protein level and lower C4B functional activity with presence of a history of nephritis, hemolytical anemia, anti-ds DNA and anticardiolipin antibodies. All these associations were also found in the Caucasian subgroup. Conclusions Complement C4 gene polymorphism, serum protein levels and for the first time also protein functional activity were studied in relationship to clinical and serological manifestations in SLE patients. The findings add to the understanding of the various phenotypes of systemic lupus erythematosus and advocate further studies in lupus nephritis patients. References Wouters D et al. Mol Immunol. 2009;46(4):592-600 Wouters D et al. Mol Immunol. 2007;44(16):3954 Disclosure of Interest None Declared

Background Low bone mineral density (BMD) is highly prevalent in patients with systemic lupus erythematosus (SLE). However, the determinants of long-term BMD changes in SLE are still largely unknown, since the majority of studies performed had a cross-sectional design and the few longitudinal studies had a small sample size (maximum 36) and short follow-up duration (≤3 years). Objectives The aim of this study was to assess the long-term BMD changes in patients with SLE and to identify the associated factors. Methods We prospectively studied 126 SLE patients. Demographic and clinical data were collected and BMD measurements of the lumbar spine and total hip were performed by dual energy x-ray absorptiometry at baseline and at follow-up. Statistical analyses were performed using repeated measures t-test, Mann-Whitney U-test, univariate and multivariate regression analyses. Results At baseline, osteopenia was present in 39.7% of the patients (90% female, mean age 39±12.2 years) and osteoporosis in 6.3%. After a median follow-up duration of 6.7 years (range 1.9 - 9.3 years), mean changes in BMD at the lumbar spine (-0.08%/year) and at the hip (-0.20%/year) were not significant. During follow-up, 70% of the patients used glucocorticoids. The mean ± SD daily glucocorticoid dose was 5.0±5.0 mg. In multiple regression analysis, BMD loss at the spine was significantly associated with higher daily glucocorticoid dose ( p =0.004) and lower baseline 25-hydroxyvitamin D levels ( p =0.030) whereas BMD gain at the spine was significantly associated with use of immunosuppressive medication other than glucocorticoids ( p =0.016). BMD loss at the hip was associated with lower 25-hydroxyvitamin D levels ( p =0.030), reduction of body mass index ( p =0.040), and baseline use of antimalarials ( p =0.006). Conclusions In this 6-year follow-up study - thus far the largest study conducted -, bone loss was remarkably low. A dose-dependent relationship between glucocorticoid use and spine bone loss was found. In addition, use of antimalarials and lower 25-hydroxyvitamin D levels were associated with bone loss whereas use of immunosuppressive medication other than glucocorticoids was associated with bone gain at the spine. These findings underline the importance of prevention and treatment of vitamin D deficiency and osteoporosis in SLE, especially in patients using glucocorticoids or antimalarials. Moreover, the importance of treatment with immunosuppressive medication to improve disease-control and to reduce glucocorticoid dosages was highlighted. Disclosure of Interest None Declared