Sibel Bakirci Ureyen

Psoriatic Arthritis Sonographic Enthesitis Instruments: A Systematic Review of the Literature

Objective. As part of the Group for Research and Assessment of Psoriasis and Psoriatic Arthritis (GRAPPA) ultrasound working group, we performed a systematic review of the literature to assess the evidence and knowledge gaps in scoring instruments of enthesitis in psoriatic arthritis (PsA). Methods. A systematic search of PubMed, EMBase, and Cochrane databases was performed. The search strategy was constructed to find original publications containing terms related to ultrasound, enthesitis, spondyloarthritis (SpA) or PsA. Data extraction focused on the properties of the sonographic enthesitis instruments used in each study following components of the Outcome Measures in Rheumatology (OMERACT) filter: feasibility, test-retest reliability, construct validity as related to clinical assessment of enthesitis, biomarkers of inflammation and imaging of enthesitis by other modalities, discriminative validity, and responsiveness to treatment. Results. Fifty-one of 310 identified manuscripts were included. Only 1 scoring instrument of enthesitis was specifically developed and validated in patients with PsA. Only 18 (35%) of the studies involved patients with PsA, while the remaining studies focused on SpA. In PsA, construct validity was assessed using biomarkers and clinical examination in 1 (2%) and 11 (21.5%) of the studies, respectively, whereas no studies used imaging for the same purpose. Only 2 (4%) of the studies assessed discriminative validity in PsA. Responsiveness to treatment was assessed in 7 studies, none of which included patients with PsA. Conclusion. Although sonographic enthesitis scoring instruments have been developed for SpA, only a few have been validated in PsA. None of them passed the OMERACT filter in patients with PsA. Additional research is required before endorsing a specific instrument for the assessment of enthesitis in patients with PsA.

What does evidence-based medicine tell us about treatments for different subtypes of psoriatic arthritis? A systematic literature review on randomized controlled trials

Abstract Objective PsA is a heterogeneous disease with various subtypes of joint manifestations, which can affect the homogeneity of randomized controlled trials (RCTs). The aim of this systematic literature review was to evaluate the inclusion criteria, demographics and outcomes of RCTs to see whether the whole spectrum of PsA was represented. Methods Medline, EMBASE and Cochrane databases were screened for RCTs on the efficacy of any treatment for PsA up to 4 October 2016 to investigate the inclusion criteria, demographics, outcomes and efficacy. Results Two thousand and sixty-eight abstracts were identified at screening; 76 articles and 52 conference proceedings were included in the final analysis. The main inclusion criteria always included the number of active joints and never axial symptoms, enthesitis nor dactylitis. Only 10 studies provided information about subtypes, of which symmetrical polyarthritis was the main subtype. Mean (s.d.) tender and swollen joints were between 7.8 and 35.8 (1.8–22.1) and between 5.2 and 25.2 (1.5–16.2), respectively. All studies had responses in joint counts as their primary outcome. Responses in enthesitis and dactylitis were usually secondary or tertiary outcomes. Response in BASDAI was among the outcomes in four studies. The comparison of efficacy in polyarticular vs oligoarticular disease was given in three studies, whereas no information was available for DIP joint disease or arthritis mutilans. Conclusion There is evidence in the literature to guide clinicians on how to treat PsA patients with polyarticular disease, but there is a gap in knowledge about the other subtypes. Protocol registration The study protocol is registered at PROSPERO (CRD42017053907).

Similar subclinical enthesitis in celiac and inflammatory bowel diseases by ultrasound suggests a gut enthesis axis independent of spondyloarthropathy spectrum

Objective Higher subclinical enthesitis on US has been reported in IBD and celiac disease, separately. The objective of this study was to compare IBD and celiac disease for enthesitis on US. Higher enthesitis scores in IBD compared with celiac disease would support a shared pathogenic mechanism between IBD and spondyloarthritis, whereas similar scores may suggest a general impact of gut inflammation on the enthesis. Methods Patients with IBD, celiac disease and healthy controls (HCs) were recruited and 12 entheses were scanned by US, blind to the diagnosis and clinical assessment. Elementary lesions for enthesitis were scored on a scale between 0 and 3, for inflammation, damage and total US scores. Results A total of 1260 entheses were scanned in 44 patients with celiac disease, 43 patients with IBD and 18 HCs. The three groups were matched for age and BMI. Patients with celiac disease and IBD had higher inflammation scores than HCs [10.4 (6.5), 9.6 (5.4) and 5.6 (5.2), respectively, P = 0.007) whereas damage scores were similar. Both age and BMI had significant effects on the entheseal scores, mostly for inflammation scores but when controlling for these the US enthesopathy scores were still higher in celiac disease and IBD. Conclusion The magnitude of subclinical enthesopathy scores is similar between celiac disease and IBD in comparison with HCs. These findings suggest that the common factor between both diseases and enthesopathy is abnormal gut permeability, which may be modified by the genetic architecture of IBD leading to clinical arthropathy.

The microvascular and morphostructural changes of nails in psoriatic patients with nail disease; a link between ultrasound and videocapillaroscopy findings in the nailfold

OBJECTIVE The objective of this study is to evaluate the link between nail fold vessel resistive index (NVRI) measured by ultrasound (US) and capillary loops diameters measured using nailfold videocapillarascopy (NVC), and to assess the morphological appearance of the nail bed in patients with psoriatic nail disease (PND) as compared with healthy controls (HCs). MATERIAL AND METHODS This study was conducted in patients with PND and HCs. General demographic data were collected and clinical assessments were performed for all subjects. The nail plate thickness (NPT) was measured on gray scale using US. The NVRI was measured using color Doppler (CD) US. The measurements of the apical, arterial, venous limb diameters and morpho-structural changes (tortuous, cross-linked capillaries) were assessed using NVC. RESULTS Thirty-four patients with PND and 15 HCs were enrolled in this study. The two groups were matched for age and body mass index (BMI). Patients with PND had higher NPT and NVRI in comparison with HCs [(20 (17-23) vs 14 (14-15), p<0.001), (0.55 (0.51-0.61) vs 0.43 (0.38-0.49), p<0.001), respectively]. A higher proportion of patients with PND had tortuous capillaries than HCs (62% and 20% respectively, p=0.005). The mean NVRI was higher in patients with PND who had tortuous capillaries than patients who did not have tortuous capillaries (0.58 (0.7) and 0.52 (0.09), respectively p=0.033). CONCLUSION Microvascular changes can be detected easily using non-invasive methods such as US and NVC. These methods can provide an objective data to better assess PND.

Axial psoriatic arthritis: the impact of underdiagnosed disease on outcomes in real life

Psoriatic arthritis (PsA) may affect different joints, including the spine. The prevalence of spinal involvement is variable depending on the definition and a subset of patients have been identified in cohorts that do not have clinical features of axial disease and yet have imaging findings. Still, there is not a consensus on how and when to screen axial disease. In this study, we aimed to investigate factors associated with being underdiagnosed for axial psoriatic arthritis (axPsA) and its impacts on outcomes. Disease features and outcomes of axPsA according to the physician (n = 415) were compared with patients with imaging findings only (sacroiliitis fulfilling the modified New York criteria, n = 112), using data from a real-life PsA registry. Patients with imaging findings only were more frequently women (83/220 (37.7%) vs 29/122 (23.8%); p = 0.008). This group also had higher peripheral disease activity (imaging only vs clinical AxPsA: mean (SD) tender joint count 5.3 (6.1) vs 3.3 (4.7), swollen joint count 1.9 (2.9) vs 1.2 (2.4); p < 0.001 for both comparisons) and was less often treated using TNF inhibitors (16.1 vs 38.2%; p < 0.001) than patients who were classified as axPsA. Patient-reported outcomes were similar in both groups. PsA patients, especially women with more severe peripheral disease, have a higher risk of being underdiagnosed for axPsA. The severity of peripheral symptoms may be a risk factor to mask the spinal features of PsA.

SAT0353 Geographical differences in psoriatic arthritis: a transatlantic comparison

Background The environmental and genetic factors play a crucial role in the pathogenesis of psoriatic arthritis (PsA) which may cause a difference in disease characteristics for patients from different geographical regions. Objectives The aim of the study was to explore the disease characteristics, treatment choices and comorbidities in patients with PsA in different countries to see the impact of geographic factors. Methods PsArt-ID (Psoriatic Arthritis- International Database) is a prospective, multicentre registry in PsA, which was initially developed in Turkey in 2014, with participation of Canada since 2015 and Italy since 2017. Patients with PsA are consecutively registered to this registry with the aim of investigating the real-life data. Patient characteristics across Turkey (n=1283) and Canada (n=119) are compared for this analysis.Abstract SAT0353 – Table 1 The demographics and clinical characteristics in two countries TURKEY CANADA p value Female* 827/1283 (64.5) 60/119 (50.4) 0.002 Age (years) 47 (36–56.7) 49 (34–61) <0.001 BMI (kg/m2) 27.47 (24.5–31.2) 29 (23.7–33.5) 0.013 At onset age for PsA 36 (29–49.7) 39 (30–48) 0.058 Smoking (package/years) 10 (3–19.7) 14.5 (5–26.25) 0.007 Education years 8 (5–12) 15 (13–16) <0.001 SJC 2 (1–5) 2 (1–7) 0.461 TJC 4 (2–8) 6.5 (2–17) 0.340 TEP 2 (1–2) 1 (1–2) 0.021 BSA 5 (1–13.75) 1 (0–5) <0.001 BASDAI 37 (20–54) 38 (22–58) 0.027 Pt GA 45 (20–60) 31 (12–70) <0.001 PGA 30 (20–50) 34 (18–66) <0.001 Pain VAS 40 (20–60) 33 (18–78) <0.001 TJC: tender joint counts; TEP: tender entheseal points; BSA: body surface area; PtGA: patient global activity; PGA: physician global activity. All data were given n/total n (percentage (%))* or median (first-third percentiles).Abstract SAT0353 – Figure 1 The distribution of the treatment choices in Turkey and Canada, excluding patients with new diagnosis at the time of recruitment. DMARD: Disease-modifying anti-rheumatic drug; anti-TNF: anti-tumour necrosis factor. All data were given n/total n (percentage (%). Results Canadian patients were older at the time of recruitment (Table). They also were more frequently smokers, had higher duration of education and higher BMI than patients in Turkey. Patients in Canada had more frequent polyarthritis (66.7% vs 39.6%, p<0.001), DIP joint disease (34.2% vs 16%, p<0.001), dactylitis (38.1% vs 29%, p=0.037) nail involvement (55.9% vs 45.7%, p=0.008) and higher number deformed joints (29.3% vs 20.7%, p=0.035) whereas Turkish patients had oligoarthritis more often (37.6% vs 24.8%, p=0.016). For disease activity, tender and swollen joint counts were similar for whereas the skin activity was higher in Turkish patients. There were no major differences between countries regarding treatment choices with similar frequencies of patients on biologic therapies (34.5% vs 30.2%, p=0.339) (figure 1). Although the numbers were very low, there was more frequent cancer in Canada than Turkey (4.3% vs 1.4%, p=0.022) whereas all the other comorbidities were similar. Conclusions Geographical differences have impacts on the disease features in PsA, which may be due to genetic, environmental and cultural differences. The treatments are comparable suggesting a similar approach by the physicians. Disclosure of Interest None declared

SAT0063 A prospective study on comparison of composite indices with ultrasound for detecting remission and prediction of flare in 2 years

Background Treat-to-target (T2T) approach suggests using a composite index when following patients with rheumatoid arthritis (RA) without identifying which one to use. Objectives In this prospective study we aimed to compare the accuracy of different indices for RA patients in remission taking Ultrasound Global Synovitis Score (GLOESS) as a gold standard and their predictive value for flares in 2 years. Methods RA patients who were considered to be in clinical remission according to the clinician were recruited. Disease activity was assessed using DAS28-CRP, CDAI, SDAI and RAPID-3 and 38 joints per patient were scanned by US and scored according to GLOESS. The total GLOESS scores were calculated for 38 joints and also for 28 joints by excluding the MTP joints. The number of joints with ≥2 GLOESS was calculated. Flare data was collected in 3 subsequent visits in the following 2 years, whenever available. Results Ninety-six consecutive patients (80.2% females) were recruited. Patients were more frequently categorized as being in remission using DAS28 (80%) compared to CDAI (50%), SDAI (45.2%) and RAPID 3 (37.5%). Patients that were in remission according to CDAI had lower GLOESS scores on 28 joints (p=0.05) and had less joints with ≥2 signals (p=0.04) (table). For SDAI patients in remission had significantly less number of joints with grade 3 signals (p=0.03) and tend to have lower GLOESS scores on 28 joints as well as lower number of joints with ≥2 signals (p=0.06). None of the US scores were able to differentiate different disease states according to DAS28-CRP or RAPID. Flare data was available in 76 patients, 22 of whom had flares. Patients that had flare had higher GLOESS scores on 28 joints at baseline (p=0.05) and tend to have higher number of joints with grade 3 signals (p=0.06). Although numerically higher, none of the clinical indices were able to predict flares based on remission status (remission vs non remission: CDAI: 22.5% vs 36.1, p=0.2; SDAI: 22.9 vs 36.8, p=0.2; DAS28: 25.4% vs 50%, p=0.1; RAPID3: 20% vs 34.8%, p=0.2).Table 1. The distribution of US-GLOESS according to different categories by indices GLOESS 38 joints GLOESS 28 joints Joint counts ≥ score 2 Joint counts with score 3 mean±SD mean±SD mean±SD mean±SD DAS-28  Remission (n=77) 10.9±7.5 7.8±7.2 3.5±3.1 0.7±1.3  LDA (n=11) 14.5±6.6 10.0±5.4 4.9±3.0 0.9±1.4 VMDA (n=5) 9.6±6.2 5.4±3.5 3.0±2.3 0.80±1.7 CDAI  Remission (n=48) 10.0±6.9 6.9±6.2 3.02±2.7 0.60±1.0  LDA (n=48) 12.7±7.7 9.1±7.2 4.3±3.3 0.94±1.5 SDAI  Remission (n=42) 9.9±6.6 6.6±5.9 2.9±2.6 0.5±0.9  LDA (n=50) 12.4±7.8 9.1±7.5 4.2±3.3 0.9±1.5  MDA (n=1) 13.0 4.0 5.0 0 RAPID  Remission (n=36) 10.3±6.2 6.7±4.6 3.1±2.6 0.5±0.8  Low activity (n=28) 9.6±5.8 6.6±4.6 2.7±2.2 0.3±0.7  MDA (n=30) 14.0±9.4 10.7±9.7 5.1±3.9 1.4±1.9  HDA (n=2) 15±1.4 8.5±2.1 5±1.4 0 LDA: Low disease activity MDA: Moderate disease activity HDA: High Disease activity. Conclusions Our results show that CDAI is superior then other clinical indices to assess remission in RA. US has the superiority over clinical indices to predict flares and 28 joint GLOESS is superior to 38 joints. Disclosure of Interest None declared

İntestinal Behçet hastalığı mı? Crohn hastalığı mı? Olgu sunumu ile literatürün gözden geçirilmesi

Crohn hastaligi ve Behcet hastaliginin intestinal tutulumunun ayirici tanisi her zaman kolay olamamaktadir. Otuz uc yasinda kadin hasta, on planda yaygin eklem sikâyetleri ile romatoloji poliklinigine basvurdu. Uzun suredir devam eden anemi ve gastrointestinal yakinmalarinin aydinlatilmasi asamasinda, endoskopide derin ulserler gozlendi. Olgumuz; Behcet hastaligi intestinal tutulumu ve Crohn hastaligi ayirici tanisi ayrintili degerlendirmelerle yapildiktan sonra, “Enteropatik Artrit” tanisi aldi. Eklem sikâyetleri ile basvuran hastalarda sistemik degerlendirmenin onemi ve eklem tutulumunun karakterinin iyi analiz edilmesinin taniya katkisi, bu vaka ile beraber bir kez daha vurgulanmistir. Bu derlemede ayni zamanda, Crohn hastaligi ve Behcet hastaligi intestinal tutulumunun ayirici tanisi, gastrointestinal, ekstra intestinal bulgulari ve endoskopide gozlenen ulser ozellikleri acisindan ozetlenmistir.

Hemophagocytic syndrome associated with adult Still's disease

Uzm. Dr. Sibel Üreyen. Sakarya E¤itim ve Araflt›rma Hastanesi, ‹ç Hastal›klar› Romatoloji Bölümü, Sakarya. e-posta: bakircisibel@gmail.com Ç›kar çak›flmas› / Conflicts of interest: Ç›kar çak›flmas› bulunmad›¤› belirtilmifltir. / No conflicts declared. www.raeddergisi.org doi:10.2399/raed.16.44127 Karekod / QR code: Eriflkin Still hastal›¤› (ESH) akut bafllang›çl› sistemik inflamatuvar bir hastal›k olmakla birlikte etiyoloji ve patogenezi tam olarak bilinmemektedir. Nedeni bilinmeyen ateflin sebepleri aras›nda kollajen doku hastal›klar› %23 oran›nda görülmektedir. Kollajen doku hastal›klar›ndan da ESH en s›k sebebi bilinmeyen atefl (NBA) etkeni olarak sorumludur. Birçok sistemi etkileyen multisistemik bir hastal›kt›r ve baflta NBA’lar olmak üzere, di¤er hastal›klar d›flland›ktan sonra tan›s› konulabilmektedir. Hemofagositik sendrom (HFS) ise, yüksek atefl, sitopeni, hepatosplenomegali, hiperferritinemi, koagülopati ve kemik ili¤i ya da retiküloendoteliyal sistemde hemofagositoz ile karakterize klinik bir tablodur. HFS çok nadir olarak ESH’e efllik etmekle birlikte fatal seyredebilmektedir. Bu olgu sunumunda ESH tan›s› konularak tedavisi bafllanan ve kortikosteroid dozu komplikasyonlar› nedeniyle h›zl› azalt›l›rken takiplerinde nötropeni saptanmas› nedeniyle ileri tetkikler sonucu histopatolojik olarak HFS’nin efllik etti¤i ESH tan›s› konulan 35 yafl›nda kad›n olguyu sunmay› amaçlad›k.