I. Janta

Patient self-assessment and physician’s assessment of rheumatoid arthritis activity: which is more realistic in remission status? A comparison with ultrasonography

OBJECTIVE The objective of this study was to compare disease activity assessed by the patient, the physician and musculoskeletal US in patients with RA in clinical remission. METHODS We evaluated 69 patients with RA in clinical remission according to their attending rheumatologist. Tenderness and swelling in 28 joints were blindly assessed by patients and physicians. The presence of B-mode and Doppler synovitis was blindly investigated in the above joints. The DAS28 and Simplified Disease Activity Index (SDAI) were calculated. RESULTS The percentage of patients in remission according to the self-derived DAS28 (26.1%) was significantly less than that according to the physician-derived DAS28 (52.2%) (P < 0.0005). There was no significant difference in the percentage of patients in remission according to the self-derived SDAI (14.5%) and the physician-derived SDAI (11.6%) (P = 0.172). We found moderate agreement between the patient-derived and physician-derived DAS28 and SDAI [intraclass correlation coefficient (ICC) = 0.620 and ICC = 0.678, respectively]. Agreement between patient and physician was better for the tender joint count (TJC; ICC = 0.509) than for the swollen joint count (SJC; ICC = 0.279). The mean (S.D.) count for B-mode synovitis [4.09 (3.25)] was significantly greater than the SJC assessed by both the patient and physician [2 (3.71) and 1.42 (2.03), respectively] (P < 0.0005 and P = 0.033, respectively). We found moderate agreement between the physician-assessed SJC and the joint count for Doppler synovitis (ICC = 0.528). CONCLUSION Patient-assessed and physician-assessed overall RA activity showed acceptable agreement. Patient self-assessment overestimated disease activity determined by the DAS28. At the patient level, physician-assessed joint swelling showed an acceptable concordance with Doppler US synovitis.

The 2017 EULAR standardised procedures for ultrasound imaging in rheumatology

Background In 2001, the European League Against Rheumatism developed and disseminated the first guidelines for musculoskeletal (MS) ultrasound (US) in rheumatology. Fifteen years later, the dramatic expansion of new data on MSUS in the literature coupled with technological developments in US imaging has necessitated an update of these guidelines. Objectives To update the existing MSUS guidelines in rheumatology as well as to extend their scope to other anatomic structures relevant for rheumatology. Methods The project consisted of the following steps: (1) a systematic literature review of MSUS evaluable structures; (2) a Delphi survey among rheumatologist and radiologist experts in MSUS to select MS and non-MS anatomic structures evaluable by US that are relevant to rheumatology, to select abnormalities evaluable by US and to prioritise these pathologies for rheumatology and (3) a nominal group technique to achieve consensus on the US scanning procedures and to produce an electronic illustrated manual (ie, App of these procedures). Results Structures from nine MS and non-MS areas (ie, shoulder, elbow, wrist and hand, hip, knee, ankle and foot, peripheral nerves, salivary glands and vessels) were selected for MSUS in rheumatic and musculoskeletal diseases (RMD) and their detailed scanning procedures (ie, patient position, probe placement, scanning method and bony/other landmarks) were used to produce the App. In addition, US evaluable abnormalities present in RMD for each anatomic structure and their relevance for rheumatology were agreed on by the MSUS experts. Conclusions This task force has produced a consensus-based comprehensive and practical framework on standardised procedures for MSUS imaging in rheumatology.

Multiobserver Reliability of Ultrasound Assessment of Salivary Glands in Patients with Established Primary Sjögren Syndrome

Objective. To evaluate the multiobserver reliability of salivary gland ultrasonography (SGUS) for scoring greyscale (GS) parenchymal inhomogeneity and parenchymal color Doppler (CD) signal in patients with established primary Sjögren syndrome (pSS). Methods. The study comprised 2 multiobserver reliability assessments in patients with pSS in 2 European centers. The first reliability exercise was performed on 24 patients with pSS and 8 controls who were independently evaluated with GS and CD US by 5 observers at the Institute of Rheumatology, Belgrade, Serbia. The second reliability exercise was carried out on 10 patients with pSS who were independently assessed with GS and CD US by 8 observers at the Hospital G.U. Gregorio Marañón, Madrid, Spain. SGUS parenchymal inhomogeneity and parenchymal CD signal were semiquantitatively scored using a 4-grade scoring system. The multiobserver agreement was calculated by the overall agreement and Light’s κ statistics. Results. A total of 640 SGUS examinations were performed in the first reliability exercise and a total of 320 examinations in the second reliability exercise. Multiobserver reliability was good (κ = 0.71–0.79) to excellent (κ = 0.81–0.82) for GS parenchymal inhomogeneity in both exercises. There was a moderate (κ = 0.53–0.58) to good (κ = 0.70) multiobserver reliability for parenchymal CD signal in the first exercise. However, there was no agreement or only a fair agreement (κ = 0.03–0.29) for parenchymal CD signal in the second exercise. Conclusion. US may be a reliable technique in the multiobserver scoring of GS parenchymal inhomogeneity of major SG in patients with established pSS. CD scoring of SG needs further standardization to be used in multicenter studies.

Structural damage in rheumatoid arthritis: comparison between tendon damage evaluated by ultrasound and radiographic damage

OBJECTIVE To compare structural damage assessed by conventional radiography and tendon damage assessed by musculoskeletal US (MSUS) at wrist and ankle in RA patients. METHODS We evaluated 72 consecutive patients [56 (77.8%) females] with RA. The MSUS evaluation consisted in a B-mode examination of bilateral extensor carpi ulnaris and tibialis posterior tendons. Tendon damage was defined and scored according to OMERACT. A total score for the tendon damage score (TDS) was calculated by summing the grades for each tendon. For the radiographic evaluations we used the van der Heijde score; a total radiographic score (RTS) was calculated by summing a bone erosion score (ERS) and a joint space narrowing score (JSNS). RESULTS We evaluated 288 tendons. The mean (s.d.) of TDS was 2.3 (1.8). Fifty-four (75%) patients presented tendon damage of at least one tendon. From all evaluated tendons, 134 (46.5%) had no tendon damage, 146 (50.7%) had grade 1 and 8 (2.8%) had grade 2 tendon damage. The mean (s.d.) for RTS was 91.4 (97), for ERS was 47.3 (61.9) and for JSNS was 44.1 (37.2). We found a significant correlation between disease duration and both TDS and RTS (r = 0.413 and r = 0.560, respectively; P < 0.0001). We found a good significant correlation between TDS and all variables of radiographic structural damage (RTS, r = 0.65; ERS, r = 0.637; JSNS, r = 0.618; P < 0.001). CONCLUSION The MSUS assessment of only four tendons can be an additional feasible method to assess structural damage in RA patients.

Three-dimensional Doppler ultrasound findings in healthy wrist and finger tendon sheaths - can feeding vessels lead to misinterpretation in Doppler-detected tenosynovitis?

BackgroundThe aim was to investigate the presence of feeding vessels in or in close proximity to extensor and flexor tendon sheaths at the wrists level and in finger flexor tendon sheaths in healthy controls, using 3D ultrasound (US), which may cause pitfalls, in order to ensure correct interpretation of Doppler signals when diagnosing tenosynovitis.MethodForty healthy participants (20 women and 20 men age 23-67 years) without prior history of arthritis, tendon diseases or present pain in their hands were included. Twenty participants had 3D Doppler US of the second and third finger and twenty of the right wrist. US was carried out using a GE Logiq E9 unit with a 3D US probe. The colour Doppler settings were to published recommendation.ResultsThe feeding vessels in or in close proximity to the tendon sheaths were found in the flexor and extensor tendons sheaths at least once in each participant. No significant difference in feeding vessels was seen between the radial and carpal level in the wrist (p = 0.06) or between the second and third flexor tendon sheath (p = 0.84).ConclusionDoppler findings in or in close proximity to the tendon sheaths were common in wrists and fingers in healthy participants. These feeding vessels can be a source of error, not only due to their presence but also because they may be interpreted as being inside the tendon sheath due to blooming and reverberations artefacts. These vessels should be taken into consideration when diagnosing Doppler tenosynovitis.

SAT0509 Clinical and Serological Profile of Children with Positive SSA-Ro/SSB-La Antibodies

Background Several studies have shown the relationship between anti-SSA-Ro/SSB-La antibodies and Systemic Lupus Erythematosus (SLE), Sjögren Syndrome (SS) and other autoimmune diseases in adult population. However, the expression of these autoantibodies and clinical correlation in juvenile patients is poorly described. Objectives To characterize the clinical and serological profile and primary rheumatic diseases in pediatric patients with positive anti-SSA-Ro and/or anti-SSB-La antibodies. Methods The data was obtained from a long term prospective cohort of patients under age 18 diagnosed with rheumatic diseases in a tertiary hospital in Spain. Demographic, clinical, and laboratory data were collected from 1986 to 2010. Patients were divided into 2 groups: anti-SSA-Ro/SSB-La positive and anti-SSA-Ro/SSB-La negative. Results A total of 187 patients were tested for anti Extractable Nuclear Antigens (ENA), with a following mean time of 11 years. Mean age at disease onset was 12.6 years and 77% were female. Fifty-four (28.9%) anti-SSA-Ro/SSB-La positive subjects were compared against 133 (71.1%) anti-SSA-Ro/SSB-La negative subjects. Among positive cases, 13 (24.1%) patients were double-positive for anti-SSA-Ro and anti-SSB-La, 51 (94.4%) were positive for anti-SSA-Ro and 3 (5.5%) were single-positive for anti-SSB-La. The anti-SSA-Ro/SSB-La antibodies were found less frequently (p=0.003) in the overlapping syndromes, and more frequently in SLE (p=0.007). In addition rheumatoid factor (p<0.001), anti-Sm (p<0.001) and anti-RNP (p<0.001) were frequently co-expressed with anti-SSA-Ro/SSB-La antibodies. Finally the anti-SSA-Ro/SSB-La positive group presented more hematological and skin manifestations than the negative group (p<0.05). Conclusions Similarly to adults, we observed a relationship between anti-SSA-Ro/SSB-La antibodies and SLE in pediatric patients. However a low proportion of childhood primary SS exists in our anti-SSA-Ro/SSB-La positive cases. This could be explained by underdiagnoses related to the atypical clinical presentation of SS in pediatric population. Single-positive anti-SSB-La patients are uncommon, the clinical significance of this serological result remains uncertain in children. References Rheumatol Int (2014) 34:1123–1127. Disclosure of Interest None declared

AB0668 Treatment of thrombotic events in behÇet disease: a systematic literature review

Background Behçet’s disease (BD) is a systemic disease which etiopathogenesis is largely unknown. It is characterised by a wide variety of clinical manifestations. Venous disorder is a serious manifestation being potentially life-threatening. There is little evidence on the management of the venous complications in BD. Objectives To perform a systematic literature review on the treatment used in venous thrombotic events in BD. Methods The objective was reformulated according to the PICO approach. Several synonyms for the main components (i.e. Behçet, thrombosis, treatment) were used. Search limits were applied for humans. The literature search was performed in Medline and Embase from databases inception to 1st November 2017. Only articles in English and Latin languages were retained. We excluded abstracts, reviews and letters. From the selected studies, data about the venous involvement and treatments were retired using a predefined data collection form.Abstract AB0668 – Table 1 Treatment N° articles (%) Anticoagulant 22 (78.6) Antiplatelet 9 (33.3) Corticosteroids 22 (78.6) Immunosuppressive 25 (89.3) Cyclophosphamide 16 (57.1) Azathioprine 16 (57.1) Cyclosporine A 5 (17.9) Mycophenolate mofetil 2 (7.1) Interferon alpha 2 (7.1) Methorexate 4 (14.3) Hydroxychloroquine 1 (3.6) Anti-TNF alpha 4 (14.3) Colchicine 11 (39.3) Thalidomide 1 (3.6) Dapsona 1 (3.6) Fibrinolytic 3 (10.7) Surgery 7 ((25.9) Results The literature search resulted in 1552 articles, of which 632 were captured in Medline and 920 in Embase. Figure 1 shows the study flow-chart for article selection. The main reasons for article exclusion after full-text review were the lack venous involvement and the lack of explanation of venous involvement treatment. 28 articles reporting 1904 patients were included in qualitative analysis. The mean (range; SD) duration time between the disease onset and the vascular onset was evaluated in 15 articles and was 4.9 (1.2–9.3; 2.7) years. Superficial thrombosis was evaluated in 6 (21.4%) articles, profound thrombosis in 19 (67.9%) articles, cerebral in 7 (25%), inferior or superior cava vein in 15 (53.6%) and Budd-Chiari syndrome in 8 (28.6%) articles. Table 1 shows the treatments described in the selected articles. Treatment response was evaluated in 20 (71.4%) articles; in 7 of these treatments response was evaluated in a subjective way. In total, 52 (2.7%) deaths were reported in relation to BD. In 319 (16.7%) patients, partial efficacy or recurrence of thrombosis was reported. Considering the heterogeneity of the reported data and the variability in the measures of treatment response, predictors of mortality risk cannot be analysed. However, in the reviewed articles, a higher mortality rate was observed in in patients with hepatic involvement due to Budd-Chiari syndrome. We have also observed a higher risk for the development of venous thrombosis in patients with patergia phenomenon and male sex. Two studies suggested that immunosuppressive treatment concomitant with anticoagulant treatment is associated with a lower risk of thrombosis relapse compared with anticoagulant treatment alone.Abstract AB0668 – Figure 1 Conclusions There is a great variability in the treatment of venous thrombosis related to Behçet’s disease. Budd-Chiari syndrome seems to be related to a worse prognosis of the disease. Disclosure of Interest None declared

Hemophagocytic lymphohistiocytosis in a patient with Sjögren's syndrome: case report and review.

Hemophagocytic lymphohistiocytosis (HLH) is a very rare syndrome with a mortality up to 95% of cases if not treated. It is characterised by an excessive activation of the immune system that leads to a disproportionate and destructive inflammatory response. The high mortality rates are in part due to a delay in the diagnosis, and therefore clinicians must maintain a high index of suspicion. When the treatment is started early, the survival rate reaches around 55% of cases. HLH usually presents with persistent fever, pancytopenia, and organomegaly and is associated with very high levels of serum ferritin. In this manuscript, we present the case of a patient with primary Sjögrens syndrome who developed HLH after an acute infection by Cytomegalovirus. We will describe and discuss the pathogenesis, differential diagnosis and a pragmatic approach to the treatment for this critically important and, when diagnosed early, potentially curable syndrome.

THU0474 A cross-sectional study into the effectiveness of the fibromyalgia rapid screening tool for detecting fm in patients with chronic arthritis undergoing full and tapered biological disease-modifying antirheumatic drug therapy

Background The determination of fibromylagia (FM) in patients presenting diffuse, chronic arthritis is fraught. The Fibromyalgia Rapid Screening Tool (FiRST) is a validated questionnaire with high sensitivity and moderate specificity shown to be able to identify up to 89% of FM cases, even when accompanied by anxiety, depression or functional disability. Decisions to embark upon a course of full or tapered biological disease-modifying antirheumatic drugs (bDMARD) are influenced in part by patient self-assessment scores as well as concomitant pathologies. Objectives To evaluate the prevalence of FM using the FiRST questionnaire in bDMARD-treated chronic arthritis patients. Methods This cross-sectional study included 325 patients [178 (54,8%) females and 147 (45,2%) males] diagnosed with chronic arthritis and treated with bDMARD. Patients were consecutively recruited from the Biological Therapy Unit from January to March 2015 all having undergone full or tapered bDMARD for at least 1 year. Dosage tapering had been applied to patients considered to be in remission. All patients self-completed the FiRST questionnaire with a score>5/6 considered positive. Clinical assessment was carried out by one specialist only. Demographic, clinical and laboratory variables were recorded with pathology-specific indices used to assess disease status, i.e.DAS28-ESR, DAS28-CRP, SDAI, CDAI, BASDAI, BASFI, ASDAS-CRP. Patient pathologies were classified as peripheral arthritis (PerAR: RA, PsA, PerSpA) or axial spondyloarthropathy type (AxSpA). Results A total of 68/325 (21%) patients scored >5/6 in the FiRST. Disease duration and previous bDMARD usage were not significant regarding scores <5/6. In the PerAR vs. AxSpA group, we observed that 19% (n=43) and 35% (n=25) scored FiRST>5/6, respectively (p=NS). Fifteen per cent of patients with tapered bDMARD registered scores >5/6 against 85% of patients in full bDMARD dosage (p=0.001). There were a higher number of remission patients in the PerAR group as defined under DAS28-ESR, SDAI and CDAI [(96%, 94% and 94%) (p=0.01, p=0.04, p=0.032), respectively]. Association was found in the PerAR subgroups between tapered bDMARD and remission in RA patients only, as defined under DAS28-VSG, SDAI and CDAI (p=0.026, p=0.04, p=0.043, respectively). In the AxSpA tapered bDMARD subset, 86% of patients were considered to be in clinical remission as set out under BASDAI (p=0.019). Conclusions No difference was observed between the PerAR and AxSpA groups for FiRST>5/6. Fewer patients undergoing tapered bDMARD dosage recorded FiRST scores >5/6. Therefore, early identification of chronic arthritic patients presenting FiRST>5/6 may prove to be an important step in furthering understanding of clinical activity in diffuse arthritis as well as offering improved diagnostic and therapeutic outcomes to bDMARD-treated patients with possible concomitant FM. Disclosure of Interest None declared

Musculoskeletal ultrasound: an effective tool to help medical students improve joint inflammation detection?

AIM The objective of this study was to evaluate whether musculoskeletal (MS) ultrasound (US) can be useful in helping medical students to detect joint inflammation through physical examination. MATERIAL AND METHODS The study was performed by two groups of four 6th year medical students. None had received any previous training in the clinical examination of joints or the use of ultrasound. Students were put through a 5-session training programme on the clinical detection of either knee [group 1] or metacarpophalangeal (MCP) [group 2] inflammation. After an initial training session on physical examination of normal and inflamed joints, the students examined 170 joints from 41 patients attending the hospital outpatient clinic in 4 separate sessions. The same joints were assessed for synovitis with US with the ensuing data compared to that of the students and analyzed for concordance with Cohens unweighted kappa. RESULTS In total 60 knees [group 1] and 110 MCP [group 2] were evaluated. The agreement between the presence of arthritis detected by the students in the four sessions and the presence of synovitis detected by US improved from the session I to sessions III with a marked improvement in the last session. CONCLUSIONS MSUS may be an effective technique for helping students to acquire the ability to detect joint inflammation.