Angelica Gattamelata

The interobserver reliability of ultrasound in knee osteoarthritis

OBJECTIVE To assess the interobserver reliability between sonographers with different levels of experience in detecting inflammatory and structural damage abnormalities in patients with knee OA. METHODS After achieving consensus on definitions and scanning protocols, three ultrasonographers with different levels of experience in musculoskeletal US examined the knees of nine patients with OA. US examinations were conducted with independent blinded evaluations of inflammatory (joint effusion, synovial hypertrophy, power Doppler signal, Bakers cysts) and structural (osteophytes, cortical bone irregularities, femoral hyaline cartilage abnormalities, protrusion of the medial meniscus) lesions. All abnormalities were scored by applying a dichotomous scale (0-1). In addition, at each knee joint site global scores for joint inflammation, cortical bone abnormalities and cartilage damage were calculated by summing the single-lesion scores. Reliability was assessed using kappa (κ) coefficients. RESULTS Seventeen knees were examined. Inflammatory abnormalities were observed with moderate to very good agreement (κ = 0.55-0.88) between the observers. From fair to very good agreement (κ = 0.31-0.82) was registered between sonographers for structural damage lesions. The overall κ was 0.716 for junior and 0.571 for beginner sonographers comparing their findings with those of senior sonographers. CONCLUSION This represents the first ultrasonographic study focusing on the analysis of interobserver reliability between sonographers with different levels of experience in demonstrating inflammatory and structural abnormalities in knee OA. Globally, even considering some variable results that were mainly obtained by the evaluation of single components of bone involvement, US offered a reliable assessment of a wide set of abnormalities in knee OA.

Adult-onset still disease: A rare disorder with a potentially fatal outcome

PurposeThe aim of this study was to assess the clinical and laboratory features of a cohort of Italian patients with adult-onset Still disease (AOSD) with particular attention on possible life-threatening complications.MethodsThe clinical charts of 41 consecutive Italian patients with AOSD referred to our rheumatological department over the last 10 years were retrospectively examined. Data regarding clinical manifestations, laboratory features and complications were collected and compared with those reported in literature.ResultsThe most frequent manifestations were: fever (90.2%), arthralgias (80.4%), skin rash (75.6%), sore throat (53.6%), arthritis (51.2%), lymphadenopathy (48.7%), hepatosplenomegaly (41.4%), myalgia (21.9%), fatigue (12%), diarrhoea and vomiting (9.7%), pleural effusion (9.7%), pericardial effusion (4.8%) and abdominal pain (2.4%). In two patients whose cases are described in detail; the course of the disease was complicated by disseminated intravascular coagulopathy, in one patient with a fatal outcome. ESR, CRP and leucocyte count mean values were 69.41 mm/h, 69.05 mg/l and 18,798.5 cell/mm3 (neutrophils 84.64%), respectively. Serum ferritin levels were increased in 48.7% of patients while transaminases were elevated in 42.6% of patients (71% considering only patients in an active phase of disease).ConclusionThe results of this study are in line with those reported for other cohorts of patients. Even if the prognosis of AOSD is considered favourable, the present study indicates that the disease is a troubling condition needing prompt intervention. Occasionally, AOSD may rapidly worsen with life-threatening events.

The Italian MSUS Study Group recommendations for the format and content of the report and documentation in musculoskeletal ultrasonography in rheumatology

OBJECTIVE The objective of this study was to draw up a set of recommendations for the format and content of the musculoskeletal ultrasonography (MSUS) report in rheumatology. METHODS A panel of rheumatologists, members of the MSUS Study Group of the Italian Society of Rheumatology, met in order to identify the main discrepancies in the MSUS report. A set of 15 recommendations was then defined, aimed at resolving the main discrepancies. They consisted of information about the motivations for the MSUS examination, the equipment, the US modalities and scanning technique, a list of the examined structures and findings, the scoring/grading systems, the number of images and main findings to include and conclusions. Subsequently a Delphi-based procedure was started in order to obtain agreement on a core set of recommendations. Consensus for each recommendation was considered achieved when the percentage of agreement was >75%. RESULTS Three complete rounds were performed. The response rate was 85.2% for the first round, 78.3% for the second and 88.9% for the third. Finally, consensus was obtained for 14 of 15 statements. These 14 statements represent the recommendations of the group for the format and content of the report and documentation in MSUS in rheumatology. CONCLUSION To the best of our knowledge, our group has produced the first recommendations for the format and content of the report and documentation in MSUS in rheumatology. The report is an integral part of the MSUS examination and its use in a homogeneous form can help in the correct interpretation of the findings.

SAT0207 Correlation of clinical, serologic and histologic findings in a large cohort of primary sjögren’s syndrome patients: A multicentric cross-sectional study

Background Although primary Sjögren’s syndrome (pSS) is often a benign condition some patients develop systemic features. Identifying independent risk factors for pSS different clinical phenotypes would provide useful insights into patients’ assessment. Objectives to describe the clinical presentation of pSS in a large cohort of patients and to identify independent risk factors for severe disease manifestations. Methods Cumulative demographic, clinical, serological, histological and therapeutic data of 1115 pSS patients were retrospectively evaluated. Independent risk factors for disease manifestations were identified by logistic regression. Results The cohort included 1115 (1067 F:48M) pSS patients with a mean age of 51.6±13.8 yrs at diagnosis and 57.5±13.7 yrs at inclusion in the study (mean follow-up=5.8±6.5 yrs). Anti-Ro/SSA and anti-La SSB were detected in the 68% and 37% of the cases. Xerostomia (92%), xerophtalmia (94%) and articular involvement (61%) were the most commonly detected clinical manifestations. Salivary gland enlargement was detected in 346/1115 (31%) pSS patients while 475/1115 (42%) developed systemic extra-glandular involvement. Moderate to severe leukopenia (28%), peripheral arthritis (11%) and sensory-motor neuropathy (1.8%) were the most severe disease extra-glandular manifestations requiring immunosuppressive drugs. Independent risk factors for leukopenia were Raynaud’s phenomenon (RP) (p=0.008), low C3 (p=0.001), hypergammaglobulinemia (p=0.0006) and cryoglobulinemia (p=0.01). Arthritis was associated to RP (p=0.005) while sensory-motor neuropathy to purpura (p<0.0001), low C4 (p<0.0001) and cryoglobulinemia (p<0.0001). From the analysis, low C3/C4, hypergammaglobulinemia and cryoglobulinemia emerged as prognostic adverse laboratory abnormalities. Table 1 summarises patients’ clinical presentation according to the number of the laboratory abnormalities detected (“group A” = no abnormalities, “group B” =1 and “group C” ≥2 abnormalities). No factors 1 factor ≥2 factors p-value n=469 n=427 n=219 p-value Salivary gland enlargement 106 (22.6%) 150 (35.2%) 90 (41.1%) 0.000 Purpura 15 (3.2%) 35 (8.3%) 56 (25.7%) 0.000 Kidney 2 (0.4%) 12 (2.8%) 10 (4.6%) 0.001 Haematological manifestations 88 (19%) 152 (35.6%) 118 (54%) 0.000 Lung 20 (4.3%) 21 (4.9%) 19 (8.7%) 0.05 Peripheral nervous system 21 (4.5%) 18 (4.2%) 20 (9.1%) 0.01 Lymphoma 9 (1.9%) 10 (2.3%) 31 (14.2%) 0.000 Anti-Ro/SSA 239 (51.5%) 336 (79.4%) 187 (85.4%) 0.000 Anti-La/SSB 96 (20.7%) 200 (47.3%) 114 (52.1%) 0.000 Rheumatoid Factor 158 (36.3%) 264 (66%) 160 (73.7%) 0.000 Focus score ≥1 240 (77.9%) 226 (82.2%) 111 (88.8%) 0.03 Glucocorticoids 168 (36.3%) 166 (39.1%) 104 (47.9%) 0.01 Immunosuppressive drugs 42 (9.3%) 62 (14.7%) 69 (30.1%) 0.0001 Conclusions This study described the presentation of pSS in a large cohort of patients allowing us to identify independent risk factors which might help to predict the signs and symptoms of the disease. Patients with laboratory signs of B-cell hyperactivation should be more closely monitored since they may be at higher risk of severe extra-glandular involvement. Disclosure of Interest None Declared

Multicolor flow cytometric analysis of TLR2 and TLR9 expression and function in NK cells from patients with ANCA-associated vasculitis

The primary objective of this study was to provide an assessment of NK cells in patients with ANCA‐associated vasculitis (AAV).

FRI0426 Sjögren Syndrome & Systemic Lupus Erythematosus: Performance of the New Slicc Criteria in the Detection of True Overlap

Background Primary Sjogren Syndrome (pSS) and Systemic Lupus Erythematosus (SLE) express a significant overlap in their clinical and immunological aspects. Moreover, SS often presents clinical and immunological manifestations included in the SLE classification criteria. A secondary SS (diagnosis according to AECG criteria) has been described in about 9-19% of SLE cases. Few data is available regarding the prevalence of a true overlap of the two disorders. Objectives To investigate the prevalence of patients with pSS who can be classified as having an overlap syndrome with SLE considering both the 1997-revised ACR criteria and the new criteria proposed in 2012 by the Systemic Lupus International Collaborating Clinics (SLICC). Methods Charts from patients with pSS (diagnosis according to AECG criteria) were retrospectively evaluated in order to collect clinical and laboratory data. Results Two-hundred seventy patients with pSS [8M, 262 F, mean age 57.8±12,5 years, mean age at diagnosis 52.6 years ±12.5, mean follow-up 5.6 years (range 0–42 years)] were considered. Taking into account the 1997-ACR criteria, 11 patients (4.1%) could be classified as having an overlap with SLE (SS-SLE). Considering the new SLICC criteria, the number of patients raise to 18 (6.7%). Both considering the old and the new criteria, the most frequent items satisfied by the SS-SLE group were: ANA, leukopenia, oral ulcer and arthritis (Table). The items responsible for the increasing number of patients fulfilling the new criteria were low complement and the presence of leukopenia, thrombocytopenia or hemolytic anemia since these are considered separately in the new criteria. Compared to the remaining patients with pSS (246 F, 8M, mean age at diagnosis 53.3±12.4 years), the SS-SLE group (18 F, mean age at pSS diagnosis 47.9±13.1 years) showed a significantly higher frequency of leukopenia (77.7% in SS-SLE vs 14.2% in pSS; p<0.001), arthritis (44.4% in SS-SLE vs 10.3% in pSS; p<0.001) and low complement (38.8% in SS-SLE vs 7.14% pSS; p<0.001). Conclusions The occurrence of a true overlap between SS and SLE is relatively infrequent and the new SLICC criteria may allow a higher sensitivity to detect such condition. The items responsible for the increased sensitivity are both the introduction of hyopocomplementemia and the separate consideration of different hematological disorders in the new classification criteria. In patients with SS-SLE, hypocomplementemia, leukopenia and arthritis appear to be the most frequent manifestations. Disclosure of Interest None declared

SAT0305 Histology of minor salivary glands in patients with sjÖgren's syndrome, association with clinical and laboratory aspects

Background Minor salivary gland (MSG) biopsy represents an useful tool not only for the diagnosis of primary Sjogrens Syndrome (pSS) but also to evaluate patients prognosis. Recognition of germinal centers (GCs) by hematoxilin eosin (HE) and/or IHC staining for follicular dendritic cells (FDC) detection is mandatory, representing a risk factor for lymphoma development. Focus score (FS) is one of the main instrument to quantify MSG impairment, nonetheless quality information regarding the type of infiltrate such as the entity, structure and localization, are lacking. Objectives Aim of this study is to find any association of specific histological features of MSG from patients with pSS with the principal clinical and laboratory features. Moreover, to investigate the utility of histological parameters, other than FS or GCs, for characterizing patients. Methods Patients with pSS were enrolled in our SS clinic, and clinical/laboratory data (table) referring to the time of MSG biopsy, gathered on a dedicated database. MSG, removed for diagnostic purposes, were preserved as paraffin embedded tissue, then cut and sequentially stained by H&E and IHC [polyclonal rabbit anti-CD3 (lymphocytes T); monoclonal mouse anti-CD20 (lymphocytes B); monoclonal mouse anti-CD21 (FDC)]. Images were collected by Zeiss Axio Scan and analysed (ZEN software) as follows: FS calculation, mean foci area, percentage of infiltration, presence of segregated foci (SF) (specifically, clear evidence of T and B cells area by CD3-CD20 double staining), GCs and lymphoepithelial lesions (LELs) detection. Results 53 MSG from patients with pSS were collected and analysed. Patients clinical and laboratory data are reported in table. FS positively correlated with the percentage of infiltration (p<0.001) as well as with the presence of SF (p=0.005), GCs (p=0.02) and LELs (P=0.005). Mean foci area and percentage of infiltration correlated with SF (p=0.0002 and p<0.001, respectively), GCs (p=0.0004 and p<0.001, respectively) and LELs (both p<0.001). SF correlated with GCs and LEL (p<0.001). Anti nuclear antibodies (ANA) were associated with the presence of SF (p=0.029, OR=5.7 CI=1.1–28.8) while gland swelling was associated with the presence of GCs (p=0.043, OR=4, CI=1.1–15). Conclusions The FS was associated with the presence of GCs and LELs, as well as with more organized infiltrates characterized by segregation in T and B areas (SF), thus representing an useful tool which mirrors the risk of lymphoma. From our study, the qualitative characteristics of the biopsy, including SF, percentage of infiltration or the mean foci area, appear to be strictly linked. Moreover, their association with the presence of GCs and LELs supports the importance to consider also these features during histological examination. The lack of correlation between histological parameters and clinical/laboratory features might reveal a weaker connection between histological findings and specific SS phenotypes except for the relationship between glandular swelling and GCs which confirms how this clinical aspect should be considered as a risk factor for lymphoma development. Disclosure of Interest None declared

AB1044 Tenosynovitis in Patients with Rheumatoid Arthritis Detected by Ultrasound: Association with Active and Seropositive Disease

Background Ultrasonography (US) is an imaging tool able to evaluate patients affected by inflammatory arthropathies, thanks to its ability in detecting inflammatory and structural abnormalities and to its higher sensitivity compared with clinical evaluation. Several studies evaluated the role of US in diagnosis and follow-up of patients with Rheumatoid Arthritis (RA), mainly focusing on joint synovitis. Few studies evaluated specifically the inflammatory tendon involvement. Objectives We aimed to evaluate the prevalence and clinical significance of tenosynovitis detected by US in patients with RA with moderate/high disease activity naïve to biological treatment. Methods In this cross-sectional study, we enrolled patients affected by RA (1987 ACR criteria) candidate to start biological treatment. The patients underwent clinical, laboratory and ultrasonographic evaluation. Disease activity was evaluated using 28-joint DAS. Health assessment questionnaire (HAQ) was also administered. Bilateral high-resolution US at the level of flexor tendons of II and III finger and extensor tendon compartments at wrist level was performed. Moreover, II metacarpophalangeal (MCP), III MCP and radiocarpal (RC) joints were evaluated. Tenosynovitis, joint effusion (JE), synovial hypertrophy (SH) and PD were evaluated according to OMERACT definitions. All the lesions were graded according to a semi-quantitative scale ranging from 0 to 3 (0=absent, 1=mild, 2=moderate and 3=severe). Then, US inflammatory scores, calculated by adding the values given to each elementary lesion, were elaborated for every single joint or tendon. Finally, a global score was obtained by adding together the scores from each joint and tendon separately. Moreover, PD both at joint and tendon levels was evaluated also by using a dichotomous score (presence/absence). Results Forty-eight patients affected by RA were evaluated. In table the main clinical, laboratory and ultrasonographic features are summarized. Tenosynovitis was observed in 30/48 (62.5%) patients. Synovitis and erosions were identified in all patients. The presence of tenosynovitis was associated with high disease activity (defined as DAS28>5.1, P=0.015), with the positivity for rheumatoid factor (P=0.025) and with the presence of anti-CCP (P=0.023). Moreover, a positive correlation between the synovitis score and, respectively, the erosion score (P<0.0001, r=0.582), the DAS28 values (P=0.006, r=0.41) and rheumatoid factor ((P=0.001, r=0.37) was observed. The presence of erosions correlated also with the joint PD score (P<0.0001, r=0.507) and with disease duration (P=0.04, r=0.35). Conclusions We demonstrated that US-detected tenosynovitis is a relevant manifestation in RA patients. Indeed, it is associated with serologically positive patients and with higher disease activity. PD was confirmed to correlate with a more severe bone damage in terms of erosions observed by US. Disclosure of Interest None declared

SAT0609 Synovitis in Patients with Inflammatory Arthropathies: Comparison Between Rheumatoid Arthritis and Psoriatic Arthritis Evaluated by Power-Doppler Ultrasound

Background Power Doppler (PD) ultrasonography (US) is playing an increasing role in the evaluation of patients with inflammatory arthropathies, due to its ability in detecting inflammatory and structural abnormalities and the higher sensitivity compared with clinical evaluation. Several studies evaluated the role of US in diagnosis and follow-up of Rheumatoid Arthritis (RA) patients. A growing number of papers investigated the potential of US in the assessment of psoriatic arthritis (PsA). Indirect data suggested similar US findings in RA and PsA patients. However, to our knowledge, no study specifically designed to compare PDUS findings in RA and PsA patients has been performed. Objectives We assessed the ultrasonographic changes in terms of prevalence and severity of synovitis in clinically active RA and PsA patients. Methods In this cross-sectional study, we enrolled patients affected by RA and PsA with active peripheral joint involvement. The patients underwent clinical, laboratory and US evaluation. Disease activity was evaluated by the 28-joint DAS. We excluded all the patients in remission (i.e. DAS28<2.6). Bilateral high-resolution US at level of II metacarpophalangeal (MCP), wrist and knee was performed. Those joints were selected on the basis of an already published reduced-joint-count method (1). US-detected lesions [joint effusion (JE), synovial hypertrophy (SH) and PD] were evaluated with a dichotomous score (absence or presence) and then graded according to a semi-quantitative scale ranging from 0 to 3. US inflammatory scores, calculated by adding the values given to each elementary lesion, were elaborated for each joint. Finally, a total score was obtained by adding the scores from each joint. Results We enrolled 43 PsA (M/F 20/23; mean age 54.8±15.2 years; mean disease duration 112.6±70.8 months) and 70 RA patients (M/F 11/59; mean age 56.4±14.8 years; mean disease duration 158.7±40.4 months). DAS28 values resulted significantly higher in RA compared with PsA (5.3±1.1 versus 4.3±1.1, P=0.0001). US evaluation demonstrated a significantly higher prevalence of all US-detected lesion in RA patients compared with PsA (JE 60.9% versus 41.1%, P<0.0001; SH 54.8% versus 31.3%, P<0.0001; PD 28.8% versus 11.6%, P<0.0001). The US score in all joints as well as the total US score were significantly higher in RA than in PsA patients (Table 1). When analysing results according with disease activity defined by DAS28, RA patients showed a significantly higher global score compared with PsA in subjects with low (13.3±14.7 versus 1.5±2.1, P=0.03) and moderate disease activity (12.6±9.2 versus 7.4±6.7, P=0.01).Table 1. US score in RA and PsA patients (mean ± SD) PsA RA P Total score 5±6.5 13.1±9.8 0.0001 MCP score 1.2±2.2 2.6±3.8 0.0369 Wrist score 2.9±2.6 6.8±4.9 <0.0001 Knee score 2.7±3.7 3.7±4.0 0.1 Conclusions The present study demonstrate that PDUS synovitis is more severe in RA than in PsA patients, regardless of the disease activity. Concerning the prevalence of each US-detected abnormality, JE, SH and PD signal resulted significantly more frequent in RA patients than PsA. References Perricone C et al. The 6-joint ultrasonographic assessment: a valid, sensitive-to-change and feasible method for evaluating joint inflammation in RA. Rheumatology (Oxford) 2012;51:866-73. Disclosure of Interest None declared

FRI0250 Involvement of Salivary Glands in Primary SjÖGren's Syndrome. Elastographic Assessment and Correlations with Ultrasonographic Findings

Background Primary Sjögrens Syndrome (SS) is an autoimmune disease characterized by early inflammatory abnormalities of salivary and lacrimal glands and subsequent progressive destruction and local fibrosis. Imaging evaluation of salivary gland involvement in SS is still a matter of debate, with different tools that may be used. Ultrasound (US) has high sensitivity and specificity in the evaluation of parotid and submandibular glands involvement in SS (1). Elastography (UE) is a new technique that has been recently used for assessing skin and tendon elasticity in rheumatic diseases (2). However, as far as we know no study is present in the literature about elastographic assessment of salivary gland in SS. Objectives To evaluate the involvement of salivary glands in SS by UE and to correlate elastographic findings with US-detected abnormalities. Methods Salivary glands of consecutive SS patients and healthy controls were examined by UE and US by an experienced ultrasonographer who was blinded to the clinical findings. The study was performed with a MyLab70 XVG (Esaote,Italy) machine equipped with a multifrequency linear probe (4-13 MHz) and elastographic software. Parotid and submandibular glands were studied bilaterally by US firstly and graded according to a 0-4 score (1). Then, UE was carried out by sequential movements of compression and decompression of the probe over the region of interest and the resulting color assessment varied from red (indicative of soft and highly elastic tissue), to green (which represented intermediate elastic tissue), and to blue (which denoted hard and barely elastic tissue). Elastographic findings were scored according to the local color prevalence. The association between US and UE score was evaluated by the Spearmans correlation coefficient, and discrimination between cases and control by the area under the ROC curve (AUC). Results 328 salivary glands of 82 patients with SS [80 (97.6%) female; mean (sd) age 53.5 (12.3); median (IQR) disease duration in months 21 (1-192) and 116 glands of 29 aged matched healthy individuals [21 (72.4%) female; mean (sd) age 48.7 (13.5)] were studied. A significant correlation between the total UE and the US score was found (0.68; p<0.0001) and was mainly due to the blue component (green -0.62, p<0.0001; red 0.05, p=0.59; blue 0.65, p<0.0001). In addition, both US (AUC =0.961) and UE (AUC =0.817) scores were able to discriminate between SS patient and healthy controls. Conclusions UE is a valuable imaging tool in the assessment of salivary gland abnormalities in SS patients and may represent a good option as a first-line imaging modality in the diagnostics of the disease. References Salaffi F, Carotti M, Iagnocco A et al Ultrasonography of salivary glands in primary Sjögrens syndrome: a comparison with contrast sialography and scintigraphy. Rheumatology 2008;47:1244-9. Iagnocco A, Kaloudi O, Perella C et al Ultrasound elastography assessment of skin involvement in systemic sclerosis: lights and shadows. J Rheumatol 2010;37:1688-91 Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.3419