Ramin Bazeli

Ultrasonographic hand features in systemic sclerosis and correlates with clinical, biologic, and radiographic findings

To investigate ultrasonographic (US) hand features in systemic sclerosis (SSc) patients and their relationship with clinical, biologic, and radiographic data.

Radiological cervical spine involvement in young adults with polyarticular juvenile idiopathic arthritis

OBJECTIVES Radiological cervical spine involvement in JIA has already been assessed with a large range of prevalence (5-80%), but most studies were performed a long time ago, in symptomatic JIA and without differentiating subsets of JIA. We set out to describe structural cervical spine involvement in young adults with polyarticular JIA (pJIA) regardless of the cervical symptoms and to compare lesions with those observed in adult RA. METHODS All consecutive pJIAs followed in a transition programme were included. Standard radiographs of the cervical spine, hands, feet and hip were analysed by two independent radiologists blinded to the diagnosis. An RA control group (<55 years), matched for sex and disease duration, was recruited. RESULTS Fifty-seven pJIA and 58 RA patients were included. Radiographs showed cervical lesions in 65% of pJIA and 67% of RA patients. In total, 51% of pJIA with radiographic abnormalities had no clinical symptoms. In pJIA, the most frequent structural lesions were anterior atlantoaxial subluxation (33%), erosion of the odontoid process (19%), C1-C2 arthritis (17%) and apophyseal joint arthritis (16%). Cervical lesions in pJIA were similar to those in RA except for ankylosis and hypotrophia (P < 0.05). The presence of cervical lesions correlated with a more severe disease. CONCLUSION Structural cervical spine involvement is common in pJIA persisting into adulthood, frequently asymptomatic and associated with a more severe disease. We suggest that radiographic assessment of the cervical spine should be done systematically at onset of the disease and regularly during its course regardless of clinical symptoms.

Hand and wrist involvement in systemic sclerosis: US features.

PURPOSE To characterize ultrasonographic (US) features in the hand of patients with systemic sclerosis (SSc) and to evaluate the sensitivity of US in the detection of calcinosis and acroosteolysis. MATERIALS AND METHODS The local ethics committee approved this study, and oral informed consent was obtained. A total of 44 consecutive patients with SSc (34 women; mean age, 56.1 years ± 12.1 [standard deviation]; 10 men; mean age, 45.0 years ± 14.0) and 30 healthy control subjects (20 women; mean age, 46.3 years ± 12.1; 10 men; mean age, 39.6 years ± 10.8) were included between October 2010 and December 2011. Bilateral US, including Doppler assessment of the wrists, hands, and fingers, was performed, and presence of synovitis, tenosynovitis with or without a layered appearance, calcifications, acroosteolysis, and distal vascularization was recorded. Radiography of both hands was performed to assess for acroosteolysis and calcinosis. Frequency of US features, sensitivity of US for calcinosis and acroosteolysis, and respective confidence intervals were calculated. RESULTS Synovitis was found in 17 patients (39%). Tenosynovitis was found in 12 patients (27%), and it had a layered pattern in 15 (41%) of 37 cases. Calcinosis was found in 17 patients (39%) with US, with a sensitivity of 89%. Acroosteolysis was found in nine (20%) patients with US and in 10 (23%) patients with radiography, with 90% sensitivity for US. Distal vascularization was detected in 26 patients (59%) and 30 control subjects (100%) and was in contact with the acroosteolysis bed in seven (78%) of nine patients with SSc. CONCLUSION US can be used to assess features of SSc, including synovitis, tenosynovitis, calcinosis, acroosteolysis, and distal vascularization and is sensitive for calcinosis and acroosteolysis detection. A layered pattern (similar to the appearance of an artichoke heart) of tenosynovitis was seen commonly. Online supplemental material is available for this article.

Incomplete thymic involution in systemic sclerosis and rheumatoid arthritis.

OBJECTIVE The thymus plays a crucial role in immune system homeostasis. Thymic abnormalities have been reported in many autoimmune diseases, but data for systemic sclerosis (SSc) and rheumatoid arthritis (RA) are sparse. The aim of this study was to evaluate the prevalence and correlates of radiological incomplete involution of the thymus in SSc and RA patients, and in a non-autoimmune group of controls. METHODS All patients were at least 40 years old: 96 SSc patients (median age 59 years, 80% women) and 65 RA patients (median age 57 years, 88% women) were compared with 32 control individuals (median age 63 years, 62% women). Pulmonary CT-scans performed for lung assessment were available for all individuals. For the purpose of our study, complete involution of the thymus was defined as the absence of a residual thymus or a gland thickness, corresponding to the short axis on the axial slice, of less than 7 mm. We defined incomplete involution of the thymus as a residual thymic tissue more than 7 mm thick. RESULTS The frequency of incomplete thymus involution was significantly higher in SSc and RA patients (respectively 15 and 14%) than in the control group (0%; P<0.05). Incomplete thymus involution was associated with pulmonary restrictive syndrome in SSc patients, and with biotherapy and an absence of antinuclear antibodies in RA patients. CONCLUSION Our findings show that two autoimmune diseases, SSc and RA, are associated with incomplete thymus involution.

IRM du rachis et des articulations sacro-iliaques dans la spondylarthrite ankylosante

MR Imaging of the spine and sacroiliac joints in ankylosing spondylitis The new diagnostic criteria for spondyloarthropathy include MRI. MRI frequently allows early diagnosis of inflammatory lesions of the spine and sacroiliac joints in patients with normal plain films. Moreover, MRI is useful for the detection and quantification of inflammatory and structural lesions, and to assess disease activity.

IRM et spondylarthropathie

Objectifs Connaitre le protocole IRM pour explorer une SPA. Connaitre la semiologie IRM d’une SPA. Savoir distinguer une IRM avec signes d’activite d’une IRM avec signes de maladie chronique. Messages a retenir L’apport de l’IRM est certain pour le diagnostic precoce, meme si cet examen peut etre negatif dans une vraie spondylarthrite. Les cohortes actuellement en cours de constitution, telle la cohorte nationale DESIR (DEvenir des Spondylarthropathies Indifferenciees Recentes), devraient, a terme, offrir un outil de travail susceptible d’evaluer de nouveaux algorithmes de diagnostic precoce, et de mieux preciser la place de l’imagerie en pratique courante. Resume Une IRM des sacro-iliaques doit etre realisee en sequence STIR (suppression de graisse) et en sequence Tl. L’injection intraveineuse de gadolinium n’est pas utile en pratique courante dans cette indication. L’IRM du rachis entier apporte des informations supplementaires et peut donc etre conseillee : il n’existe cependant pas de consensus a ce sujet, pour notre part nous conseillons cet examen. Il est important de preciser qu’il s’agit d’une recherche d’inflammation, afin que les sequences adaptees (STIR) soient realisees.

AB0885 Anti-Cyclic Citrullinated Peptide (ANTI-CCP) Antibodies in Polyarticular Juvenile Idiopathic Arthritis at Adulthood

Background Several studies have already focused on anti-cyclic citrullinated peptide (anti-CCP) antibodies in juvenile idiopathic arthritis (JIA). Results of these studies showed that anti-CCP were associated with polyarticular sub-type (pJIA) and more severe disease, especially for structural damages. However, these studies analysed global JIA without focusing on the polyarticular form and especially on rheumatoid factors (RF) positive pJIA. Objectives Determine the prevalence of anti-CCP and evaluate their clinical and radiographic significance in polyarticular JIA at adulthood. Methods Anti-CCP antibodies were determined in a large cohort of polyarticular form of JIA at adulthood. Following demographic and clinical data were collected: sex, age at diagnostic, medical treatments (corticosteroids, DMARDs and biotherapies), history of surgery, number of synovitis and C-reactive protein at the last medical visit. Rheumatoid factors (RF) and antinuclear antibodies (AAN) have been collected. Radiographic damages were evaluated by the Sharp Van Der Heijde score. Results 56 patients with polyarticular form of JIA, fulfilling the ILAR criteria, persisting at adulthood were included with mean age of 25±8.1 years and mean disease duration of 14.3±8.7 years. Antibodies status (anti-CCP, RF and AAN) was performed after a mean of 11.6±9 years after the diagnosis. 29/56 (52%) patients were anti-CCP positive. Anti-CCP positive pJIA had a significant association with presence of RF (97% vs 26%, p<0.0001), a higher use of biotherapies (86% vs 70%, p=0.02, OR=4.3 [1.2-15.8]) and were older at diagnosis (13±3.4 vs 7.6±4.9, p=0.00001) than anti-CCP negative patients. No differences have detected for sex, presence of antinuclear antibodies, synovitis, CRP, Corticosteroids, DMARDs and surgery. Radiographic damages were similar between the two sub-groups for erosion (16.2±21.3 vs 14.8±28.3), joint space narrowing (24.3±29.3 vs 23.8±34.9) and total scores (40.4±46.8 vs 38.7±61.8). In the sub-group of RF positive pJIA (n=35), 28 were anti-CCP positive and 7 negative. No differences for sex, clinical data, medical treatments, surgery and radiographic damages have been detected. Conclusions In this large cohort of polyarticular JIA patients at adulthood, anti-CCP antibodies are associated with a higher need of biotherapies but not with a more structural radiographic severe disease. A small sub-group of patients were RF+ and anti-CCP-. Comparison of RF+ and anti-CCP+ and RF+ and anti-CCP- patients showed that anti-CCP did not seem to bring more information as RF but a larger cohort will be necessary to confirm these results. Finally, our results suggest that polyarticular JIA patients evolving at adulthood are independent of anti-CCP antibodies status at the difference of the pJIA in childhood in which anti-CCP status remains a poor prognostic factor. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.5319

Assessment of hand involvement in systemic sclerosis by ultrasonography. Reply.

We thank Drs. Ingegnoli and Cuomo for their comments regarding our study of the potential role of US in the assessment of hand involvement in SSc. In this study, we observed that articular involvement is a common feature in SSc, with approximately half and approximately onethird of SSc patients exhibiting US synovitis and tenosynovitis, respectively. This articular involvement was strikingly underestimated by the single clinical examination. Regarding tenosynovitis, we observed that in almost half of the cases, the tendons with tenosynovitis were characterized by a hyperechoic tendon sheath thickening that we described as sclerosing tenosynovitis. This pattern was different from the standard tenosynovitis pattern that has been reported in the literature and was specific to SSc patients in comparison to rheumatoid arthritis patients. The pattern predominantly involved the extensor tendons and was associated with the clinical detection of TFRs. Ingegnoli and Cuomo pointed out that this could be related to an increased thickness of the retinacula by US, a thickening of the A1 pulley, or connective tissue infiltrates. Indeed, in another fibrotic disease (i.e., de Quervain’s tenosynovitis), the first compartment retinaculum rather than the tendon sheath is thickened, with an inflammatory involvement of the tendons in most of the cases (1). More recently, Cuomo et al detected increased thickness of the extensor retinaculum at the fourth compartment, which was closely associated with the detection of TFRs, suggesting that this was the lesion underlying the sign (2). We also evaluated the thickness of the common extensor retinaculum at the fourth compartment between 12 patients with tenosynovitis and 30 healthy controls, but we found no significant difference (mean SD 0.98 0.13 mm and 0.97 0.17 mm, respectively; P 0.77). Therefore, the retinaculum thickness seems to be not universally involved in the sclerosing pattern of tenosynovitis. Tagliafico et al recently reported a thickening of the A1 pulley in SSc patients that was correlated with hand mobility (3); however, we did not assess this anatomic structure in our study. Another hypothesis is that this sclerosing pattern might be related to fibrosis and particularly to fibrinous deposits on the surface of the tendon sheaths (4). Because of the clinical meaning of TFRs (5,6), further studies are warranted to better determine the anatomic structure of this sclerosing tenosynovitis, with particular attention to the A1 pulley and tendon sheaths, and to clarify the best way to assess tenosynovitis in SSc patients and determine its potential input to predict clinical outcomes.

THU0327 Radiological peripheral involvement at hands, feet and hips in young adults with polyarticular idiopathic juvenile arthritis

Background Radiographic damage was recently considered to be a feature of poor prognosis in cases of polyarticular juvenile idiopathic arthritis (pJIA). However, most radiographic studies did not differentiate pJIA from other subtypes of JIA and did not include a control group. Furthermore little is known about radiographic damages in pJIA persisting into adulthood. Objectives To describe structural peripheral involvement in pJIA persisting into adulthood and compare observed lesions to those seen in rheumatoid arthritis (RA) using a cross-sectional observational study. Methods All consecutive pJIA followed in a transition program were included. Age, sex, disease duration, medical or surgical treatments were collected. Laboratory tests (ESR, CRP, Rheumatoid Factor (RF) and anti-CCP) and standard radiographies of the hands and wrists, feet and hip were performed. A RA control group (<55 years), matched for sex and disease duration, was recruited. Radiographs were analyzed by two independent radiologists blinded to the diagnosis. Structural lesions on the hands and feet were assessed by the modified version of Larsen’s scoring method. The hands and feet scores range from 0 to 110 and from 0 to 50, respectively. Hips were assessed for presence of coxitis. Student and Fischer exact test were used. Results 58pJIA (48 females/10 males) and 59 RA (52/7) were included. Respectively, mean age was 23.5±10.0 years and 43.2±9.6 years and mean disease duration 13.1±11.1 and 12.2±7.1 years. 60% and 80% were RF positive and 57% and 78% were anti-CCP positive (p=0.02). The inter-observer concordance coefficient kappa was 0.614 between the two investigators. Four pJIA patients had previous hand or feet surgery and 7 in RA group avoiding radiographs evaluation. Radiographs showed hand lesions in 41/54 (76%) pJIA and 42/50 (84%) RA-patients, feet lesions in 35/54 (65%) pJIA and 45/57 (79%) RA-patients and coxitis in 16/54 (30%) pJIA and 8/47 (17%) RA-patients (p=NS for all comparisons). Mean hands and feet scores were 17.9±21.8 and 7.7±10.8 in pJIA and 18.5±17.6 and 9.9±11.3 in RA, respectively (p=NS). Coxitis was bilateral in 13/16 (81%) in pJIA and in 2/8 (25%) of the cases of RA-patients, respectively (p=0.005). Hand damages involved wrists, metacarpophalangeal, proximal interphalangeal and distal interphalangeal joints in pJIA without statistical significant difference when compared to RA. RF-positive patients differed from RF-negative patients only by a shorter disease duration (10.4±9.3 vs. 17.3±12.6, p=0.02). Radiographic comparison between RF-positive and RF-negative patients revealed a higher frequency of hand damage (29/34 vs. 12/21) and less coxitis (5/31 vs. 11/23) in RF-positive subgroup (p=0.02 and 0.01, respectively). In pJIA, presence of radiographic damage correlated with a more severe disease phenotype: higher frequencies of biotherapies and surgery (p=0.009 and p=0.02, respectively), but not with RF status. Conclusions Structural peripheral damages are frequent in young adults with pJIA and correlated with a more severe disease. A specific feature to pJIA seems to be a high risk of bilateral coxitis. This requires a particular following and monitoring of pJIA patients with unilateral hip involvement to prevent bilateralization. Disclosure of Interest None Declared

Radiological cervical spine involvement in polyarticular idiopathic juvenile arthritis

erosions of the odontoid process (34%) and atlanto-axial impaction (20%). Apophyseal joint ankylosis (AJA) was rare (10%) but involvement of the posterior cervical spine (AJA and apophyseal joint arthritis) was more frequent (42%). Presence of radiographic cervical lesions was correlated in univariate analysis with a more severe disease phenotype: more erosive disease (hand and feet), higher number of biotherapies per patient (respectively 1.4±1.3 vs 0.5±0.5, p=0.003), and higher frequency of surgery (p=0.03, OR 7.74[0.92-65]) but not with RF status. Multivariate analysis confirmed only the association with the higher number of biotherapies per patients (p=0.04). 50% of pJIA with radiographic abnormalities had no clinical symptoms. In pJIA sub-group with cervical symptoms (n=24), patients needed more DMARDs and biotherapies per patient. Finally, comparison between pJIA and RA for radiographic findings showed only differences for two structural features: hypotrophy of vertebrae (24% vs 7%) and AJA (10% vs 0%). Conclusion Structural cervical spine involvement is a frequent manifestation in pJIA followed at adulthood and is correlated with a more severe disease. Radiologic assessment of cervical spine should be done systematically at onset and regularly during the course of the disease regardless of clinical symptoms. Cervical symptoms may be considered as a warning signal revealing presence of AJA.