Jun 12, 2015 - Friday, 12 June 2015. Scientific Abstracts. FRI0258 A NOVEL MRI-BASED LONGITUDINAL SCORING SYSTEM FOR. ARTERIAL ...
518
Scientific Abstracts
Friday, 12 June 2015
FRI0258
A NOVEL MRI-BASED LONGITUDINAL SCORING SYSTEM FOR ARTERIAL INVOLVEMENT IN LARGE-VESSEL VASCULITIS
E. Tombetti 1 , A. Zia 2 , D. Gopalan 2 , A. Kiprianos 1 , K. Bechmam 1 , B. Ariff 2 , J.C. Mason 1 . 1 Rheumatology; 2 Radiology, Imperial College London, London, United Kingdom Background: Although prevention of arterial progression is a hard therapeutic goal in large-vessel vasculitides (LVV), there is neither an agreed definition of progression nor a proposed method to globally assess arterial injury. This lack of validated clinimetry limits the feasibility of clinical trials. Although distinguishing disease activity from damage in Takayasu arteritis (TA) is difficult, current damage indices lack imaging data. Outside LVV, the only validated quantitative methods to assess arterial damage are NASCET and ECST, both designed for carotid atherosclerosis. Objectives: Starting from NASCET and ECST, we sought to develop an objective widely applicable magnetic resonance (MR)-based system for scoring large artery injury in TA and large vessel-giant cell arteritis (LV-GCA). Methods: NASCET was selected as a starting point as it only requires intraluminal data which are: i) more widely accessible in routine clinical practice, ii) require less acquisition time than arterial wall sequences, iii) more directly describe the haemodynamic derangement in LVVs, where wall thickening may occur in the absence of demonstrable luminal changes. NASCET was modified to account for the intrinsic characteristics of LVV by: i) defining a core set of arteries to be evaluated, with the final score determined by summing individual artery scores, ii) measuring the reference diameter for every artery in a plane judged “uninvolved”; iii) including the length of the stenotic segment in the algorithm, iv) scoring aneurysmal disease similarly to stenotic disease, based on the percent diameter dilatation and length of the involved segment (Fig. 1A). A cohort of 60 patients with TA or LV-GCA was selected, and those with vascular progression or improvement identified based on the radiologist’s judgement. Cross-sectional and longitudinal analysis by two independent observers was performed. The K-inter-observer variability and the sensitivity of the score in distinguishing patients with stable or progressive vascular involvement was evaluated. Results: Although more time-consuming than conventional assessment, our initial impression is that this new index is more precise. To date, we have evaluated 41 scans from 21 patients and scoring of the remaining images will be concluded in 3 months. Arterial involvement was classified as type IIa, IIb, III and V in 2, 1, 1 and 17 patients respectively. The median vascular score of the 21 pts was 16 (range 7-48). Analysis of Type V disease identified pts with both minimal and extensive arterial artery injury (median 24, range 9-42), demonstrating the precision of the index. 12 scans were judged to be stable, 6 improved and 2 worsened. This correlated with changes in the arterial score (median from baseline scan of -1.5 (range -5 to -1) for improved scans, of 0 (range 0 to 1) for stable scans and 7.5 (range 1 to 14) for worsening scans (pA) mutation in the CECR1 gene in all five patients. All were initially treated with corticosteroids; however none responded. One adult patient with extensive systemic amyloidosis was resistant to immunosuppressive and plasma treatments and died due to necrotizing pneumonia. The second adult patient partially improved with etanercept and plasmapheresis. The pediatric patients had better prognosis; one is on monthly plasma replacement and the other one on etanercept treatment with a good control of disease. The patient with a previous diagnosis of cutaneous PAN has responded to mycophenolate mofetil treatment. At literature review there are 44 DADA2 patients reported, including five ours. Patients who are homozygous for the p.G47R mutation have fewer strokes and predominantly a PAN-like phenotype compared to patients with other mutations. Conclusions: DADA2 may be classified as a secondary vasculitis due to a probable cause. The response to immunosuppressive treatment and the B cell defects in these patients places the disease in between the spectrum of innate and adaptive immune dysregulation. A genotype-phenotype correlation may exist for DADA2, with the G47R mutation causing a predominantly PAN-like phenotype while other mutations causing predominantly a vasculopathy with central nervous system disease (especially strokes). References: [1] Navon Elkan P, Pierce SB, Segel R, et al. Mutant adenosine deaminase 2 in a polyarteritis nodosa vasculopathy. N Engl J Med 2014;370(10):921-931 [2] Zhou Q, Yang D, Ombrello AK, et al. Early-onset stroke and vasculopathy associated with mutations in ADA2. N Engl J Med 2014;370(10):911-920 [3] Garg N, Kasapcopur O, Foster J, 2nd, et al. Novel adenosine deaminase 2 mutations in a child with a fatal vasculopathy. Eur J Pediatr 2014;173(6):827830 Disclosure of Interest: None declared DOI: 10.1136/annrheumdis-2015-eular.3493
FRI0260
THE ASSESSMENT OF DISEASE ACTIVITY IN TAKAYASU ARTERITIS; SIX YEARS EXPERIENCE FROM A SINGLE CENTER
S. Gulsaran 1 , P. Cetin 2 , D. Solmaz 2 , S. Akar 2 , H. Yarkan 2 , A. Gulcu 3 , Y. Goktay 3 , M. Birlik 2 , N. Akkoc 2 , F. Onen 2 . 1 Internal Medicine; 2 Rheumatology; 3 Radiology, Dokuz Eylul University School of Medicine, Izmir, Turkey
Conclusions: We are evaluating a new index for objectively quantifying arterial injury in LVV. Initial data suggest this approach precisely assesses arterial injury and reflects its evolution over time. This novel index might represent an important clinical measure of arterial disease also suitable for inclusion in LVV damage indices and hence a possible outcome measure for new clinical trials. Disclosure of Interest: None declared DOI: 10.1136/annrheumdis-2015-eular.6265
Background: Takayasu Arteritis (TA) is a chronic vasculitis of the aorta and its major branches affecting commonly young females. The current clinical and laboratory parameters of TA are insufficient for proper assessment of disease activity. Objectives: The aim of this study is to analyze the activity parameters that affect the physicians’ global opinion (PGO) in TA. Methods: The demographic and clinical data of patients with TA who were followed in the Rheumatology clinic at Dokuz Eylul University were retrospectively evaluated. Disease activity was assessed using various tools, including Kerr’s criteria, disease extent index-Takayasu (DEI.Tak), the Indian Takayasu Activity Score (ITAS2010), PGO, radiological activity parameters, and acute-phase reactants at every 3-6 months.According to the TA follow-up protocol of the Rheumatology and Radiology Board of our hospital, the patients were followed using 3-6 monthly B-mode/Doppler US examinations and 6-12 monthly magnetic resonance imaging (MRI) examinations with MRA.The agreement between disease activity parameters were assessed by kappa coefficient and Spearman rank correlation. Results: In total, 52 patients (48 females; mean age: 46.8±13.2 years) who fulfilled the American College of Rheumatology (ACR) criteria for TA were enrolled in this study. Their mean disease duration was 9.7±7.6 years and mean follow-up duration was 6.4±2.9 years. 33 (63.5%) patients had type 5 TA and 13 (25%)
Scientific Abstracts
Friday, 12 June 2015
had type 1 TA. 40 (76.9%) patients were categorized as having active disease in their first visits according to PGO. In the last visit, 31 (59.6%) patients had inactive disease, 19 (36.5%) had persistent disease and 2 (%3.9) had active disease. All the patients used the long-term glucocorticoid therapy and DMARD/immunosuppressive drugs. Six (%11.5) of them were on the treatment with biologics. Five (9.6%) patients underwent vascular surgery and 35 (67.3%) were performed endovascular repair for arterial stenosis. In total, 360 visits of 52 patients were evaluated. PGO was that the disease was inactive in the 181 visits, persistan in the 99 visits and active in the 80 visits. To obtain more clear results, visits with persistan disease were excluded and final evaluation included the 261 visits of 52 TA patients. Correlations of the clinical, laboratory and radiological activity parameters with each other and PGO were shown in Table 1. Table 1. Agreement rates and correlations of the disease activity parameters with each other and PGO in Takayasu arteritis Hs-CRP Hs-CRP ITAS2010 score DEI.Tak score NIH score Positive B mode/ Doppler US Positive MRI
ITAS2010 score
DEI.Tak score
NIH score
Positive B mode/ Doppler US
Positive MRI
PGO
0.2**
0.19** 0.93**
0.29* 0.68** 0.70**
0.19* 0.47** 0.47** 0.55*
0.32* 0.43** 0.44** 0.51*
0.32* 0.55** 0.54** 0.64*
0.53*
0.80* 0.79*
0.2** 0.19** 0.29*
0.93** 0.68**
0.70**
0.19* 0.32*
0.47** 0.43**
0.47** 0.44**
0.55* 0.51*
0.53*
*kappa statistic, **Spearman rho. All p values were
