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Saturday, 14 june 2014

SAT0283

THE INCIDENCE OF GIANT CELL ARTERITIS IN SLOVENIA

N. Potocnik 1 , . Rotar 1 , A. Hocevar 1 , J. Piem 2 , M. Hawlina 3 , A. Fakin 3 ,

M. Tomic 1 . 1 Department of Rheumatology, University Medical Centre Ljubljana;
2
Institute of Pathology, Universtiy of Ljubljana, Faculty of Medicine; 3 Department
of Ophtalmology, University Medical Centre Ljubljana, Ljubljana, Slovenia
Background: Giant cell arteritis (GCA) is the most common systemic vasculitis in
adults aged 50 years or above. Annual incidence rates vary widely from 6.976.6
per 105 of adults in this age group, depending on the region.1
Objectives: To determine the incidence rate of GCA in our population.
Methods: We prospectively collected incident cases of GCA from January 1st
2011 to December 31st 2012 at our department of rheumatology which is a
part of an integrated secondary/tertiary university teaching hospital that is the
only referral center serving a region representing approximately a quarter of the
national adult population. Additionally, newly diagnosed cases of GCA between
January 1st 2009 and December 31st 2010 were retrospectively identied by
searching the electronic patient records for ICD-10 codes M31.5 and M31.6 at our
department. The retrospective approach and search strategy was also applied
on electronic medical records at the departments of infectious diseases and
ophthalmology between January 1st 2009 and December 31st 2012. To further
reduce possibility of underreporting, the attached medical facultys Institute of
Pathology provided a list of all temporal artery biopsies examined during the
observation period which were then cross-referenced with the hospitals electronic
medical records. Annual incidence rate for GCA was then calculated.
Results: During the four year observation period we identied 97 new cases
of GCA (68% female; mean (SD) age 75.6 (8.1) years) from a well-dened
adult white Caucasian population of 232,041 inhabitants aged 50 or above. The
temporal artery biopsy was consistent with GCA, negative or not performed in
75.3%, 18.6, and 6.2% of cases, respectively. Thus, the annual incidence of GCA
in our population is 10.5 (95% CI 8.512.7), and 4.7 (95% CI 3.85.7) per 105
adults aged 50, and 20 years or above, respectively.
Conclusions: The annual incidence rate of 10.5 per 105 adults aged 50 or
above makes GCA the most common systemic vasculitis in this age group of our
population. Although the GCA almost exclusively affects adults over 50 years of
age, the incidence rate of 4.7 per 105 adults aged 20 and above, also makes
it the most common systemic vasculitis in the adult population overall, with IgA
vasculitis coming a close second with 3.7 per 105 adults aged 20 years or above.
References:
[1] Gonzalez-Gay MA, Vazquez-Rodriguez TR, Lopez-Diaz MJ et al. Epidemiology of Giant Cell Arteritis and Polymyalgia Rheumatica. Arthritis Rheum
2009;61:1454-61.
Disclosure of Interest: None declared
DOI: 10.1136/annrheumdis-2014-eular.1968

SAT0284

LARGE VESSEL INVOLVEMENT IN GIANT CELL ARTERITIS INCIDENCE, DISTRIBUTION AND PREDICTORS

N. Naderi 1 , A.J. Mohammad 2,3 , C. Turesson 4 . 1 Department of Rheumatology,

Kristianstad Hospital, Kristainstad; 2 Section of Rheumatology, Department of
Clincal Sciences, Lund, Lund University, Lund, Sweden; 3 Department of Renal
Medicine, Vasculitis and Lupus Clinic, Addenbrookes Hospital, Cambridge,
United Kingdom; 4 Section of Rheumatology, Department of Clinical Sciences,
Malm, Lund University, Malm, Sweden
Background: Over the years it has become increasingly clear that vascular
involvement in giant cell arteritis (GCA) is widespread, that vasculitic changes
may become evident many years after onset of the disease, and that these
anomalies may not always be symptomatic.
Objectives: To investigate the cumulative incidence of large vessel involvement
(LVI), dened as aneurysm/ectasia/stenosis of the aorta and its main branches, in
patients with biopsy-proven GCA, and to describe the distribution of LVI. Furthermore, to identify predictive factors and to evaluate to what extent the presence
of LVI is systematically investigated at the time of diagnosis and during follow-up.
Methods: Patients with biopsy-proven GCA in the region of Kristianstad, the
County of Skne in southern Sweden, diagnosed between 1997 and 2010, were
investigated. Patients were identied using the regional register of the Department
of Pathology in Skne as previously described (1). A retrospective review of case
records and histopathology reports of all the identied patients was performed.
Results: A total of 169 patients were identied, of whom 24 (14.2%) had LVI
with the following distribution: aortic ectasia n=17 (ascendens n=10, descendens
n=3, abdominal n=4), aortic aneurysm n=10 (ascendens n=3, descendens n=2,
abdominal n=5), aortic arch branches (cervical and upper-extremities) n=3,
visceral arteries (truncus coeliacus, mesenteric arteries, renal arteries) n=5,
lower extremity arteries (iliac, femoral, crural) n=10, intracranial vessels n=1. Two
patients had concomitant aortic aneurysm and aortic dissection, two had ectasia
at several levels of aorta and four had concomitant aortic aneurysm and ectasia.
Fourteen (58%) had isolated aortic involvement and six (25%) had concomitant
affection of the aorta and its tributaries.
There were no major differences between those with LVI and those without regarding sex (71% vs 76% females) or age at diagnosis (mean 76.5 vs 75.8 years).
LVI manifestations were initially noted a median of 3.7 years [interquartile range
(IQR) 0.7-7.5] after the diagnosis of GCA. Presence of giant cells in the biopsy
was signicantly less frequent among patients with LVI (23% vs 52%; p=0.01).

Scientic Abstracts
There was also a trend towards lower levels of CRP [median 75 (IQR 46-111) vs
90 mg/L (IQR 50-146); p=0.31] and ESR [mean 66 (SD 27) vs 75 mm1h (SD 28);
p=0.15] at diagnosis among those with LVI, although this did not reach statistical
signicance. In general, patients were not systematically investigated regarding
the presence of LVI at the time of diagnosis or during the course of the disease.
Conclusions: LVI of a number of different arteries is seen in GCA. The lower
proportion with giant cells in the biopsy and the lower initial inammatory response
suggest that this is a subset of GCA with particular characteristics and disease
mechanisms.
It is likely that the occurrence of LVI in GCA is severely underrated due to lack of
physician awareness of such complications.
References:
[1] Mohammad A et al. Ann Rheum Dis 2014; doi:10.1136/annrheumdis-2013204652. Epub Jan 17.
Disclosure of Interest: None declared
DOI: 10.1136/annrheumdis-2014-eular.2853

SAT0285

CHARACTERISTICS OF MPO-ANCA POSITIVE

GRANULOMATOSIS WITH POLYANGITIS

N. Ono 1 , A. Ueda 1 , S. Uezono 1 , D. Himeji 1 , T. Sawabe 2 , S. Yoshizawa 3 ,

S. Yoshizawa 4 , H. Nishizaka 5 , I. Furugo 6 , C. Kiyohara 7 , Y. Tada 8 , T. Horiuchi 9 .
1
Department of Internal Medicine, Miyazaki Preferetial Miyazaki Hospital,
Miyazaki; 2 Rheumatology, Hiroshima Red Cross Hospital, Hiroshima;
3
Rheumatology, Hamanomachi Hospital; 4 Rheumatology, National Fukuoka
Hospital, Fukuoka; 5 Rheumatology, Kitakyushu Medical Center; 6 Rheumatology,
JR Kyushu Hospital, Kitakyushu; 7 Public Health, Kyushu University, Fukuoka;
8
Rheumatology, Saga University Hospital, Saga; 9 Rheumatology, Kyushu
University Beppu Hospital, Beppu, Japan
Objectives: Through the investigation of the actual situation of ANCA associated
vasculitis in Japan, we attempted to clarify the characteristics of MPO-ANCA
positive Granulomatosis with polyangitis (MPO-GPA).
Methods: Retrospectively we recruited 38 GPA cases from 8 hospitals, and 41
Microscopic polyangitis (MPA) cases from one hospital. To exclude diagnostic
overlaps, GPA and MPA were classied by EMA classication. Their clinical
courses were analyzed based on sex, age, ANCA, organ involvements and
treatment outcomes.
Results: The mean age of GPA and MPA were 64.9 and 72.3 years old
respectively. Among GPA, 15 cases (39%) were positive for PR3-ANCA, 17
(45%) cases were positive for MPO-ANCA, and 6 cases (16%) were ANCA
negative. All MPA were MPO-ANCA positive. The mean ages are 60.4, 69.6,
63.0 and 72.3 years old respectively. The ratios of female are 20%, 82%,
50% and 56%. Compared to PR3-ANCA positive GPA (PR3-GPA), MPO-GPA
had more otitis media, less arthritis, lower serum creatinine levels and more
neuronal involvement. Compared to MPA, MPO-GPA had signicantly more ENT
involvements, pulmonary involvements and lower creatinine level. Both GPAs
experienced more relapses than MPA. MPA showed signicantly poor outcome
than GPA (p=0.008). The mortality rates of GPA and MPA were 5% (mean
follow-up time 1576 days) and 24.4% (mean follow-up time 778.2 days). MPOGPA showed signicantly fairer outcome than MPA even though their higher age
(p=0.013). The univariate analysis selected following factors as predictors of a
poor outcome: pulmonary UIP pattern (P=0.001), Cr1.7mg/dl (p=0.005), absence
of ENT involvement (p=0.032), higher age (65 years old, p=0.062) and pulmonary
hemorrhage (p=0.081).
Conclusions: MPO-GPA is characterized by older female patients with otitis
media, neuronal involvement and less renal injuries. Like PR3-GPA, MPO-GPA
showed fairer treatment outcome but more relapse than MPA. Because of their
higher age and fairer outcome, we need to manage MPO-GPA differently from
PR3-GPA and MPA.
Disclosure of Interest: None declared
DOI: 10.1136/annrheumdis-2014-eular.1283

SAT0286

PAEDIATRIC VASCULITIS DAMAGE INDEX: A NEW TOOL FOR

STANDARDISED DISEASE ASSESSMENT

P. Dolezalova 1 , N. Wilkinson 2 , P.A. Brogan 3 , J. Anton 4 , S.M. Benseler 5 ,

J. Brunner 6 , D.A. Cabral 7 , R. Cimaz 8 , K.M. ONeil 9 , S. zen 10 , R. Luqmani 11 ,
on behalf of Paediatric Rheumatology European Society (PReS) and Childhood
Arthritis & Rheumatology Research Alliance (CARRA). 1 Paediatric
Rheumatology, Charles University in Prague, Praha, Czech Republic; 2 Nufeld
Orthopaedic Centre NHS Trust, Oxford; 3 Great Ormond Street Hospital For
Children NHS Foundation Trust, London, United Kingdom; 4 Hospital Sant Joan
de Du, Universitat de Barcelona, Barcelona, Spain; 5 The Hospital for Sick
Children, University of Toronto, Toronto, Canada; 6 Innsbruck Medical University,
Innsbruck, Germany; 7 BC Childrens Hospital, Vancouver, Canada; 8 AOU Meyer,
Firenze, Italy; 9 University of Oklahoma Health Sciences Center, Oklahoma City,
United States; 10 Hacettepe University Department of Paediatrics, Ankara,
Turkey; 11 NIHR Musculoskeletal Biomedical Research Unit, University of Oxford,
Oxford, United Kingdom
Background: Standardised assessment of disease damage is one of the main
components of the OMERACT core set of outcome measures in adult ANCA-

Saturday, 14 june 2014

Scientic Abstracts
associated vasculitis. There is no validated tool to assess disease damage in
children with vasculitis. While paediatric vasculitis shares many features with adult
disease scoring tools validated for use in adults may miss some childhood specic
damage. The Paediatric Vasculitis Outcome working group gathers physicians
interested in childhood vasculitis from the PReS Vasculitis Working Group and
the North American CARRA Vasculitis Committee. In collaboration with EUVAS,
adaptation of adult vasculitis assessment tools was considered appropriate for
paediatric disease. Vasculitis damage tool development followed our publication
of the Paediatric Vasculitis Activity Score (PVAS).1
Objectives: To develop a paediatric modication of the Vasculitis Damage Index
(VDI).2
Methods: Invited paediatric specialists and the group members reviewed existing
items of the VDI and some additional items. Using nominal group technique
consensus was obtained on damage items and their denitions for use in a
paediatric modication of VDI (PVDI). Feasibility, face and content validity were
assessed by paper case evaluations.
Results: Vasculitis damage is dened as the presence of irreversible features
present for at least 3 months since the onset of vasculitis. While the VDI is an
inventory of 64 items grouped into 11 organ systems, PVDI contains 72 items
in 10 systems: musculoskeletal, skin/mucous membranes, ocular, ENT, chest,
cardiovascular, abdominal, renal, nervous and other. A detailed PVDI glossary
was produced. A separate assessment of school absence was added to the
one-page form. Each item can be scored as present or no longer present(NLP),
in order to address the potential reversibility of items that full the denition of
damage by duration and psychosocial impact but may completely resolve (e.g.
growth delay). Every scored item always receives only one point, whether scored
present or NLP, in order to retain compatibility with the VDI.
Conclusions: PVDI development is an important step towards better disease
assessment in children which together with the PVAS allows paediatric vasculitis
clinical trials and collaborative studies to gather reliable data on these rare
diseases. The PVDI has yet to complete the validation process by its prospective
use in real patients, which is currently underway. We aim to ensure that it remains
a dynamic data-driven tool reecting ongoing developments in the eld of adult
vasculitis damage assessment.
References:
[1] Dolezalova P, Price-Kuehne FE, zen S et al. Disease activity assessment in
childhood vasculitis: development and preliminary validation of the Paediatric
Vasculitis Activity Score (PVAS). Ann Rheum Dis 2013 Oct;72(10):1628-33.
[2] Exley AR, Bacon PA, Luqmani RA, et al. Development and initial validation of
the Vasculitis Damage Index (VDI) for the standardised clinical assessment of
damage in the systemic vasculitides. Arthritis Rheum 1997;40:371-80
Disclosure of Interest: None declared
DOI: 10.1136/annrheumdis-2014-eular.5893

SAT0287

ASSOCIATION BETWEEN TEMPORAL ARTERY ULTRASOUND

HALO SCORE AND BIOPSY IN NEWLY DIAGNOSED GIANT
CELL ARTERITIS

R. Brier 1 , F.A. Borg 1 , P. Patil 1 , C. Dejaco 2 , B. Dasgupta 1 . 1 Rheumatology,

Southend University Hospital, Westcliff-on-Sea, United Kingdom;
2
Rheumatology, Medical University Graz, Graz, Austria
Background: Temporal artery ultrasound (TAUS) is increasingly used in the
diagnosis of giant cell arteritis (GCA). Vessel wall oedema (Halo) is suggestive
of GCA. However, its prognostic importance and association with temporal artery
biopsy (TAB) remain unclear. We report a semi-quantitative composite score for
vascular oedema (Halo Score), its ability to predict positive histology on TAB, and
the association between ultrasound characteristics and clinical and laboratory
features in GCA.
Objectives: To investigate whether the extent & severity of temporal and axillary
artery oedema (Halo Score, HS) or the number of affected arterial branches is
associated with clinical and laboratory characteristics and TAB ndings in newly
diagnosed GCA.
Methods: We conducted a single centre retrospective data analysis from 91
consecutive patients (mean age 72.6 years, 72.5% female) with GCA according to
ACR criteria, who underwent ultrasound of bilateral temporal and axillary arteries
by a single blinded sonographer. The branches of the vessels were systematically
assessed for the Halo sign. This was then semi-quantitatively scored at each site
as no halo (score 0), the smallest third (1st tertile) of halo at each branch as
mild (score 1) halos, 2nd tertile as moderate (score 2) and the largest third of
halo (3rd tertile) as extensive (score 3), as the extent of the halo sign is linked
to the diameter of the arterial branch. Total Halo Score (HS) was constructed
as the sum of grades from all sites. TAB results were available from 79 (86.8%)
patients. For statistical analysis, Mann-Whitney U, Spearmans rank correlation,
Chi-square tests and ROC curve analysis were applied.
Results: Halo was detected in 57 (62.6%) patients. ESR (37 vs 20mm/h, p=0.018)
and CRP (27 vs 5mg/l, p=0.039) were higher in patients with positive halo. TAUS
positive patients were more commonly male (35.1% vs 14.7%, p=0.035), and
more often had scalp tenderness (75.4% vs 41.2%, p=0.001), constitutional
upset (68.4% vs 44.1%, p=0.022) and positive TAB (40.4% vs 7.4%, p=0.002).
They were less likely to have visual symptoms (15.8% vs 38.2%, p=0.016). No
difference was found in headache, temporal swelling or pulsation, jaw or limb
claudication, anaemia and polymyalgic symptoms.

697

In TAUS positive patients, the median number of affected branches was 2 (range
1-6) and median HS 4 (range 1-18). Association with patient characteristics are
given in the table.
Table 1
Variable

Median number affected branches

Male
+ve TAB
Reduced TA pulse
Jaw claudication

Median Halo Score

Yes

No

p value

Yes

No

p value

3.5
3
4
3

2
2
2
2

0.02
0.03
0.181
0.047

6.5
7
8
6

3
3
4
3

0.013
<0.001
0.019
0.019

There was good agreement between HS and number of affected branches. HS

also correlated with age (corrcoeff 0.29, p=0.028), ESR (0.27, p=0.04) and CRP
levels (0.36, p=0.005).
The relationship between positive TAB and HS was assessed using ROC curve
analysis yielding AUC 0.81 (95% CI 0.68-0.93). A Halo Score of 4 predicts positive
TAB with high sensitivity (90.5% sensitivity, 71.0% specicity) and HS 7 gives
high specicity (sensitivity 42.9%, specicity 90.3%).
Conclusions: Our ndings support vascular ultrasound as a useful non-invasive
investigation in GCA. Halo ndings were associated with demographic, clinical
and laboratory features in GCA. Halo score was able to predict positive TAB
ndings. A high HS may replace the need for diagnostic TAB in some cases.
Disclosure of Interest: None declared
DOI: 10.1136/annrheumdis-2014-eular.3986

SAT0288

EOSINOPHILIC GRANULOMATOSIS WITH POLIANGEITIS

(EGPA): CLINICAL FEATURES AND OUTCOME IN A LARGE
SERIE OF SPANISH PATIENTS

R. Solans-Laqu 1 , G. Fraile 2 , M.J. Castillo 3 , X. Solanich 4 , L. Caminal 5 ,

M. Rodriguez 6 , J.J. Rios 7 , M. Zamora 8 , J.L. Calleja 9 , P. Fanlo 10 , I. Garcia 11 ,
L. Saez 12 , J. Oristrell 13 , M. Abdilla 14 , F. Pasquau 15 , M. Lopez-Dupla 16 ,
A. Perez 17 , E. Fonseca 18 , B. Sopea 19 , on behalf of REVAS Study Group.
1
Internal Medicine, H Vall Hebron, Barcelona; 2 Internal Medicine, H Ramon y
Cajal, Madrid; 3 Internal Medicine, H Virgen del Rocio, Sevilla; 4 Internal Medicine,
H Bellvitge, Barcelona; 5 Internal Medicine, H Central, Asturias; 6 Internal
Medicine, H Mutua Terrassa, Barcelona; 7 Internal Medicine, Hospital La Paz,
Madrid; 8 Internal Medicine, H Virgen de las Nieves; 9 Internal Medicine, H San
Cecilio, Granada; 10 Internal Medicine, Clinica de Navarra, Pamplona; 11 Internal
Medicine, H Infanta Leonor, Madrid; 12 Internal Medicine, H Miguel Servet,
Zaragoza; 13 Internal Medicine, H Parc Tauli, Sabadell; 14 Internal Medicine, H La
Ribera Alzira, Valencia; 15 Internal Medicine, H Marina Baixa, Alicante; 16 Internal
Medicine, H Joan XXIII, Tarragona; 17 Internal Medicine, Complejo Hospitalario,
Orense; 18 Internal Medicine, H Cabuees, Asturias; 19 Internal Medicine, C
Hospitalario Vigo, Vigo, Spain
Objectives: To analyze the demographic, clinical, laboratory features and
outcome of patients with EGPA in a large cohort of Spanish patients with AAV
Methods: multicenter retrospective-longitudinal study that included patients
diagnosed with AAV between January 1995 and December 2012 in 19 Hospitals
from Spain (REVAS Study). Statistical analysis was performed using the SPSS
vs17.
Results: 87 patients with EGPA (mean age 52.516.1 yr) from 405 with AAV were
analyzed. Asthma was present in all cases and preceded EGPA by 6 months to
29 yr (mean 81.6 mo) except in 6 cases in which began simultaneously. A history
of allergic rhinitis was noted in 42.5% cases with nasal polyposis in 23% and
recurrent sinusitis in 49.4%. ANCA were positive in 66% cases (91% MPO ANCA).
Mean follow-up was 82.575.3 months. The most frequent clinical manifestations
at diagnosis were fever (44%), arthralgia (47%), constitutional symptoms (41%),
neurological symptoms (54%), and purpura (35%). Renal failure was present in
14% cases, reno-pulmonary sd. in 4.6% and cardiac involvement in 27.6%. Renal
and neurological involvement were more frequent in positive ANCA patients
(p=0.005) and cardiac involvement in ANCA negative (p=0.001). Chest-x-ray
showed lung inltrates (56.3%) and nodules (16%). Eosinophilia was present
in all cases. Serum IgE levels were raised in 73.3% of tested patients. A total
of 101 biopsies were done (25 nerve,15 lung,6 renal,7 nasal,38 skin,4 bowel,2
pericardial,2 liver,2 pleural) and showed eosinophil inltrates in 52 cases and
necrotizing vasculitis in 47. Oral prednisone (1mg/kg) was given to all patients, iv
metilprednisolone to 45%, iv cyclo-phosphamide (CF) to 41.4%, oral CF to 24.1%,
azathioprine to 21.8%, and mycophenolate to 5.7%. Rituximab was successfully
administered in 2 cases with refractory disease. Leukopenia was more frequent
in patients treated with oral CF (p<0.000). During the follow-up, 27.6% patients
developed bacterial infections, 11.5% opportunistic infections and 9 (10.3%) died.
Dead was mainly related to infections (p=0.011) and refractory disease. EGPA
patients had less renal involvement (p=0.005) and more cardiac involvement
(p=0.002) than those with GPA and MPA and a lower mortality (p=0.001)
Conclusions: patients with EGPA have less severe clinical manifestations than
those with GPA and MPA and a lower mortality. Renal involvement is rare and
cardiac involvement frequent, as previously described. Most patients with FFS>1
improve with corticosteroids and CF. RTX may be useful in patients with refractory
disease. The overall EGPA survival rate is good and dead is mainly related to
infections and refractory disease