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.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Arthritis+Res+Ther
2014 ; 16
(2
): R89
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Ultrasonographic assessment reveals detailed distribution of synovial
inflammation in Blau syndrome
#MMPMID24713464
Ikeda K
; Kambe N
; Takei S
; Nakano T
; Inoue Y
; Tomiita M
; Oyake N
; Satoh T
; Yamatou T
; Kubota T
; Okafuji I
; Kanazawa N
; Nishikomori R
; Shimojo N
; Matsue H
; Nakajima H
Arthritis Res Ther
2014[Apr]; 16
(2
): R89
PMID24713464
show ga
INTRODUCTION: Arthritis is the most frequent manifestation of Blau syndrome, an
autoinflammatory disorder caused by the genetic mutation of NOD2. However,
detailed information on arthritis in Blau syndrome on which the therapeutic
strategy should be based on is lacking. This multi-center study aimed to
accurately characterize the articular manifestation of Blau syndrome and also to
demonstrate the utility of musculoskeletal ultrasound in Blau syndrome. METHODS:
Patients who had been diagnosed with Blau syndrome by genetic analysis of NOD2
were recruited. A total of 102 synovial sites in 40 joints were assessed
semiquantitatively by ultrasound for gray-scale synovitis and synovial power
Doppler (PD) signal. RESULTS: In total, 10 patients whose age ranged from 10
months to 37 years enrolled in this study. Although only 4 joints (0.8%) were
tender on physical examination, 81 joints (16.9%) were clinically swollen.
Moreover, 240 (50.0%), and 124 (25.8%) joints showed gray-scale (GS) synovitis
and synovial PD signal on ultrasound, respectively. Importantly, GS synovitis was
present in 168 out of 399 non-swollen joints, in which 61 also exhibited synovial
PD signal. Among 40 joint regions, the ankle, the wrist, and the proximal
interphalangeal joints were the most frequently and severely affected joints.
Comparisons between different synovial tissues demonstrated a significantly
higher proportion of the joints with tenosynovitis as compared with that with
intra-articular synovitis (41.5% versus 27.9%, P?0.0001). In respect of age and
treatment, synovial PD signals were minimal in the youngest patient and in the
oldest two patients, and were relatively mild in patients receiving treatment
with methotrexate plus TNF antagonists. In two patients who underwent the second
ultrasound examination, total PD scores markedly decreased after initiating the
treatment with a tumor necrosis factor (TNF) antagonist. CONCLUSIONS: The
detailed information on synovial inflammation obtained by ultrasound confirms the
dissociation between pain and inflammation and the frequently involved joint
regions and synovial tissue in the arthritis of Blau syndrome. Our data also
demonstrate that ultrasonography can be a potent tool in monitoring the activity
of synovial inflammation and in investigating the pathophysiology of arthritis in
this rare but archetypical autoinflammatory condition.