Scand J Rheumatol 2010;39:373379 373

2010 Taylor & Francis on license from Scandinavian Rheumatology Research Foundation
DOI: 10.3109/03009741003685624 www.scandjrheumatol.dk
SRHE
Craniofacial growth disturbance is related to temporomandibular joint
abnormality in patients with juvenile idiopathic arthritis, but normal
facial profile was also found at the 27-year follow-up
LZ Arvidsson et al
LZ Arvidsson
1
, MG Fjeld
2
, H-J Smith
3
, B Flat
4
, B gaard
2
, TA Larheim
1
Departments of
1
Maxillofacial Radiology and
2
Orthodontics, Faculty of Dentistry, University of Oslo, and Departments of
3
Radiology
and
4
Rheumatology, Rikshospitalet, Oslo University Hospital and University of Oslo, Oslo, Norway
Objectives: To assess the long-term outcome of craniofacial morphology related to disease variables and
temporomandibular joint (TMJ) involvement as demonstrated with computed tomography (CT) and magnetic resonance
imaging (MRI) in adult patients with juvenile idiopathic arthritis (JIA).
Methods: Sixty of 103 patients participated in a re-examination on average 27 years after baseline. Craniofacial
morphology, with emphasis on size and position of the mandible, was assessed in lateral cephalographic images and
related to disease variables and TMJ involvement by CT and MRI. Definitions of craniofacial growth disturbances were
based on measurements outside 2 SD from the mean of healthy adult controls.
Results: Sagittal craniofacial growth disturbances were found in 57% and micrognathia in 27% of the 60 patients. Of
those with JIA TMJ involvement, 70% had some form of growth disturbance. Micrognathia occurred only in patients
with bilateral TMJ involvement. The bilateral TMJ group had significantly different craniofacial morphology than
healthy controls and patients without TMJ involvement. Growth disturbances and TMJ involvement were present in all
subtypes of JIA, except for one subtype comprising one patient. Patients with growth disturbances had more severe
disease than patients with normal craniofacial growth, regarding both present and previous disease activity. Unexpectedly,
half of the patients without craniofacial growth disturbances also had TMJ involvement, many from before the age of 12.
Conclusions: Craniofacial growth disturbances were found to be frequent in adult JIA patients, especially in those with
bilateral TMJ involvement. However, growth disturbances did not always follow TMJ involvement, not even when
affected early.
Recent studies indicate that temporomandibular joint
(TMJ) involvement is very frequent in juvenile idio-
pathic arthritis (JIA) (14) and the TMJ has been sug-
gested to be one of the most frequently involved joints
in patients with the disease (5). Attention is therefore
currently being focused on the JIA-involved TMJ, which
has been claimed to be the forgotten joint in rheuma-
tology (6).
A number of studies have demonstrated that cranio-
facial growth may be disturbed, with TMJ involvement
being the most important aetiological factor (710).
Even minor radiographic TMJ abnormalities have been
associated with disrupted mandibular growth and seve-
ral significant craniofacial changes (5, 11). However, a
limited number of longitudinal studies focusing on TMJ
abnormalities and craniofacial growth are available (12)
and, to our knowledge, no comprehensive studies have
followed patients from childhood to adulthood, compar-
ing mandibular growth in patients with and without TMJ
involvement, using growth in healthy individuals as
control.
From a group of 103 children with JIA, initially exami-
ned between 1976 and 1979, we re-examined 60 patients
on average 27 years after baseline. The aim was to assess
craniofacial growth disturbances and relate them to
disease variables and TMJ involvement as demonstrated
by computed tomography (CT) and magnetic resonance
imaging (MRI).
Materials and methods
The study was carried out in compliance with the
Helsinki Declaration and approved by the Regional
Committee for Medical Research Ethics, Southern Nor-
way. All patients gave their informed consent. At baseline
the mean age of the 103 JIA patients was 9.0 (range
2.516.4) years and the mean age of onset was 5.5
Linda Arvidsson, Institute of Clinical Dentistry, Department of
Maxillofacial Radiology, University of Oslo, PO Box 1109, Blindern,
0317 Oslo, Norway.
E-mail: l.z.arvidsson@odont.uio.no
Accepted 8 February 2010
374 LZ Arvidsson et al
www.scandjrheumatol.dk
(range 0.814.5) years, as described by Stabrun et al
(7). The patients were recruited consecutively from the
Oslo Sanitetsforening Rheumatism Hospital, where the
majority of Norwegian children with JIA or with sus-
pected JIA were referred. All 103 patients underwent
skull and TMJ examinations at the Faculty of Den-
tistry, University of Oslo, and were re-examined 2 and
4 years after baseline. Between 2002 and 2006, 60
patients (44 females, 16 males) with a mean age of 35.2
years were re-examined 27.0 1.5 years after baseline.
At all of the examinations, lateral cephalographs of the
skull and panoramic and transcranial radiographs of the
TMJs were obtained. The cephalographic examinations
from childhood to adulthood have been reported in one
study (13) and the TMJ examinations in another (14).
In adulthood the conventional TMJ examinations were
complemented with CT of all 60 patients and MRI of
47. The CT examination of the TMJs was performed
with a LightSpeed Ultra scanner (General Electric,
Milwaukee, WI, USA) using thin sections (0.6251.25
mm), a bone algorithm, and axial sections parallel to the
hard palate. The examinations were viewed in axial and
reformatted oblique coronal and oblique sagittal planes.
MRI was performed using 1.5 tesla units (Magnetom
Sonata or Magnetom Avanto, Siemens, Erlangen, Ger-
many) with a wide flex coil (Sonata) or 12-channel head
coil (Avanto). Pre-contrast oblique sagittal T1-weighted
(repetition time ms/echo time ms, 400/15 or 717/12) and
PD/T2-weighted (3090/1485 or 2800/1482) turbo
spin echo (TSE) images, followed by post-contrast
oblique sagittal and oblique coronal T1-weighted TSE
images, were obtained with closed mouth. Post-contrast
oblique sagittal gradientecho images (125/11 or 133/
11; flip angle 30) were obtained with open mouth. All
sequences had slice thickness 3 mm (gap 0.3 mm),
matrix 512 (305512), and field of view 140160 mm.
Contrast enhancement was provided by 0.1 mmol/kg
body weight gadopentetate dimeglumine (Magnevist,
Schering, Berlin, Germany) injected intravenously (15).
All patients underwent a clinical rheumatology exami-
nation and were classified according to the International
League of Associations for Rheumatology (ILAR) classi-
fication of JIA (16) by one of the authors with old and
new patient files at hand. Current medication was also
registered.
In the present analysis of the relationship between
craniofacial growth disturbances and the imaging TMJ
involvement, we used seven cephalographic measure-
ments and reference lines for size and shape of the man-
dible, and its position in relation to other craniofacial
structures, relevant for JIA, as shown in Figure 1A.
Cephalographic analysis was performed on 54
patients; the remaining six had undergone previous jaw
surgery. From the University of Oslo Craniofacial Growth
Archives, cephalographs from 54 randomly selected
healthy individuals (16 men and 38 women), matched
for gender and age, were used as controls (17).
Patients with at least one cephalometric value larger or
smaller, depending on the variable, than 2 standard devia-
tions (SD) from the mean of the control group were diag-
nosed as having craniofacial growth disturbances
(hereafter called growth disturbances). Micrognathia
was diagnosed when the length of the mandible (ArGn
distance, Figure 1A) was shorter than 2 SD from the mean
of the control group. All cephalometric values were com-
pared separately for females and males. The jaw surgery
group was excluded from the cephalometric evaluation in
adulthood but their values at the last examination in ado-
lescence, before surgery, were compared to studies per-
formed on healthy subjects of similar age (18, 19).
CT and MR images of the TMJs were evaluated by
three radiologists and a consensus was met with regard
to TMJ abnormalities in bone, disc, synovium, and bone
marrow. A detailed description of the CT and MRI findings
Figure 1. (A) Cephalographic measurements and angles. snB is the angle between the anterior cranial base or the nasion-sella line (NSL) and the
B-point: relationship between the mandible and the anterior cranial base. AnB is the sagittal relationship between the maxilla (A) and mandible (B).
ML/NSL is the angle between the mandibular line (ML) and the NSL: tilting of the mandible relative to the anterior cranial base. argn is the length
of the mandible from the articulare (ar) to the gnathion (gn). PFH is the posterior facial height: perpendicularly from the NSL to tgo (the constructed
point at the intersection between ML and a line drawn from ar tangentially to the posterior mandible). AFH is the anterior facial height: from nasion
(n) to gn, perpendicular to the Frankfurt Horizontal (FH). A/PFH is the the ratio between the anterior and posterior facial height. (B) Lateral cepha-
lograph of a 33-year-old female patient with bilateral JIA TMJ involvement and micrognathia, with antegonial notching of the lower mandibular
border. (C) Lateral cephalograph of a 34-year-old female patient with bilateral JIA TMJ involvement and micrognathia, without antegonial notch-
ing. (D) T1-weighted MR image of the right TMJ in the same patient as in C. The condyle is deformed, the articular fossa is widened, and the disc is
flattened but apparently in the normal position. Similar abnormalities were seen in the contralateral joint.
A B C D
Craniofacial morphology and TMJ in JIA 375
www.scandjrheumatol.dk
of 47 of the patients is reported separately (15), the main
finding being deformations of the condyles, fossae, and
discs of the JIA-involved TMJs.
Concerning TMJ involvement, the patients were
divided into the following five subgroups based on find-
ings at CT and MR imaging: (i) normal TMJ group (no
JIA TMJ involvement, n = 12), (ii) unilateral TMJ group
(unilateral JIA TMJ involvement, n = 4), (iii) bilateral
TMJ group (bilateral JIA TMJ involvement, n = 33),
(iv) jaw surgery group (patients with JIA TMJ involve-
ment and previous jaw surgery; orthognathic surgery,
chin augmentation, or combined surgery, n = 6), and
(v) internal TMJ derangement group (disc displacement
being the main finding, n = 5).
Cephalometric measurements in groups smaller than
10 individuals were not tested statistically. When com-
paring a dichotomous variable in two patient groups,
a c
2
test was used. For other continuous variables
a MannWhitney U-test was used. Linear regression
analyses were used to adjust mean differences for gender.
A 5% significance level was used throughout. The statist-
ical analyses were performed using SPSS version 14.0.
Results
The characteristics of the JIA patients are shown in
Table 1. Thirty-four of the 60 patients (57%) had some
form of craniofacial growth disturbance. Among the 43
patients with JIA TMJ involvement, 30 patients (70%)
had growth disturbances.
Significant differences between the bilateral TMJ group
and the healthy controls, as well as between the bilateral
TMJ group and the normal TMJ group, were found for six
and five of the seven cephalometric variables, respec-
tively, when adjusting for gender (Table 2). There were no
significant differences between the normal TMJ group and
the healthy controls when adjusting for gender in any of
the seven cephalometric variables analysed (Table 2).
In the bilateral TMJ group, 23 of 33 patients (70%)
had growth disturbances, that is one (seven patients) or
between two and six (16 patients) cephalometric values
outside 2 SD from the mean of the control group. In the
jaw surgery group, the five with bilateral, but not the one
with unilateral, TMJ involvement had sagittal growth
disturbances before surgery in adolescence.
In the unilateral TMJ group, two of four patients had
one cephalometric value each outside 2 SD from the
mean of the controls. Among the five patients with
internal derangement, two patients showed similar values.
Two of 12 patients (16%) in the normal TMJ group had
one cephalometric value each outside 2 SD.
Micrognathia was only found in patients with bilate-
ral TMJ involvement: in 12 patients (10 females) in the
bilateral TMJ group (Figure 1BD) and in four of the
five patients with bilateral TMJ involvement in the jaw
surgery group, that is in 27% of the 60 patients.
Table 2. Craniofacial skeletal measurements (angular and linear) on lateral head radiographs: Comparison between a healthy adult
control group (C, 54 individuals) and adult JIA patients: PB group (33 patients with bilateral JIA TMJ involvement) and P0 group
(12 patients with normal TMJs).
Sagittal cephalometric
variables C P0 PB
Difference between
P0 and C adjusted
for gender
Difference between
PB and C adjusted
for gender
Difference between
PB and P0 adjusted
for gender
snB, (SD) 79.9 (3.5) 78.6 (3.6) 76.1 (3.9) 0.4 1.9*** 1.2
AnB, (SD) 2.6 (2.3) 2.8 (2.0) 5.7 (2.4) 0.1 1.5*** 1.5**
ML/NSL, (SD) 27.7 (5.3) 27.6 (5.3) 35.7 (6.4) 0.1 4.0*** 3.6**
argn, mm (SD) 106.1 (6.6) 106.8 (5.3) 97.0 (8.0) 0.3 4.3*** 3.8**
PFH, mm (SD) 77.5 (6.7) 79.2 (9.2) 67.2 (7.5) 0.1 4.9*** 4.5***
AFH, mm (SD) 113.5 (6.2) 116.1 (6.7) 111.7 (8.1) 0.3 0.7 1.0
A/PFH, ratio (SD) 147.2 (11.8) 147.7 (12.8) 167.3 (16.1) 0.6 9.8*** 8.3**
Significance levels:
*p < 0.05, **p < 0.01, ***p < 0.001.
Table 1. ILAR subtype, gender, age, and disease variables in
60 adult JIA patients.
ILAR subtype Patients, n (%) Females/Males
Oligoarthritis, n (%) 13 (22), 12/1
Extended oligoarthritis 17 (28) 15/2
Polyarthritis, rheumatoid
factor negative
14 (23) 10/4
Polyarthritis, rheumatoid
factor positive
2 (3) 2/0
Systemic arthritis 7 (12) 4/3
Enthesitis-related arthritis 6 (10) 0/6
Psoriatic arthritis 1 (2) 1/0
Total 60 44/16
Mean (SD) Range
Age (years) 35.2 (3.6) 27.944.4
Onset age (years) 5.5 (3.1) 0.612.1
Disease duration (years)* 29.7 (3.3) 24.438.0
No. of active joints* 6.7 (10.8) 050
No. of joints with LROM* 15.0 (18.6) 069
Physicians global
assessment*
20.0 (25.2) 085
Patients global
assessment*
21.5 (20.3) 072
Physical function HAQ* 0.6 (0.7) 0.02.6
LROM, limited range of motion; HAQ, Health Assessment
Questionnaire.
*Disease variables assessed at mean 27-year follow-up.
376 LZ Arvidsson et al
www.scandjrheumatol.dk
In the 12 patients with micrognathia and bilateral TMJ
involvement, two-thirds had radiographic TMJ involve-
ment at the baseline examination and the remaining one-
third within 2 years thereafter. Six of the 12 patients had
TMJ involvement before the age of 10; the youngest
before the age of 6. The mean JIA onset age of the 12
patients was 4.9 years.
Thirteen patients with JIA TMJ involvement had no
growth disturbances (Figure 2). Six of those had TMJ
involvement at baseline and three patients within 2 or 4
years thereafter. Six of the 13 patients had TMJ involve-
ment before the age of 10; the youngest before the age
of 4. The mean JIA onset age of the 13 patients was 4.9
years.
Patients with growth disturbances had higher num-
bers of active joints and joints with a limited range of
motion, and higher levels of physicians global
assessments of disease activity than those without
growth disturbances (Table 3). The bilateral TMJ
group was significantly different from the normal
TMJ group regarding four of six rheumatological var-
iables (Table 4).
Growth disturbances and TMJ involvement were
present in all subtypes of JIA (except for the one patient
with psoriatic arthritis), and more frequently in the
patients with extended oligoarthritis and polyarthritis
than in those with oligoarthritis. Within these three largest
subtypes, the number of patients with TMJ involvement
corresponded well with the number of patients with growth
disturbances, except for the extended oligoarthritis
subtype in particular, where more than a third (38%) of
the patients with TMJ involvement had a craniofacial
morphology similar to the controls.
Regarding present medication in the 60 patients,
36 were under no medication whereas 13 were using
disease-modifying anti-rheumatic drugs (DMARDs) and/
or biological drugs, two patients were on corticosteroids,
and nine were using non-steroidal anti-inflammatory
drugs (NSAIDs) only.
Discussion
This is the first comprehensive study of craniofacial
morphology related to TMJ involvement in adult JIA
patients followed from childhood.
The patients with normal TMJs had a craniofacial
growth pattern similar to the healthy controls. To our
knowledge no previous JIA study has documented the
joint normalcy by using advanced imaging diagnostics.
Micrognathia occurred in patients with bilateral
TMJ involvement only, and was found in 42% of
Figure 2. (A) Lateral cephalograph of a 34-year-old female patient with bilateral TMJ involvement since the age of 9 and without craniofacial
growth disturbance. (B,C) T1-weighted post-contrast MR images of right (B) and left (C) TMJs in the same patient as in A. Both joints are deformed,
the disks are fragmented or destroyed and the fossae are completely remodelled with overgrowth of the eminences especially in the right TMJ.
A B C
Table 3. Disease variables in 60 adult JIA patients with (n = 34) and without (n = 26) craniofacial growth disturbance.
Craniofacial growth disturbance Without craniofacial growth disturbance
p-value Mean (range) SD Mean (range) SD
Onset age in years 5.3 (0.612.1) 3.1 5.9 (1.212.1) 3.3 0.47
No. of active joints 8.2 (050) 11.5 4.7 (035) 9.7 0.034*
No. of joints with LROM 19.2 (064) 19.5 9.4 (069) 16.1 0.015*
Physicians global assessment 24.3 (085) 26.3 14.3 (080) 22.9 0.040*
Patients global assessment 25.6 (072) 20.7 16.0 (057) 18.9 0.077
Physical function HAQ 0.7 (0.02.6) 0.7 0.5 (0.02.0) 0.6 0.070
LROM, limited range of motion; HAQ, Health Assessment Questionnaire.
*Statistical significance.
Craniofacial morphology and TMJ in JIA 377
www.scandjrheumatol.dk
those and in 27% of the entire series of patients. A
value of between 4% and 30% was mentioned by
Sairanen (20) and has been repeated in other studies
on childhood arthritis, but these figures seem to be
based on clinical impression and not on well-defined
criteria. Odenrick (21) reported 26% micrognathia in
106 children (316 years) with a similar definition as
the one we have used, and Rnning et al (22) and
Pearson et al (23) found micrognathia in 1819%
based on other cephalometric variables.
The majority of the patients with JIA TMJ involve-
ment had some form of growth disturbance (70%),
although not necessarily micrognathia. Typically, in
those with bilateral TMJ involvement, the mandibular
plane angle was steeper and the mandible was shorter
and more retrognathic than in healthy controls as well
as in the JIA patients without TMJ involvement. The
posterior facial height in patients with bilateral TMJ
involvement was shorter than in the other groups,
resulting in a larger ratio between the anterior and
posterior facial heights. These findings are expected
and have been described in previous studies on facial
growth in children and a few adults with JIA (810,
22, 2427).
Craniofacial growth disturbances occurred in 50, 59,
and 77% in the most frequent subtypes: oligoarthritis,
extended oligoarthritis, and seronegative polyarthritis,
respectively. Although growth disturbance was fre-
quently associated with TMJ involvement, some patients
had not developed growth disturbances. However, it
should be noted that facial asymmetry was not evaluated
in the present study.
Unexpectedly, of the 26 patients without growth
disturbances, 50% had JIA TMJ involvement. This is a
much higher figure than the 22% with radiographic
TMJ involvement found by Pearson et al (23) among
15-year-old JIA patients with normal facial growth.
They found that, in patients with TMJ involvement but
normal facial growth, the TMJ involvement usually
occurred after the age of 12. In patients with TMJ
involvement and growth disturbances, the TMJ
involvement often occurred before the age of eight (23).
Because of TMJ involvement already present at the first
examination of the patients in our study, or because of
young age at the last re-examination in childhood, the
age at TMJ involvement was difficult to determine pre-
cisely in some of the patients. However, eight of our 13
patients with TMJ involvement but without growth dis-
turbances had TMJ involvement definitely before the
age of 12, and four of those before or at 8 years of age.
In comparison, nine of our 12 patients with microg-
nathia could have had TMJ involvement before the age
of 8, and this occurred with certainty in three of them.
The finding that TMJ involvement did not lead to
growth disturbances fairly frequently will be addressed
in a longitudinal study of the present patient series, in
which the TMJ and craniofacial growth related to TMJ
changes over time in childhood will be analysed. In our
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378 LZ Arvidsson et al
www.scandjrheumatol.dk
study of early follow-up of the present patients we did
observe repair/remodelling processes in the TMJ (14)
and the consequence for the craniofacial growth pattern
needs to be investigated.
Patients with growth disturbances had a more severe
disease than the patients with normal craniofacial
growth, regarding both present and previous disease
activity. Our JIA patients were children and adolescents
during the 1970s and 1980s. Medication has changed
markedly in the past 30 years. Today, individual medi-
cation regimens are used according to the subtype of JIA
(28). With early intervention todays patients may have
a better chance of avoiding severe craniofacial growth
disturbances. There is also an increasing use of intra-
articular corticosteroid injections in the TMJs of JIA
children, although so far without evaluation of the con-
sequences on mandibular growth. We hope that the find-
ings from the present study can be used as reference
material when evaluating future patient and treatment
outcomes.
The present series seem to be rather representative
for JIA patients in Norway, as well as in other Scandi-
navian countries (14). The baseline observation of radi-
ographic TMJ involvement in the entire series of
children was about 40% (7, 29), a figure that was con-
firmed by Swedish and Finnish studies, although their
patient populations were somewhat older than ours (23,
30, 31). The high frequency of unilateral radiographic
TMJ involvement in our series of patients at baseline
(about 40%) was also similar to other Scandinavian
studies (23, 3133) reporting children with mean ages
between 10 and 15 years.
In conclusion, in this study of the long-term outcome
of jaw skeletal abnormalities in JIA, the majority of
patients with TMJ involvement had some form of
craniofacial growth disturbance. Micrognathia occurred
with bilateral TMJ involvement only, and in 27% in the
entire series of patients. Of note, about one-third of the
patients with TMJ involvement did not have sagittal
craniofacial growth disturbances even though they had
TMJ involvement from an early age.
Acknowledgements
We thank Dr Odd Vinje and Dr Jrn Thoen for valuable advice and
help with data collection, Dr Leiv Sandvik for help with the statistical
analysis, and Hkon Strmer for help with the illustrations. This work
was supported by grants from the University of Oslo, Norway. LZA
and MGF are financed by PhD stipends from the University of Oslo,
Norway.
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