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Abstract:
Objective:
To describe the long-term outcome of juvenile idiopathic
arthritis (JIA) in Moroccan patients.
Patients and methods:Cross-sectional
study including patients with JIA according to the ILAR criteria
for the diagnosis of JIA, old of more than 18 years, followed
into hospital or consultation. The evaluation included a
clinical evaluation (articular mobility, activity of the
disease), functional estimation (HAQ) and structural analyze (Steinbrocker).
Results:Fifty
one patients were recruited (27 women and 24 men), of average
age 27.60 years ± 7.35 [18-46]. Subtypes at inclusion were: 15%
enthesitis related arthritis, 12% seronegative polyarthritis,
11% seropositive polyarthritis, 5% systemic arthritis, 5%
oligoarthritis and 3% psoriatic arthritis. After on average
15.06 ± 8.88 years [3-39] disease onset, forty two patients
(82.35%) had an active disease. Our patients had a disabled
functional status with a median of HAQ higher than 1.0. Half of
our patients had a stage III or IV of Steinbrocker. The pain
visual analogue scale, health assessment questionnaire and
erythrocyte sedimentation rate were significantly higher among
patients having an active disease at the time of study. The
duration of evolution and the delay of diagnosis were the major
factors associated with a pejorative outcome at adulthood.
Conclusion:
This study showed that more than 80% of our Patients with JIA
remained active at the adulthood and were generating of a
disability and a structural destruction. The interest carried to
the early treatment of the infantile diseases must also include,
in our country, that of the JIA.
J.Orthopaedics 2010;7(3)e9
Keywords:
long-term outcome; juvenile idiopathic arthritis; HAQ,;SF-36;
Steinbrocker; quality of life.
Introduction:
Juvenile idiopathic arthritis (JIA) can persist over many years
with children that could experience disability and dysfunction
in adulthood [1]. Despite a well conducted medical treatment,
that could have a beneficial effect on joint inflammation, it is
not curative and induces occasionally remission. The outcome of
juvenile arthritis is generally regarded as good, with the
possibility of a spontaneous remission. However, clinical
experience suggests that JIA is not a benign disease, and a
review of the literature indicates that the outcome in adulthood
is variable [5-26]. At least one-third of children could keep an
active disease into, and many will have great limitations in
their daily activities [19,20,23,25]. However, studies of the
outcome of juvenile arthritis are often not comparable because
of disease definition differences’ (i.e., JRA or JCA) and
treatment differences (reflecting changes in the approach to
management over several decades). In addition, different
measures of outcome have been used with a variable duration of
follow-up. Nonetheless, these outcome studies showed that
approximately one-third of patients have marked functional
disability and many patients (10-45%) have ongoing active
disease at 10 years. The prognosis of chronic arthritis varies
quite a lot, and the perception of different dimensions and the
prognosis may vary depending on cultures and societies. There
are few published studies about outcome of JIA in no
industrialized countries [2-4]. The aim of our study was to
describe clinical and functional features on adult
Moroccan patients with long-standing JIA.
Materials
and Methods:
Patients:
Patients were enrolled at rheumatology department of university
hospital of Rabat in Morocco on a period of 08 months.
Eligibility criteria included patients with a diagnosis of JIA
according to the International League of Association of
Rheumatology (ILAR) criteria [27].
All patients were over 18 years old and gave informed consent to
tacking part in the study, which also received the approval of
the local ethics committee.
Patients were assessed individually. Subsequently, a full review
of the patient's clinical notes was performed.
Disease subtype was determined retrospectively. Previous medical
history was specified, including medication use,
concurrent diseases and surgery. All patients were assessed
using joint counts (number of joints with swelling
(range 0–62); number of joints with tenderness/pain on
passive motion (range 0–75) and number of joints with
limited range of motion (range 0–67)). Patients
completed a self-rated 10-cm VAS for pain, based on their pain
experience during the week before the clinical assessment.
Laboratory tests included erythrocyte sedimentation
rate (ESR; Westergren method) and rheumatoid factor. A
patient was considered to be in remission if at follow-up if he
or she had no symptoms or objective signs of disease
activity, in the presence of normal ESR for more than
two consecutive years without any anti-rheumatic
treatment. Joint symptoms due to JIA sequelae were not
considered. A modified Steinbrocker functional class
was used to allow comparison with older [28].
The health assessment questionnaire (HAQ) [29] was used as
specific measure of functional disability, and the medical
outcomes study 36-item Short Form (SF-36) [30-33] was used to
assess health status and QOL; both of these tools are translated
and validated into Moroccan dialect. Additional questionnaires
regarding educational experience and employment status were
collected including items regarding current employment status
and highest level of education attained.
Statistics
The statistical package for the social sciences (SPSS) version
13.0 was used for the analysis. Data for patients were
expressed as mean and standard deviations for continuous
variables and number and frequencies for categorical variables.
To compare the median scores between the study groups, we used
the Mann-Whitney U test for nonparametric data. Pearson's
correlation coefficients were calculated to evaluate linear
correlation between two numerical variables. Using the variables
with p < 0.05., multiple linear regression analysis was
performed. Significance level was set as p < 0.05.
Results :
51 patients with JIA
were included in this cross sectional study. Patients’
characteristics are presented in table I. The median age was
27.5 years (range 18-46); median disease duration was 15 years
(range 3-39). There were no recorded deaths in this group. JIA
subtypes were: oligoarticular onset (18 patients, 3 with
persistent disease and 2 with extended disease), polyarticular
onset (20 patients, 11 of whom were rheumatoid factor [RF]
positive), systemic onset (5 patients), psoriatic (3 patients),
and enthesitis-related arthritis (5patients). Uveitis resulting
in visual impairment occurred in 2 patients (a persistent
oligoarticular pattern in 1 patients and an enthesitis-related
pattern in 1 patient).
Table I: Patient demographics
|
Age, mean (range) years
Sex, no. women/men
Age at diagnosis, median
±
SD years
Disease duration, median (range) years
No.(%) patients with active joint disease
HAQ score, median (range)
Self-reported pain score on VAS, mean (range) |
27.60 [18-46]
27/24
12.55
±
3.66 [4-16]
15 [3-39]
42 [82.35]
1.29 [0-3]
50 [0-90] |
HAQ = Health Assessment Questionnaire; VAS = visual analog
scale.
Outcome measures:
Disease activity:
Only 9 of our patients (17.65%) had a remission. The mean active
joint count was 4.3 (range 0-24), and the mean restricted joint
count was 6.4 (range 0-26). Among the 23 patients with
polyarticular-onset disease, 20 had active disease and 10 were
RF positive. Only one patient with a RF-positive polyarticular
disease had inactive disease. Disease-modifying antirheumatic
drugs (DMARDs) were used by 33 of the 51 patients (methotrexate
[n = 17], sulfasalazine [n = 12], hydroxychloroquine in
combination with methotrexate [n = 1] and 3 patients had a
biologic treatment).
Functional disability and QOL
The median HAQ score was 1.29 (range 0-3; higher scores
correspond to a greater degree of functional disability). There
was a significant difference in the HAQ scores according to
disease duration (P = 0.01) and early onset of disease (P =
0.01). The HAQ scores (2.6, range 0-3) was greater in patients
with active disease rather than patients with inactive disease
(0.62, range 0-3) (P = 0.03). The SF-36 scores compared to
healthy age- and sex-matched controls are shown in Table II.
Results show a major impact of JIA on health status. Our
patients had low scores for all physical domains (physical
functioning, vitality, bodily pain, general health); social
functioning and emotional role in the mental domains, compared
to controls. Table III summarizes the PSS (physical summary
score), MSS (mental summary score), and HAQ scores, stratified
by age. The SF-36 scores were lower with increasing age. HAQ
scores were higher, indicating worse functional disability, in
older patients. There were no statistically significant
differences in HAQ, PSS and MSS scores between subtypes of JIA
(ANOVA variance analysis-results not shown). Also, the
educational level and work status didn’t influence the health
status (p>0.05). We’ve found high statistically significant
correlations between HAQ scores and all domains of SF-36
(p<0.001). For all domains of QoL, a severe functional
disability was associated with an impaired QoL. The Steinbrocker
functional class distribution was represented: 20 (39.4%)
patients were in functional class I, 5 (10.6 %) in functional
class II, 10 (19.2%) in functional class III and 16 (30.8 %) in
functional class IV. A statistically significant correlation was
found between Steinbrocker functional class and disease duration
(P < 0.001) and polyarticular subtype (P < 0.001).
In multiple
linear regression, disease
duration was the most important parameter that influences
negatively function, QoL and Steinbrocker functional class.
Table II: QOL as measured by the SF-36 in adults with
juvenile arthritis and age- and sex-matched controls
|
Domain |
Patients |
Controls |
P |
|
Physical
Physical functioning
Vitality
Bodily pain
General health
Mental
Physical role
Social functionning
Emotional role
Mental health |
30.84 (10-100)
29.96 (62-95)
30.05 (0-100)
27.99 (15-90)
27.20 (5-94)
28.43 (2-87)
27.80 (3-79)
27.05 (9.5-95) |
71.56 (58.85-99)
72.46 (5-90)
72.37 (71-97)
74.47 (74-97)
75.28 (75-99)
74.02 (74-97)
74.66 (74-95)
74.43 (6.9-9.5) |
< 0.001
< 0.001
< 0.001
< 0.001
< 0.001
< 0.001
< 0.001
< 0.001 |
Values are the mean score (range) for each domain. The higher
the score, the better the quality of life for that particular
domain. P values were determined by the Mann-Whitney U
test. QOL = quality of life; SF-36 = Short Form 36; NS = not
significant.
Table III: Functional disability (HAQ) and summated
quality of life (SF-36) scores in adults with juvenile arthritis
|
|
Age < 30 years
(n=30) |
Age ≥30 years
(n=21) |
|
PSS
MSS
HAQ score |
29.98
±
3
30.89
±
5
1.16
±
0.3 |
20.08
±
2
20.08
±
4
0.85
±
0.3 |
Values are the mean ± SD. Statistical analysis by Kruskal-Wallis
one-way analysis of variance showed statistical significance
when comparing PSS, MSS, or HAQ scores between age groups with p
= 0.007, 0.02, 0.008 successivly. HAQ = Health Assessment
Questionnaire; SF-36 = Short Form 36; PSS = physical summation
score; MSS = mental summation score.
Discussion :
In this cross-sectional study, the majority of patients with JIA
had unfavourable outcome at adulthood. More than 80% of our
patients had active disease after median disease duration of
15years. Those results concord with the findings of Aggarwal and
al [2].But in that Indian study the disease duration was lower
(6 years). In contrast, in a Sweden population of 124 JIA, only
49% of patients had active disease at median disease duration of
7 years [2]. In majority of previous follow-up studies [5-11],
the follow-up time has ranged between 5 and 15 years and only in
three studies has exceeded 15 years. In two of the later
studies, about 30-50% of patients had active disease. According
to our results and in comparison with results of Zak and al
[12]; disease activity persisting into adulthood is perhaps one
of the most important outcome parameters in JIA. In our study,
Enthesitis related arthritis and seronegative polyarthritis are
the most representative subtypes in our patients. Patients with
persistent oligoarticular disease, who are generally regarded as
having the best prognosis, are underrepresented in our study
thing that could explain the high proportion of patients with
active disease [2, 5, 12]. According to Aggarwal and al study
[2], among 506 patients with JIA, rates remission were 45% for
oligoarticular-onset disease for only 20% in the polyarticular
group. Also, Guillaume and al reported [13] that patients with
oligoarticular-onset disease were most often in remission,
probably because of the heterogeneity of this group. In the same
study, the authors reported that among patients with
oligoarticular-onset JIA, the probability of a polyarticular
course was 50% [13]. In addition the high proportion of patients
with active disease in our patients in which the polyarticular
onset en Enthesitis related arthritis are the most
representative, concords with results of Guillaume and al in
which only 18% of patients with Enthesitis related to arthritis
had remission [13]. Many (64%) of our patients were taking
DMARDs. This observation is consistent with other outcome
studies [13-18].
On the other hand, our patients had a disabled functional status
with a median of HAQ higher than 1.0 and more than 30% of
patients were in Steinbrocker functional class IV. Our results
are comparable with the findings of Minden and al [5] who state
that 6.5% of patients had an HAQ score of 1.0 or higher, and 10%
were severely disabled according to Steinbrocker functional
class status [10, 19, 20, 21, 22]. In our patients, the subtype
of the JIA didn’t influence the functional disability. In the
present study, there was a significant difference in the HAQ
scores according to disease duration and early onset of disease.
In patients with active disease, the median HAQ score was
higher. Other long-term studies have shown that the fraction of
disabled patients clearly increased in association with longer
time-span to follow-up [2, 5, 6, 12, 23 and 24]. In Minden and
al study [5], disease activity had the strongest influence on
functional status but disease duration was not significantly
correlated with the HAQ score. Zak and al [12] confirmed that
disease duration was the strongest predictor of an unfavourable
disease outcome but also patients with polyarticular affection,
history of systemic steroid treatment and long disease duration
had experienced major discomfort with deteriorated HAQ and
functional class [25, 26].
The results of our study are important because the present study
is one of few others in which the SF-36 was used as a validated
measure of health status and QoL and which the International
League of Associations for Rheumatology criteria for JIA were
applied [6, 23, 27-29]. In the present study, SF-36 scores were
low for all JIA subtypes compared with controls. Our patients
had worse scores on the SF-36 compared with those of the
patients described by Foster et al [6] or by Peterson and al
[23], which can be explained by the low proportion of the
oligoarticular-onset group. Measures of SF-36 demonstrate poor
global outcome for patients and showed lower scores in the older
patients suggesting the impact of the disease duration. We’ve
found a high correlation between domains of SF-36 and HAQ but
the subtype of the JIA didn’t influence the QoL. In discordance
of our results, Foster and al [6, 29] demonstrated significant
differences in outcome between the subgroups of JIA. The authors
noticed that patients with seropositive polyarticular-onset and
systemic-onset JIA had worst functional outcome. Many of our
patients had persistent active disease, significantly worse HAQ
scores than those without active disease; and many were taking
DMARDs. Our patients had evidence of multiple joint involvement
and we’ve found a significant correlation between disease
activity and number of active joints, which can have an impact
on physical status on QoL that may not be adequately assessed by
the HAQ. Finally, we didn’t found significant differences in
SF-36 or HAQ scores between subtypes of JIA or according to
educational and work status of patients. Those results
contraries the study results of Foster and al who suggested that
employed patients had higher scores of SF-36 than the scores of
those who were unemployed [21, 25].
In the current study we found that the majority of patients with
JIA had unfavourable outcome in adulthood. 82% of the
participants had active disease and disease activity was
suggested to be the strongest outcome parameter in JIA. Our
study results suggest in concordance with other studies results
that polyarticular subtype and also Enthesitis related to
arthritis were associated with disease persisting into adulthood
and that the degree of functional residua (HAQ) and functional
class (Steinbrocker) increased with time. Also, all of our
patients have a deteriorated quality of life with decreased
SF-36 scores that are influenced by an increased HAQ score.
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