Professional Documents
Culture Documents
DOI 10.1007/s00296-013-2707-2
ORIGINAL ARTICLE
Received: 24 May 2012 / Accepted: 12 February 2013 / Published online: 20 March 2013
Springer-Verlag Berlin Heidelberg 2013
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2316 Rheumatol Int (2013) 33:2315–2322
Our objective was to determine the prevalence and distri- Only patients residing in permanently in one of the districts
bution of various patterns of juvenile idiopathic arthritis in of Sharkia Governorate were included. Following the cur-
Sharkia Governorate of Eastern Egypt. rent definition of arthritis in children, a patient had to have
swelling of one or more joint or limited range of joint
movement with pain or tenderness observed by physician.
Materials and methods The 2004 revised ILAR classification for JIA was used
in this study. Patients can be classified into seven sub-
Background population groups: systemic arthritis, oligoarticular arthritis, polyar-
thritis with rheumatoid factor, and polyarthritis without
We conducted a population-based, multicenter study rheumatoid factor, psoriatic arthritis, enthesitis-related
among rheumatologists and pediatricians in Sharkia arthritis (ERA), and undifferentiated arthritis [10].
Governorate to identify patients with JIA less than 15 years The following information was collected from each
and seen between November 2009 and November 2010. patient: age, sex, disease onset, disease duration, whether the
Sharkia Governorate is located in east of Egypt, and it patient had uveitis or iridocyclitis by slit lamp examination,
comprises 19 districts. According to national agency for results of tests for rheumatoid factors and antinuclear
mobilization and statistics, it has a population of 8,566,465 antibodies, and the treatments used [nonsteroidal anti-
inhabitants at the end of 2010. The total number of children inflammatory drugs (NSAIDs), systemic glucocorticoids,
under the age of 15 years was 3,844,718 on that year. disease-modifying antirheumatic drugs (DMARDs), local
A total of 829,496 were in the urban areas and 3,015,222 glucocorticoid injections, and physical therapy].
were in the rural areas. Boys comprise 1,976,715 while
girls were 1,868,003. Exclusion criteria
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Rheumatol Int (2013) 33:2315–2322 2317
140
120 Frequency
90
100 80
70
80 60
50
60 40
30
40 20
10
20 0
Urban Rural
0
Frequency of JLA Privelance /100.000 Residence
Fig. 1 Frequency and prevalence of JIA in Sharkia governorate Fig. 2 Prevalence of JIA in Sharkia according to residence
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heterogeneity of the diseases and their subtypes encom- (2004–2006), prospective, population-based study was then
passed under this rubric [24]. In addition, variability in carried out in Catalonia (Spain) calculated a relative risk of
disease course among the subtypes of JIA may make it girls having JIA was 2.1 [95 % confidence interval (CI)
difficult to compare prevalence estimates for this condition 1.7–2.7, P \ 0.001] [26]. In Taiwan, the prevalence of JCA
across different study settings. In some types of the disease, in girls was 4.1 per 100,000 (95 % CI 3.3–4.9) and for boys
extended remissions occur, so that prevalence estimates was 3.5 per 100,000 (95 % CI 2.9–4.2) [4]. In contrast to
include individuals who were ever affected, but are not the previous results, Turkish children showed higher
currently affected. prevalence of JIA among boys. Yilmaz et al. [28] found
In a study carried out in specific area of Norway, the that there were 102 boys and 94 girls with a mean duration
prevalence of JIA was 83.7 (95 % CI 72.4; 95.8) per 100 of disease of 4.1 years. Also, in another epidemiological
000 children aged 0–15 years [25]. Modesto et al. [26] study in the republic of Bashkortostan (Russia), the pre-
carried out prospective and retrospective studies in Cata- valence of JIA in boys was higher, than in girls (93.9 and
lonia, a region in the north of Spain, based on ILAR 73.7 per 100,000 children, respectively, P \ 0.001) [29].
classification criteria; prevalence was 39.7 (36.1–43.7)/100 Further studies are required to understand how genetic and
000 children younger than 16 years. It was the first popu- environmental differences affect JIA expression.
lation-based study on the epidemiology of JIA in Catalonia. In this work, the overall mean age at diagnosis of pre-
Incidence and prevalence rates are lower than those valent patients was 10.5 ± 3.6 (range 4–15) years. Among
reported for several areas in Nordic countries of Europe. the total, 70.5 % had been diagnosed before the age of 7
Solau-Gervais et al. [5] yielded a prevalence of and only 29.5 % patients had been diagnosed at the age of
15.7/100,000 in France. Many factors contribute to the 12 or older. Our results are similar to the mean age of JIA
discrepancies between reported prevalence and incidence in Quebec (Canada) which was 9.8 ± 4.6 years, 68 %
for JIA. Studies based truly in the community reported the were female, and more persons were diagnosed in winter
highest prevalence, as previously undiagnosed cases were [30]. Yilmaz et al. [28] found that the mean age at the first
included. Future studies involving standardized criteria and visit was 8.8 years, and the mean age at onset of disease
standardized case ascertainment done by fully trained cli- was 6.8 years (range 8 months–15 years). Approximately
nicians should show greater consistency of results [20]. similar results reported in Catalonia (Spain), the mean
We noted a clear female preponderance of JIA in this annual incidence was 6.9/105 children aged less than
study, in which the prevalence of JIA in girls was 4.33 per 16 years (range 5.8–8.1 years; 9.0 years for girls and
100,000 (95 % CI 3.3–5.1) and in boys was 2.58 per 4.8 years for boys) [26]. Also, in southwestern Sweden, the
100,000 (95 % CI 2.4–3.6). Girls predominated over boys peak incidence rate of 18.3 per 100,000 was found in girls
(81/51) and showed a relative risk of developing JIA of 0 through 3 years old. The lowest incidence rate, 6.4 per
1.68 (1.18–2.39, P = 0.003). Our results are similar to the 100,000, was found among boys 12 through 15 years old
reports from Western countries in the female preponder- [13]. Solau-Gervais et al. [5] reported that the mean age of
ance of JIA. In East Berlin, the frequency of JCA is higher JIA at diagnosis was 6.6 years (range 1–15 years). In
for girls, with an incidence of 4.3 per 100,000 and a another study done in France, age at appearance of the first
prevalence of 2.3 per 10,000. The figures for boys are 2.7 symptoms was 4.7 years (±3.2 years) in girls, as compared
per 100,000 and 1.7 per 10,000, respectively [27]. A 2-year to 7.2 years (±3.7 years) in boys [19].
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The subgroup distribution in our work reported that ANA and uveitis in patients with oligoarthritis (62.3 %)
oligoarthritis was the largest group (n = 69, 52.2 %), and in polyarthritis without RF (42 %). Danner et al. [19]
followed by polyarticular group (n = 39, 29.5 %), and found that uveitis occurred in 41 % of children with olig-
systemic group (n = 18, 13.6 %), and then finally, enthe- oarthritis and in 14 % of those with polyarthritis without
sitis-related arthritis group (n = 6, 4.5 %) of the total RF. Skarin et al. [34] showed that fifty-five out of 350
population. There was no undifferentiated or psoriatic patients with JIA developed uveitis (15.7 %). Forty-six
arthritis. Solau-Gervais et al. [5] found that oligoarticular (84 %) of these had oligoarthritis, 6 (11 %) had polyar-
disease was the most common pattern with 20 (41.6 %) thritis, and 3 (5 %) had systemic disease. Also, in a large
patients while enthesitis-related arthritis contributed eight population-based nationwide study in Germany of patients
(16.6 %) patients, and there were nine patients with poly- with JIA, the prevalence of uveitis was 12 % of all JIA
articular disease and seven with systemic disease. A 5-year patients. The most frequent were oligoarthritis extended
prospective population study in southwestern Sweden (25 %) and persistent (16 %): ANA positive (86 vs. 42 %)
found that oligoarticular onset type constituted 68.3 % of than the patients without uveitis [2]. Grassi et al. [35]
the prevalence cases, while 21.9 % were polyarticular and recorded sixty-two patients developed uveitis (20.1 %); 57
6.6 % had systemic onset [13]. Also, in France, Danner patients had oligoarticular-onset, 3 polyarticular-onset, and
et al. [19] reported the most frequent forms were oligoar- 2 systemic-onset JIA. Saurenmann et al. [36] found that
thritis (n = 27, 40.3 %), polyarthritis without rheumatoid 142 of 1,081 patients (13.1 %) had developed uveitis, and
factor (RF; n = 15, 22.4 %), and enthesitis-related arthritis the risk factors were young age at diagnosis, female sex,
(n = 12, 17.9 %). Other forms, notably systemic arthritis antinuclear antibody positivity, and the subtype of JIA.
(n = 6, 8.9 %) and psoriatic arthritis (n = 3, 4.5 %), were Berk et al. [37] demonstrated that uveitis was diagnosed in
more rare, and in this study, there was no case of polyar- 11 patients (12.2 %). Of these, seven (63.6 %) had olig-
thritis with RF. Only 4 patients (6 %) were classified in the oarticular, two (36.4 %) had polyarticular, and one (9.1 %)
undifferentiated arthritis group. Arguedas et al. [31] found had systemic-onset juvenile rheumatoid arthritis (JRA).
that 77 % of the JCA cases in Costa Rica were of pauc- Antinuclear antibodies (ANA) were positive in seven
iarticular onset, and 23 % were of polyarticular onset. No (63.6 %) of the 11 uveitis patients, confirming ANA as a
cases of systemic JCA were diagnosed. Martinez Mengual significant determinant for uveitis in juvenile arthritis. The
et al. [32] reported that the most frequent form of onset was previous results was unlike to that reported by Bolt et al.
persistent oligoarticular arthritis (41.7 %), followed by [38], who found that the rate of uveitis was 13.2 % in
spondyloarthropathies (11.7 %), conditions that did not cohort of Swiss children with JIA and the subgroup with
meet the criteria for any category (11.7 %), polyarticular the highest rate of uveitis was ‘‘other arthritis,’’ followed
arthritis (11.7 %), systemic disease (10 %), psoriatic by oligoarticular JIA. Extended and persistent course of
arthritis (6.7 %), and extended oligoarticular arthritis oligoarticular JIA had a similar uveitis incidence. Yilmaz
(6.7 %). However, in Turkey, polyarticular JIA was the et al. [28] also reported that chronic uveitis occurred in two
most frequent onset type (37.2 %). Other subtypes included patients with oligoarthritis; two patients with enthesitis-
oligoarthritis (34.2 %), systemic arthritis (15.3 %), psori- related arthritis had acute uveitis. Authors considered the
atic arthritis (1 %), enthesitis-related arthritis (9.7 %), and factors to be the cause of the variations seen were diag-
other arthritis (2.2 %) [28]. Also, an Indian study done by nostic difficulties, development of new diagnostic criteria,
Kunjir et al. [33] found enthesitis-related arthritis (ERA; differing definitions of a clinical case, and small sample
36 %), oligoarthritis (OLA-persistent; 17 %), polyarthritis sizes leading to larger variations in reported rates.
rheumatoid factor (RF) negative (17 %), polyarthritis RF In this study, ANA tested positive account 48.5 % of
positive (12 %), systemic arthritis (8 %), OLA extended prevalent cases, RF tested positive in 27.2 % of the studied
(4 %), and psoriatic arthritis (1 %). The remaining 11 patients, and HLA-B27 was positive in 4 out of 6 patients
children (5 %) were classified with undifferentiated of ERA (66 %). Uveitis was present in 26/132 prevalent
arthritis. The discrepancies between the previous two cases (19.7 %) diagnosed at the time of the study.
studies and those reported from Western countries are Modesto et al. [26] reported that ANA tested positive in
possibly the results from true differences pertaining to 57.6 % of prevalent cases. Among ANA-positive patients,
ethnicity, geography, or both. Future studies are necessary 79.5 % were girls, 80 % of ANA carriers were included in
to elucidate the implications suggested by these data. the oligoarthritis group, and RF was documented in three
Our study showed that uveitis occurred in 26 patients cases only (0.8 % of prevalent cases). HLA-B27 was
(19.7 %) of the total 132 JIA patients. Oligoarthritis was positive in 19.4 %. The latter group was distributed mostly
the largest group representing (n = 21, 30.4 %) followed in the enthesitis-related arthritis group, few in the olig-
by polyarticular group without RF representing (n = 3, oarthritis and undifferentiated arthritis groups. Uveitis was
10.7 %). Our results also highlight the frequent presence of present in 12 % only of prevalent cases. In study done in
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