Epidemiology and demographics of juvenile idiopathic arthritis in Africa and Middle East

Al-Mayouf, Sulaiman M.; Al Mutairi, Muna; Bouayed, Kenza; Habjoka, Sara; Hadef, Djohra; Lotfy, Hala M.; Scott, Cristiaan; Sharif, Elsadeg M.; Tahoun, Nouran

doi:10.1186/s12969-021-00650-x

Pediatric Rheumatology

Table 2 Demographic Characteristics

From: Epidemiology and demographics of juvenile idiopathic arthritis in Africa and Middle East

Sr. No.	Reference	Country	N (no. of cases)	F:M	Mean Age of onset (years)	Subtype			ANA positivity		Uveitis		RF positivity		HLA-B27
Sr. No.	Reference	Country	N (no. of cases)	F:M	Mean Age of onset (years)	Type	No.	%	%	Methodology of testing	%	Methodology/ Nature	%	Methodology of testing	%	Methodology of testing
Regional
1	Consolaro et al., 2019 [22]	Africa and Middle East	1209	1.61	6·0 (2·9–9·8)*	Psoriatic arthritis	37	3.1	NA	NA	5.9	NM	NA	NA	NA	NA
						RF-positive polyarthritis	61	5.0
						Undifferentiated arthritis	68	5.6
						ERA	111	9.2
						Systemic	204	16.9
						RF-negative polyarthritis	271	22.4
						Oligoarticular	457	37.8
2	Al-Mayouf et al., 2021 [28]	Arab (Saudi Arabia, Libya, United Arab Emirates, Jordan, Oman, Egypt, Kuwait)	702	2.04	5 (IQR 2.0- 9.0)*	Undifferentiated	11	1.6	30.9	Immunoassay	8.3	NM	9.3	Immunoassay RF was tested at least twice, with a minimum of 3 months apart. Test results were interpreted according to cutoff values of the local laboratories.	5.3^#	Flow cytometry
						Psoriatic	28	3.9
						ERA	39	5.6
						Oligoarticular Extended	43	6.1
						Polyarticular RF positive	48	6.8
						Polyarticular RF negative	159	22.6
						Systemic	172	24.5
						Oligoarticular persistent	202	28.8
Country
3	Khuffash et al., 1988 [13]	Kuwait	41	1.28	NA	Oligoarticular ANA negative	4	9.8	NA	NM	NA	NA	NA	NA	NA	NA
						Polyarticular seropositivity	5	12.2	NA		NA	NA
						Oligoarticular ANA positive	5	12.2	12.2		60.0^†	Asymptomatic chronic uveitis detected by slit lamp examination by an ophthalmologist
						Systemic polyarticular	6	14.6	NA		NA	NA
						Systemic oligoarticular	10	24.4			NA
						Polyarticular seronegative	11	26.8			NA
4	Khuffash et al., 1990 [14]	Kuwait	108	1.04	NA	Oligoarticular seropositive	3	2.8	NA	NA	NA	NA	NA	NA	NA	NA
						Oligoarticular ANA positive	9	8.3	8	NM
						Polyarticular seropositive	10	9.3	NA	NA
						Systemic polyarticular	13	12.0
						Systemic oligoarticular	18	16.7
						Oligoarticular ANA negative	19	17.6
						Polyarticular seronegative	36	33.3
5	Abdwani et al., 2015 [11]	Oman	107	2.57	6.85 ± 3.86 years	Psoriatic	1	0.9	32	Indirect Immunoflorescence; titer of ≥1:80 obtained on at least 1 clinic visit during the disease course was considered positive	None	Slit lamp examination by ophthalmologist during regular follow up visits at 3, 6 or 12 monthly intervals as per the pediatric screening recommendation	7.5	ELISA; RF was considered to be positive when titers were >20 IU/ml (If only one test of RF was performed, which was the case for many patients, then the results of this test were used to assign a JIA subtype rather than apply the subtype category of “other JIA.”)	NA	NA
						ERA	3	2.8
						Polyarticular RF positive	8	7.5
						Systemic JIA	19	17.8
						Oligoarticular JIA	34	31.8
						Polyarticular RF negative	42	39.3
6	Ozen et al., 1998 [15]	Turkey	30	0.67	NA	Systemic	1	12.5	NA	NA	NA	NA	NA	NA	NA	NA
						Polyarticular	12	42.4
						Oligoarticular	17	45.1
7	Abou El-Soud et al., 2013 [12]	Egypt	132	1.59	12.5 ± 4.56	Systemic	18	13.6	48.5	Indirect immunofluorescence on Hep-2 cells, with positive titers from 1/40 with at least two determinations 3 months apart during the first 6 months of the disease	19.7	Detected by slit lamp examination	27.20	Semi-quantitative latex test; titers ≥30 IU/mL were considered positive with at least two determinations 3 months apart during the first 6 months of the disease	66^‡	Low-resolution PCR analysis
						ERA	6	4.5
						Polyarticular RF positive	11	8.3
						Polyarticular RF negative	28	21.2
						Oligoarticular	69	52.3
8	Furia et al., 2020 [47]	Tanzania	28	1.15	NA	Oligoarticular	1	3.6	0.0	NM	NA	NA	NA	NA	NA	NA
						Systemic	6	21.4
						Polyarticular	21	75.0
9	Aiche et al., 2018 [31]	Algeria	70	1.8	7.3*	Psoriatic	1	1.4	2.9	NM	1.5	NM	NA	NA	NA	NA
						Systemic	7	10.0
						ERA	8	11.4
						Polyarticular RF positive	14	20.0
						Polyarticular RF negative	15	21.4
						Oligoarticular	25	35.7
10	Al Marri et al., 2017 [32]	Saudi Arabia	23	6.67	3.5	Psoriatic	1	4.3	8.7	NM	NA	NA	13.0	NM	NA	NA
						Polyarticular RF positive	3	13.0
						Polyarticular RF negative	5	21.7
						Systemic	14	60.9
11	Al-Mayouf et al., 2018 [35]	Saudi Arabia	100	1.70	4.5*	ERA	3	3.0	15.0%	NM	8.1%	NM	NA	NA	NA	NA
						Undifferentiated	3	3.0
						Psoriatic	6	6.0
						Polyarticular RF positive	13	13.0
						Oligoarticular	23	23.0
						Polyarticular RF negative	25	25.0
						Systemic	27	27.0
12	Salah et al., 2009 [63]	Egypt	196	1.09	6.257±3.41 years	Systemic-onset	47	24.0	21.7	Indirect immunofluorescence; positive at serum dilution between 1:80 to 1:60	5.6	Slit lamp examination; all detected patients had chronic uveitis	NA	NA	NA	NA
						Polyarthriticular	68	34.7
						Extended oligoarticular	18	9.2
						Persistent Oligoarticular	63	32.1
						Oligoarticular	81
13	Al-Abrawi et al., 2018 [33]	Oman	57	2.35	5.9*	ERA	0	0.0	7.0	NM	0	NM	NA	NA	NA	NA
						Undifferentiated	0	0.0
						Psoriatic	2	3.5
						Polyarticular RF positive	6	10.5
						Systemic	13	22.8
						Oligoarticular	16	28.1
						Polyarticular RF negative	20	35.1
14	Demirkaya et al., 2018 [45]	Turkey	466	1.49	6.3 (2.7–10.8)	Polyarticular RF positive	11	2.4	9.9	NM	8.1	NM	NA	NA	NA	NA
						Undifferentiated	12	2.6
						Psoriatic	15	3.2
						Systemic	64	13.7
						ERA	70	15.0
						Polyarticular RF negative	105	22.5
						Oligoarticular	189	40.6
15	El Miedany et al., 2018 [46]	Egypt	100	0.89	9.2 (5.3–11)*	Polyarticular RF positive	2	2.0	0.0	NM	6.0	NM	NA	NA	NA	NA
						Psoriatic	2	2.0
						ERA	2	2.0
						Oligoarticular	10	10.0
						Systemic	20	20.0
						Polyarticular RF negative	24	24.0
						Undifferentiated	40	40.0
16	Hashad et al., 2018 [48]	Libya	100	2.33	6.4 (3.1-10.4)*	Psoriatic	4	4.0	7.0	NM	2.0	NM	NA	NA	NA	NA
						Polyarticular RF positive	5	5.0
						Undifferentiated	5	5.0
						ERA	13	13.0
						Systemic	22	22.0
						Polyarticular RF negative	25	25.0
						Oligoarticular	26	26.0
17	Oyoo et al., 2016 [55]	Kenya	68	2.4	8.45	ERA	4	5.9	10.9^§	NM	1.47	Slit lamp examination by an ophthalmologist	17.6^¶	One positive or negative RF assay was considered adequate to classify polyarticular patients	NA	NA
						Systemic JIA	10	14.7
						Polyarticular RF positive	12	17.6
						Oligoarticular arthritis	16	23.5
						Polyarticular RF negative	26	38.2
18	Scott et al., 2018 [57]	South Africa	91	1.68	5.9*	Systemic	4	4.4	2.2	NM	8.2	NM	NA	NA	NA	NA
						Polyarticular RF positive	6	6.6
						Psoriatic	6	6.6
						Undifferentiated	8	8.8
						ERA	14	15.4
						Polyarticular RF negative	21	23.1
						Oligoarticular	32	35.2
19	Sen et al., 2015 [58]	Turkey	213	1.07	8.1 (range 8 months-15.4 years)	Psoriatic	2	0.90	11.70	Immunofluorescent antibody method; titers >160 IU/mL were considered positive	4.20	Slit lamp examination by an ophthalmologist	13.10	Nephelometric method; positivity defined by titers >20 U/mL on at least two occasions during the first six months of disease onset	2.8^	PCR
						Undifferentiated	0	0.00
						Systemic	19	8.90
						Polyarticular RF positive	23	10.80
						ERA	23	10.80
						Polyarticular RF negative	67	31.50
						Oligoarticular	79	37.10
20	Shafaie et al., 2018 [59]	Iran	102	2.19	5.2*	ERA	0	0.0	2.9	NM	1.0	NM	NA	NA	NA	NA
						Undifferentiated	0	0.0
						Polyarticular RF positive	1	1.0
						Psoriatic	1	1.0
						Systemic	15	14.7
						Polyarticular RF negative	16	15.7
						Oligoarticular	69	67.6
21	Yener et al., 2020 [61]	Turkey	116	1.58	NA	Undifferentiated	0	0.0	44**	Immunofluorescence; titer of 1/100 was considered positive	2.6	Slit lamp examination by an ophthalmologist every 6 months	22.7^##	Two RF values above 10 U/L measured at an interval of 3 months in a 6-month period were considered significant	21.1^††	Positive or negative for antigen
						Psoriatic	4	3.4
						Systemic	15	12.9
						Polyarticular RF positive	5	4.3
						Polyarticular RF negative	17	14.7
						Oligoarticular	37	31.9
						ERA	38	32.8
22	Çakan et al., 2017 [41]	Turkey	265	0.95	NA	Undifferentiated	5	1.9	27.20	Indirect Immunofluorescence; titers ≥1:100 were classified as positive	4.5	All cases were of anterior uveitis	3.8	Verified by a second analysis at least 3 months later	26^‡‡	NM
						Psoriatic JIA	5	1.9
						Polyarticular RF positive	10	3.8
						Systemic JIA	35	13.2
						Persistent oligoarticular	81	30.6
						Polyarticular RF negative	36	13.5
						Extended Oligoarticular JIA	6	2.3
						ERA	87	32.9
23	Kasapçopur et al., 2004 [51]	Turkey	198	0.87	6.62 ± 4.12	Other	5	2.5	18.2	Hep-2 cell; titers above 1/40 were considered positive	10.1	Slit lamp and a detailed ophthalmologic examination by ophthalmologist; single evaluation was considered sufficient for uveitis positivity; repeated every 3 months in uveitis and ANA positive patients	3.5	Nephlometric method	NM	Histocompatibility antigen determination
						Polyarticular RF positive	7	3.5
						Extended Oligoarticular	9	4.5
						Psoriatic JIA	11	5.6
						Polyarticular RF negative	34	17.2
						Oligoarticular JIA	37	18.7
						ERA	43	21.7
						Systemic JIA	52	26.3
24	Ozdogan et al., 1991 [56]	Turkey	147	0.77	8.4±3.9	Juvenile spondylitis	3	2.0	5.6	Indirect Immunofluorescence using human leukocytes as nuclear substrate and fluorescein anti IgG antisera	7.5	Slit lamp examination; Chronic uveitis in 7 patients and acute anterior uveitis in 1 male patient	10	Latex slide agglutination test	45	Standard microcytotoxicity test
						Polyarticular sero-positive	7	5.0
						Polyarticular sero-negative	19	13.0
						Systemic	37	25.0
						Pauciarticular	81	55.0
25	Abdul-Sattar et al., 2014 [30]	Egypt	52	2.06	NA	Polyarticular RF positive	5	10.0	NA	NA	NA	NA	NA	NA	NA	NA
						Oligoarticular persistent	9	17.0
						Polyarticular RF negative	11	21.0
						Systemic	12	23.0
						Oligoarticular extended	15	29.0
26	Abdul-Sattar et al., 2014 [29]	Egypt	58	2.41	NA	Polyarticular RF positive	5	8.6	NA	NA	NA	NA	NA	NA	NA	NA
						Oligoarticular persistent	11	19.0
						Polyarticular RF negative	12	20.7
						Systemic	13	22.4
						Oligoarticular extended	17	29.3
27	Albokhari et al., 2019 [36]	Saudi Arabia	44	1.59	NA	ERA	0	0.0	NA	NA	NA	NA	NA	NA	NA	NA
						Psoriatic	2	4.5
						Oligoarticular	6	13.6
						Polyarticular	7	15.9
						Systemic	12	27.3
						Unknown	17	38.6
28	Al-Hemairi et al., 2015 [34]	Saudi Arabia	82	1.65	7.1 ± 3.6 year	Undifferentiated	0	0.0	36.58	ELISA; titer of 1:80 or more was considered positive. Positivity was confirmed only if two samples were positive at least three months apart	8.53	Slit lamp examination by an ophthalmologist	4.87^§§	RF positivity was confirmed only if two samples were positive, tested three months apart	100% in ERA	NM
						ERA	1	1.2
						Polyarticular RF positive	4	4.9
						Psoriatic	4	4.9
						Polyarticular RF negative	20	24.4
						Oligoarticular	23	28.0
						Systemic	30	36.6
29	Amine et al., 2009 [38]	Morocco	80	1.42	7.53	Extended oligoarticular	4	5.0	NA	NA	NA	NA	NA	NA	NA	NA
						Systemic	21	26.0
						Polyarticular	25	31.5
						Persistent oligoarticular	30	37.5
30	Bahabari et al., 1997 [39]	Saudi Arabia	115	1.21	6(0.75-16)	ERA	0	0.0	30.0	Indirect immunofluorescence; positive at serum dilution between 1:80 to 1:60	1.70	Chronic uveitis	10.0	Slide agglutination test (till 1991); ELISA (after 1992)	6.0 ^¶¶	Standard microcytotoxicity
						Polyarticular RF positive	12	10.4
						Polyarticular RF negative	23	20.0
						Oligoarticular	30	26.1
						Systemic	50	43.5
31	Bouaddi et al., 2013 [40]	Morocco	33	0.83	NA	Polyarticular RF negative	1	3.0	76	NM	NA	NA	12.10	NM	NA	NA
						Oligoarticular	4	12.1
						ERA	5	15.2
						Systemic	8	24.2
						Polyarticular RF positive	15	45.5
32	Chipeta et al., 2013 [42]	Zambia	78	1.23	8.70 years (range: 1–15 years)	Psoriatic	1	1.3	NA	NA	11.50	Chronic uveitis in 3 patients with oligoarticular JIA and in 2 patients with ERA; Acute uveitis in 1 each of ERA and polyarticular JIA	NA	NA	NA	NA
						ERA	5	6.4
						Polyarticular RF positive	9	11.5
						Systemic	11	14.1
						Oligoarticular	25	32.1
						Polyarticular RF negative	27	34.6
33	Hussein et al., 2018 [49]	Egypt	63	0.90	6.1 (range 3-14) ±2.8	ERA	0	0.0	20.6	NM	6.3	Slit lamp examination	69.8	NM	NA	NA
						Undifferentiated	0	0.0
						Psoriatic Arthritis	0	0.0
						Polyarticular RF negative	6	9.5
						Systemic	15	23.8
						Polyarticular RF positive	16	25.4
						Oligoarticular	26	41.3
34	Olaosebikan et al., 2017 [54]	Nigeria	28	NA	NA	Systemic	5	17.9	NA	NA	NA	NA	7.14^^	Nephlometry	NA	NA
						Oligoarticular	9	32.1
						Polyarticular	14	50.0
35	Weakley et al., 2012 [60]	South Africa	78	1	8 (4–10)*	Psoriatic Arthritis	1	1.3	3.8***	Majority ELISA, remaining Hep 2 immunofluorescent	NA	NA	14.1^^^	One positive or negative assay for RF was considered sufficient to classify a patient with polyarthritis	23^###	NM
						Oligoarticular Extended	4	5.1
						Systemic	6	7.7
						Polyarticular RF positive	11	14.1
						Persistent Oligoarticular	17	21.9
						ERA	18	23.0
						Polyarticular RF negative	21	26.9
36	Mostafa et al., 2019 [53]	Egypt	48	2.42	NA	Psoriatic	0	0.0	NA	NA	NA	NA	42.0	NM	NA	NA
						ERA	0	0.0
						Oligoarticular	8	17.0
						Polyarthritis	28	58.0
						Systemic	12	25.0
37	Dagher et al., 2014 [43]	Lebanon	66	1	5.2 years (range: 9 months - 14 years).	Polyarticular RF positive	0	0.0	23	NM	6.1	NM	NA	NA	NA	NA
						Oligoarticular extended	3	4.0
						Undifferentiated	3	5.0
						ERA	11	17.0
						Systemic	15	23.0
						Polyarticular RF negative	16	24.0
						Oligoarticular persistent	18	27.0
38	Khawaja et al., 2017 [52]	UAE	66	2.47	NA	ERA	1	1.5	NA	NA	7.6	NM	NA	NA	NA	NA
						Psoriatic	1	1.5
						Oligoarticular extended	3	4.5
						Polyarticular RF positive	12	18.2
						Systemic	13	19.7
						Oligoarticular persistent	16	24.2
						Polyarticular RF negative	20	30.3
39	Alzyoud et al., 2020 [37]	Jordan	210	1.23	5.08±3.4 (7 months to 14 years)	Polyarticular RF positive	8	3.8	33.60	Indirect immunofluorescence using Hep-2 cells; titers > 1/80 were considered positive	14.2^†††	Slit lamp examination at a dedicated uveitis clinic	3.80	Nephelometry; Considered positive when titers were ≥ 15 units/mL. and at least two positive results, 3 months apart, in the first 6 months of observation	NA	NA
						ERA	15	7.1
						Polyarticular RF negative	18	8.5
						Psoriatic arthritis	18	8.5
						Systemic arthritis	36	17.1
						Persistent Oligoarticular	96
						Extended Oligoarticular	19
						Oligoarticular	115	54.7
40	Demirkaya et al., 2011 [44]	Turkey	634	1.26	7.69±4.41 (1-11 years)	Psoriatic	13	2.1	30.1	Titer of 1:80 was chosen as a cut-off point for ANA positivity for at least two positive results at least 3 months apart	11.6	Defined in accordance with the criteria of the SUN Working Group^^^^	3.1^‡‡‡	NM	63.3^§§§	NM
						RF positive polyarthritis	20	3.2
						Extended Oligoarticular	26	4.1
						Systemic	92	14.5
						ERA	120	18.9
						RF negative polyarthritis	129	20.3
						Persistent Oligoarticular	234	36.9
41	Karadag et al., 2020 [50]	Turkey	281	NA	NA	RF positive polyarticular	4	1.4	NA	NA	NA	NA	NA	NA	NA	NA
						Undifferentiated	7	2.5
						Systemic	11	3.9
						Psoriatic	13	4.6
						RF negative polyarticular	19	6.8
						ERA	97	34.5
						Oligoarticular	130	46.3
42	Yilmaz et al., 2008 [62]	Turkey	196	0.92	6.9 ± 3.7	Psoriatic arthritis	2	1.0	14.2	Indirect Immunofluorescence using Hep-2 cell; titers >1/80 were considered positive	2	Slit lamp and detailed ophthalmological examination by ophthalmologist every 4 – 6 months; chronic uveitis occurred in 2 patients with persistent oligoarticular JIA	8.1	Nephelometry; Considered positive when titers were 15 units/mL and confirmed with two positive results, 3 months apart, during the first 6 months of observation	5.6	Lymphocytotoxicity assay
						Others	5	2.5
						RF (+) polyarticular JIA	13	6.6
						Oligoarticular Extended	19	9.6
						ERA	19	10.3
						Systemic JIA	30	15.0
						Oligoarticular Persistent	48	24.4
						RF (–) polyarticular JIA	60	30.6

* Represents values in median
^#All patients underwent HLA-B27 testing; number patients tested not available in the article
^†3 out of 5 oligoarticular JIA patients tested positive for uveitis, however no full cohort uveitis rate is mentioned
^‡ HLA testing was carried out in only ERA (6 cases)
^§Not all cases were tested (5/46; 2 oligoarticular, 3 polyarticular RF Positive)
^¶Only in RF positive patients
^Note that HLA-B27 test was done in only 47 of the 213 JIA patients
**overall (62.22% in oligo)
^##In polyarticular JIA
^††in ERA
^‡‡HLA-B27 was studied in 169 patients (in all patients with ERA phenotype and male patients over the 6 years of age)
^§§Note that only the RF positive polyarticular patients tested positive (n=82)
^¶¶HLA-B27 was tested in 32 patients
^^Positive in 2 polyarticular positive RF and this is not specific to JIA patients only
***ANA testing was performed only on oligoathritis patients(n=67)
^^^Performed for polyarticular subtype
^###HLA tests were only performed for ERA subtype; all patients tested positive
^†††Most of them were Oligoarticular JIA 25/115 (21.7%) and were associated with positive ANA in 16/115 (14%)
^‡‡‡Tested in all except systemic; positive in only RF positive cases
^§§§Tested only in ERA patients
^¶¶¶In patients with uveitis; 24.6% in patients without uveitis; 28.4% combined population
^^^^Both the publications have cited Jabs et al., 2005 for the SUN Working Group Anatomic Classification of Uveitis. SUN working group has classified uveitis based on the primary site of inflammation: anterior uveitis (anterior chamber); intermediate uveitis (vitreous); posterior uveitis (retina or choroid); panuveitis (anterior chamber, vitreous, and retina or choroid).
ANA, anti-nuclear antibody; ARA, American Rheumatology Association; ELISA, Enzyme-Linked Immunoassay; ERA, enthesitis-related arthritis; EULAR, The European League Against Rheumatism; HLA, human leukocyte antigen; ILAR, International League of Associations for Rheumatology; IQR, interquartile range; JIA, juvenile idiopathic arthritis; NA, not available (study did not assess the parameter); NM, not mentioned; PCR, polymerase chain reaction; RF, rheumatoid factor.

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