ORIGINAL ARTICLE

Physical disability, articular, and extra-articular damagein patients with juvenile idiopathic arthritis

Pradip Kumar Sarma & Ramnath Misra &

Amita Aggarwal

Received: 13 February 2008 /Revised: 21 March 2008 /Accepted: 3 April 2008 / Published online: 24 May 2008# Clinical Rheumatology 2008

Abstract Data on outcome of juvenile idiopathic arthritis(JIA) from the Indian subcontinent is limited. JuvenileArthritis Damage Index (JADI) is a newly proposed indexwhich measures articular (JADI-A) and extra-articulardamage (JADI-E). We studied the outcome of JIA usingJADI in Indian patients. We assessed the damage in patientswith JIA using JADI, and to see if JADI scores correlate withvarious parameters of damage and disease activity. Westudied 89 patients of JIA (excluding enthesitis-relatedarthritis) with a ≥1-year duration of the disease. BesidesJADI, clinical assessment included active joint count, jointswith limited mobility, ESR, and CHAQ. Radiologicaldamage was assessed according to the Dale scoring system.Correlation of JADI with various parameters was done bySpearman’s rank correlation coefficient. The patient’s distri-bution of JIA subtypes was polyarticular (47), systemic onset(SoJIA 23), oligoarticular (15), psoriatic arthritis (one) andothers (three). The median duration of disease was 5 years(1–20). JADI-A ranged from 0–61 (Median 2); 60.7% ofchildren had articular damage. Thirty-five (39.3%) patientshad extra-articular damage; out of which, growth failure wasthe commonest. Persistent oligoarticular subtype had lesserJADI-A score as compared to SoJIA and polyarticular JIA.JADI-A correlated significantly with (p<0.01) with radio-logical damage (0.538), CHAQ (0.567), JADI-E (0.513),duration of disease (0.385), and loss of education years dueto disease (0.352). Further it also correlated with measuresof disease activity like: ESR (0.286), duration of morning

stiffness (0.258, p<0.05), physician’s global assessment(rS 0.623), and parent’s global assessment (0.446), Almosttwo-thirds of patients with JIA had articular damage andone third had extra-articular damage. JADI is a good tool tomeasure damage in children with JIA.

Keywords HAQ . Health outcome . JADI

Introduction

Juvenile idiopathic arthritis (JIA) is the most commonchronic pediatric rheumatic disease that affects about 0.1%of children [1]. Contrary to previous belief that JIA is acondition that often remits with time, recent studies indicatethat it continues to be active in 40% to 60% of patientsduring adulthood [2, 3]. The frequency of joint erosionsvaries from 25 to 68% [4].

Population-based studies on functional outcomes of JIApatients showed that 11% to 39% of the patients weresignificantly incapacitated, either wheelchair-bound orbedridden (Steinbrocker Classes III or IV) [5–7]. Permanentchanges may also develop in extra-articular organs/systems.Childhood Health Assessment Questionnaire (CHAQ) andradiography are the most commonly used tools to assessphysical disability and joint damage, respectively. How-ever, despite advantages and widespread use, both havelimitations in research and clinical settings [8].

The Juvenile Arthritis Damage Index (JADI) is a newlydescribed tool to assess the overall articular (JADI-A) andextra-articular damage (JADI-E) [8]. In a previous study,it was validated among 158 JIA patients. The JADI-Ascore correlated highly with the number of joints withlimited range of motion and moderately with the CHAQ,Steinbrocker functional classification, and radiographic

Clin Rheumatol (2008) 27:1261–1265DOI 10.1007/s10067-008-0901-5

P. K. Sarma : R. Misra :A. Aggarwal (*)Department of Immunology,Sanjay Gandhi Postgraduate Institute of Medical Sciences,Lucknow 226014, Indiae-mail: amita@sgpgi.ac.ine-mail: aa.amita@gmail.com

damage. Correlation with the JADI-E score was low. TheJADI-A discriminated well among different levels ofdisability. The internal consistency of the JADI-A andJADI-E was excellent to good, respectively (8).

Outcome data in JIA from India are scant [9]. Therefore,we undertook this study to measure outcome in patientswith JIA who attended a tertiary care hospital; JADI wasmeasured and it was correlated with different parametersincluding CHAQ, duration of disease, radiological damageand disease activity.

Materials and methods

This was a cross-sectional study. The study populationconsisted of 89 patients with JIA (ILAR criteria [10])attending the outpatient clinic at Sanjay Gandhi PostgraduateInstitute of Medical Sciences, Lucknow, a tertiary carereferral hospital. JIA patients more than 5 years of age andgreater than 1 year of disease duration were included in thestudy. A verbal consent was taken from the parents/patient.As in the previous study, enthesitis-related arthritis (ERA)patients were excluded since JADI has not been validated forthem. The same rheumatologist (PKS) examined all patients.Onset of symptoms of arthritis or systemic features wastaken as the date of onset of disease.

Examination included physician’s global assessment ona 100-mm visual analogue scale (VAS), number of swollenjoints, number of joints with pain on movement/tenderness,and number of joints with limited range of motion (ROM).Active joint was defined as joint with swelling or any twoof limitation of motion (LOM), pain, heat, or tenderness[8]. For articular indices, sixty-seven joints were assessed.ESR was measured by the Westergren method.

JADI scores were calculated as described previously [8].Further assessment included educational level achieved,

loss of school years, functional status measured by CHAQ.For the purposes of analysis, the CHAQ score was dividedinto four categories: 0=no disability, >0, and ≤0.5=milddisability, >0.5, and ≤1.5=moderate disability, and >1.5=severe disability [8]. Parents/patients also assessed theirdisease activity on a 100-mm VAS.

Radiographic examination was done on clinical indica-tion for involved joints. Radiographs were graded accord-ing to the Dale radiographic classification system for JIA[11]. A patient was considered to be in remission if therewere no joints with active arthritis, fever, rash, serositis,splenomegaly, or generalized lymphadenopathy attributableto JIA, no active uveitis, normal ESR, and physician’s globalassessment of disease activity indicates no disease activitywithout DMARD or steroid for at least 12 months [12].

Statistical analysis Spearman’s rank correlation coefficient(rS) was used to assess correlations among differentparameters. Mann–Whitney U test was used for inter-groupcomparisons.

Result

The median age of 89 patients with JIAwas 14 years (range5–32 years) and the median duration of disease was 5 years(1–20 years). Rheumatoid factor (RF) negative was thecommonest subtype (Table 1); the patients with psoriaticarthritis (PsA) [01 (1.1%)] and others [03 (3.4%)] were notincluded in further statistics as numbers were small.

Seventy-one patients were on NSAID. Naproxen was thecommonest NSAID used. Thirty one patients were on oralsteroids, dose ranging from 1.25 to 20 mg daily with a mediandose of 7.5 mg;13/23 SoJIA and 12/35 polyarticular RF−patients were on oral steroids; only a few of the other subtypeswere on steroid. Sixteen patients had received intra-articular

Table 1 Demography and disease activity parameters

All subtypes Systemic onset Poly RF+ Poly RF− Oligo-extended

Oligo-persistent

Number of patients (%) 89 23 (25.8) 12 (13.5) 35 (39.3) 6 (6.7) 9 (10.1)Median age at onset in years 9 (1–16) 6 (1–14) 12 (5–16) 10 (5–15) 6 (3–10) 8 (2–13)Median duration of disease in years 5 (1–20) 5 (1–16) 3 (1–11) 5 (1–20) 5 (3–17) 6 (1–11)Median number of active joints 1 (0–26) 2 (0–20) 0 (0–26) 0 (0–12) 0 (0–4) 1 (0–6)Patients in remission (%) 9 (7.9) 1 (4.9) 1 (2.9) 3 (8.6) 1 (16.7) 1 (1.1)Median EMS (min) 0 (0–120) 0 (0–120) 0 (0–120) 0 (0–60) 0 (0–15 0 (0–10)Median ESR (mm) 55 (7–40) 75 (7–132) 40 (22–80) 48 (12–140) 58 (49–122) 42 (12–105)Median parent’s/patient’s global assessmentin 100 mm VAS

50 (0–100) 50 (0–100) 20 (0–100) 40 (0–100) 50 (0–100) 50 (0–100)

Median physician’s global assessmentin 100 mm VAS

40 (0–100) 40 (0–90) 20 (0–100) 40 (0–100) 30 (0–100) 30 (0–90)

All parameters where median is shown, the value in parentheses represents the range

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(IA) steroid. Seventy two patients were on DMARD;Methotrexate was the commonest DMARD used—67patients were on it as monotherapy. One patient each was onetanercept, leflunomide, and sulphasalazine. Two patientswere on combination DMARDs.

The male to female ratio was 1.7:1. SoJIA patients weresignificantly younger than polyarticular subtypes at diseaseonset (RF+: p<0.01; RF−: p<0.001). Age of onset wasearlier in patients with oligoarticular-extended diseasecompared to other subtypes ( p<0.05; Table 1).

Disease activity Number of active joints varied from 0–26(Median 1). Disease activity parameters among differentsubtypes were comparable (Table 1) except SoJIA patientshaving significantly higher ESR than RF− polyarticularsubtype ( p<0.05). Patients in remission were very few(Table 1).

JADI-A On JADI-A scoring, 54 patients (60.7%) haddamaged joints. JADI-A score varied from 0–61 with amedian score of 2. Poly RF+ ( p<0.05) and Poly RF− ( p<0.01) subtypes had significantly higher JADI-A thanpersistent oligoarticular subtype ( p<0.05; Table 2). Apattern of damaged joints are shown in Fig. 1.

JADI-E Thirty-five (39.3%) patients had extra-articulardamage. Growth failure was the commonest extra-articulardamage seen (Table 2)

Disability There was no disability (CHAQ=0) in 40.4%patients; mild disability (>0 and ≤0.5) in 14.6%; moderatedisability (>0.5 and ≤1.5) in 22.5% and severe disability(> 1.5) in 22.5%. Disability was similar in all groups (Table 3).

Radiological damage Radiology was available in 58patients; radiological damage was present in 25 patients(43.1%). Radiological damage was commonest in poly-articular RF+ and was least common in SoJIA (Table 3).

Loss of school years Eighty four (94.4%) patients wereeducated. Fifty one (57.3%) patients lost some years ofeducation, with number of years lost due to disease rangingfrom 0–17 (median 1) years (Table 3). Forty-two patients(47.2%) lost 1–5 years; six patients (6.7%) lost 5–10 yearsand three patients (3.4%) lost >10 years.

Correlation of JADI with other disease variables As shownin Table 4, JADI-A correlated well with physician’s globalassessment, radiological damage, CHAQ, JADI-E, parent’s

0

10

20

30

40

50

60

70

Hip Knee Ankle MTP

SoJIA Poly RF- Poly RF+ Oligo-P Olgo-E

0

10

20

30

40

50

60

70

TMJ Cx Spine Shoulder Elbow Wrist MCP PIP

A

B

% o

f patients

% o

f patients

Fig. 1 The bar diagram showsthe distribution of damagedjoints in different subtypes ofjuvenile idiopathic arthritis

Table 2 Extra articular damage in JIA

Extra articular damage Frequency (%)

Growth failure 61 (39.3)Pubertal delay 18 (20.2)Abnormal vertebral curve 5 (5.6)Leg length difference 3 (3.3)Muscle atrophy 3 (3.3)Subcutaneous atrophy 2 (2.2)Osteoporosis 2 (2.2)Striae rubrae 1 (1.1)Amyloidosis 1 (1.1)AVN 1 (1.1)

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global assessment, duration of disease, loss of educationyears due to disease ( p<0.01, Table 4). It also correlatedwell with parameters of disease activity like active jointcount, morning stiffness and ESR ( p<0.01).

When JADI was 0 (N=35), patients still had disability—mild, moderate, and severe disability was seen in 20.6%,17.6%, and 5.9%, respectively. This seems to be due todisease activity as 20 of the 35 patients with zero score inJADI and without active joint had no disability.

Discussion

Our study shows that at median disease duration of 5 years,60% of patients with JIA have articular damage and 39.3%had extra-articular damage. Moderate to severe limitation offunction was present in 45% patients. Though the pattern ofdamaged joints was different among different subtypes,there was no significant difference in overall functionallimitation. More than half of the patients lost some years ofeducation because of disease.

Ours is the first study that has used JADI to assessoutcome in patients with JIA in India. JADI is a simple,easy to use clinical tool without the need of any instrument

and X-ray, which makes it practical for use in the clinicalsetting. Median JADI-A score in our study is comparable tothe previous study by Viola S et al. [8] at a median durationof disease of 5 and 7.3 years, respectively.

Our data suggests that articular damage in children withpersistent oligoarticular subtype of JIA is lower thanpolyarticular JIA and SoJIA. However, CHAQ in allsubtypes of JIA is similar. This may be due to preferentialdamage of lower limb joints in oligoarticular disease, whichcontributes to significant functional disability in CHAQscore. The degree of disability in our other subtypes of JIApatients mirrors that found in other studies [7, 8].

More than half of our patients lost some education yearsbecause of disease. This is higher compared to the westerndata. Only 3% of patients were behind expected grade in astudy from Canada [13]; the educational attainments ofadults with JIA were comparable to controls in UK [2] andFinland [14]. The difference in our study may be due to lackof social support for education in patients with arthritis.

JADI-E scores in our study were comparable to theprevious study [8]. However, we found a higher preva-lence of growth failure and lower prevalence of oculardamage. Higher growth failure could be due to coexistentmalnutrition and poor socio-economic status. None of ourpatients had ophthalmological damage in contrast to 10%reported earlier [8]. In another study from the UK [7],10.2% had cataracts and 10.6% of patients required eyesurgery. It is probably due to under-representation of theoligoarticular group in our study as compared to 55% inthe UK study.

The prevalence of patients with amyloidosis is also lowin our study compared to 8.9% reported in the study fromUK. However, they had used a very sensitive technique ofserum amyloid protein scans as well as the median diseaseduration was 23 years in contrast to 5 years in our study [7].Our data is comparable to another study involving 215patients where prevalence of amyloidosis was very low[15]. It may also reflect the change in treatment paradigm inthe current years.

Similar to previous long-term outcome studies [14, 16]50% of all patients in this study also continued to have

Table 3 Outcome in different subtypes of JIA [Median (range)]

All subtypes Systemic onset Poly RF+ Poly RF- Oligo-extended– Oligo-persistent

Number of joints with LOM 2 (0–34) 2 (0–16) 3 (0–26) 2 (0–34) 1 (0–3) 2 (0–3)% with radiological damagea 43.1 22.22 66.67 42.30 33.3 40Number of education years lost 1 (0–13) 1 (0–10) 0 (0–5) 0 (0–13) 1 (0–1) 1 (0–3)CHAQ 1 (0–3) 2 (0–3) 1 (0–3) 1 (0–3) 0 (0–3) 2 (0–3)JADI-A 2 (0–61) 2 (0–27) 4 (0–44) 2 (0–61) 0 (0–5) 0 (0–5)JADI-E 1 (0–10) 1 (0–4) 1 (0–2) 0 (0–8) 0 (0–2) 0 (0–2)

a Radiography done in 58/89 patients

Table 4 Correlation of JADI-A in JIA (2 tailed Spearman’scorrelation coefficient)

Parameter rS

Physician’s global assessment 0.623**Radiological damage 0.538**CHAQ 0.567**JADI-E 0.513**Parent’s global assessment 0.446**Duration of disease 0.385**Loss of education 0.352**ESR 0.286**Early morning stiffness 0.258*No. of active joints 0.293**

*p<0.05**p<0.01

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detectable inflammation. This does not support the conceptthat JIA becomes less inflammatory with time and ‘burnsout’.

In conclusion, even with improved treatment modalitiescurrently available, articular, and extra-articular damage arecommon in JIA leading to functional disability. Further,JADI has a good correlation with traditional outcomemeasures in JIA and may be a good tool to use in clinicalpractice.

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