VIMS Journal: July 2016

Original Article

Juvenile Idiopathic Arthritis - A Clinic Based Study

Dr. S. Guha, Dr. S. R. Pal, Dr. Indranil Das

Abstract :
Introduction :
Arthritis is an inflammation of the joint cavity[1]. It may be directly or indirectly caused by infectious agent, it may be idiopathic or may be associated with other diseases.[2] Early diagnosis and treatment is necessary to prevent chronic life threatning and debilitating conditions[3,5].

Key words : JIA

Aims and Objectives :
Data on Juvenile Idiopathic Arthritis (JIA) is lacking from Eastern India[4]. This study is an attempt to describe early disease, patient characteristics, microbiological features and immunological factors in children with different subgroups of childhood arthritis using a clinic based approach.

Study Design:
This is a cross sectional observational study. Pediatric patients attending the rheumatology clinic of Vivekananda Institute of Medical Sciences were enrolled from March 2013 to clarification September 2014 and classified as per ILAR (International League Against Rheumatism)[1].

Study Population :
Children less than 16 years of age with possible or evident arthritis determined on the basis of one or more of the following criterion, described as inclusion criteria.

Inclusion Criteria :
Children < 16 yrs. of age with at least one of the following 3 signs-
1. Swelling of joint.
2. Restricted mobility of a joint with warmth, tenderness or pain.
These should atleast last for a period of 6 weeks.

Exclusion Criteria :Patients whose complaints are due to an obvious local cause like trauma or who are suffering from non rheumatological disorders (malignancy, hemophilia, sickle cell anemia) and patients of rheumatic fever are excluded from this study.
Post Streptococcal Reactive Arthritis (PSRA) and post viral reactive arthritis may be classified as undifferentiated arthritis as per ILAR classification. These patients fit the inclusion criteria of having arthritis of >6 weeks.

Methodology :
After careful history taking patients were subjected to meticulous musculoskeletal ophthalmological and systemic examination. DAS 28[15] and JAFAS[16] were noted.
Besides routine investigations all patients were tested for rheumatoid factor (RF), ASO titre by latex agglutination anti nuclear antibody(ANA) using indirect immunoflurosence, and HLAB27 assay were also done. Among imaging studies, Echocardiography, ultrasonography power Doppler and magnetic radio imaging (MRI) were performed. Slit lamp examination of the eyes were performed in all subjects.

Of the 26 patients diagnosed as JIA using ILAR classification 3 presented with oligoarticular arthritis, 5 were systemic in onset, 9 of polyarticular variety, 7 were enthesitis related arthritis (ERA), 1 PSRA, 1 post viral reactive arthritis. There was a male preponderance (8 of 9) in polyarticular variety and in ERA. Systemic onset variety and oligoarticular arthritis had a female predominance. Majority of the JIA in our study had a later age of onset (>10years) except the systemic onset variety which showed an earlier presentation (earliest being 3 months). The PSRA and post viral arthritis were oligo articular in onset and mean age of presentation were similar.
50% of the cases were below 3rd percentile of height for age (WHO criteria). This was distributed through out all the subtypes commonest with systemic onset variety. The total joint scores varied according to age of onset and type of disease. They were highest in systemic onset variety (SOJIA) and in poly articular subtypes.
All the SOJIA patients had fever ranging from moderate to high grade, mostly of the classical quotidian variety. Evanescent maculopapular rash was universally present. 3 of the 5 SOJIA patients had generalized lymphadenopathy and hepatosplenomegaly.
Pan uveitis was present in one case of oligoarticular JIA. One patient with ERA had anterior uveitis. RF was positive in one case of polyarticular JIA.
ANA was positive in low titre (1:80) in one case of oligoarticular JIA with panuveitis. HLA-B27 was positive in 4 out of 7 cases of ERA, the youngest being 7 years of age.
One child with polyarticular JIA had overlap with juvenile dermatomyositis had exhibited gottron papule and heliotrope rash. Muscle enzymes (creatinine phosphokinase, CPK, aldolase) were elevated and there was evidence of muscle inflammation in MRI (T2). USPD showed enthesitis in 2 cases of ERA. MRI done over one year follow up picked up bilateral sacroilitis in 2 cases of ERA.
Echo cardiography revealed grade 2 mitral regurgitation(MR) and grade 1 mitral valve prolapse in one case of oligoarticular variety, and grade 2 MR was seen in PRSA. None of the patients had any cardiovascular symptoms.

JIA is the most common cause of arthritis in children and represents upto 65% of arthritic disease in children [6]. Various epidemiological studies of JIA report divergent results owing to heterogenecity of the disease, differnces in standerdised diagnostic criteria, patient retrieval and study designs [7-10]. Although JIA is a rare disease, its true frequency is not known in our country. In the west its incidence is reported to be 6-8/100,000 population per year [10].
1. JIA has been divided into various subgroups, and this categorization helps in diagnosis, follow up and subsequent care of these children. Like other indian studies [9, 10] polyarticular variety was the commonest in our study though Singh and colleuges reported oligoarticular variety to be more commoner from the northern part of the country [11]. This was closely followed by ERA. Unlike North America and Europe where oligoarticular represents 50-75% cases of JIA, this variety was the least common in our study, an observation similarly reported by Aggarw et al [11].
2. The mean age of onset in the west is usually 1-3 years[10] and rare below 6 months. It appears that in India JIA has a later age of onset. An indian study by Kabra et al[9] showed that the mean age of onset aws earliest in SOJIA (5.2YRS), followed by oligoarticular (6.8yrs) and polyarticular (7.2 yrs) subtypes. The overall onset of disease in our study tended to peak in later age groups. The mean age of onset was lowest in SOJIA, with the earliest case report being 3 months.
3. Regarding sex, males predominated over females in all subtypes like other indian studies [9, 10]. However females predominated in oligoarticular variety and SOJIA, unlike most indian observations. The male preponderance of JIA in this part of the subcontinent may be due to the special characteristic of the disease seen in India; another explanation may be that boys in our country are cared more by family members and hence brought early for treatment.
4. Unlike studies from western countries [13] we had a very low incidence of uveitis, a similar finding reported from most parts of the country [10, 4]. Uveitis could be only detected in 2 case inspite of thorough slit lamp examination of eyes of all patients. One was an oligoarticular ANA+ case and the other was an HLA-B27 + ERA variety. Probably occurrence of uveitis is closely related to ANA+vity() which is again reported to be very low in indian children.
5. RF+vity was seen in only 1 child with polyarticular variety. RF +vity is seen only in 15-20% of JIA cases[6]. None of our children had rheumatoid nodules.
6. Of all the 26 cases of JIA 7 were ERA. This finding was at par with other indian studies[19]. This is in contrast to a similar clinic based study from Eastern India by ghosh et al[4] where only 2 cases of ERA were reported out of 50 JIA cases. Studies from the west[18] report that 6- 19% of JIA cases are ERA. Barring one, all were males and >10 years of age. Only one had a family history of spondyloarthropathy. Of the 7 cases of ERA, 6 presented with peripheral arthritis, 2 had history of axial involvement. Enthesitis was present in 2 cases as demonstrated by USPD. 2 patients had bilateral sacroilitis in MRI over 1 year follow up. HLA-B27+vity was present in 4 cases. This observation is at par with a study on ERA in indian children by Agarwal. M et al[19] which showed a higher proportion of HLA-B27+VITY, early onset axial involvement, and paucity of family history, unlike their western counterparts.
Heart disease is a rare complication of JIA. None of the patients had any history of breathlessness or palpitations. Grade 2 MR was seen in the sole child with PRSA. This finding was not diagnostic of rheumatic carditis. In absence of any control group this finding cannot be taken as significant.

Types of JIA and its distribution :

Types of JIA and its distribution

Table 1: Characteristics of JIA according to different relevant clinico- pathological factors:
JIA Number of cases(%) Male female ratio Mean age of onset Rash Hepatosplenomegaly Lymphadenopathy Uveitis RA factor ANA HLA B-27
Polyarticular 9(35) 8:1 9.5yrs - - - - 1 - -
SOJIA 5(19) 1:4 9.5yrs 5 3 3 - - - -
Oligoarticular 3(11) 1:3 9.5yrs - - - 1 - 1 -
ERA 7(27) 6:1 9.5yrs - - - 1 - - 4
PSRA 1(4) All F 9.5yrs - - - - - - -
PVRA 1(4) All M 9.5 yrs - - - - - - -

Table 2: Clinico-Immunological Profile of JIA in Other Studies
Clinicoimmunological Present study Singh et al Casidy et al. (1) Seth et al. (2) Parkodi et al. (4)
M:F ratio - 1.8:1 1:2 1.3:1 1.6:1
Pauciarticular (%) 11 42.2 50 30 49
Polyarticular (%) 35 37.8 40 46 41
Systemic (%) 19 14.8 10 24 10
Rheumatoid nodules (%) - 5.4 10 0.8 3
Uveritis (%) 7.7 1.3 5 1.1 3
ANF* (%) 3.8 0 45 6.5 -
Rheumatoid factor* (%) 3.8 0 10 15 9.7

Conclusion :
JIA is the commonest cause of chronic arthritis in children. Data from Eastern India is lacking. Ours was a pilot study attempting to study the incidence reporting at a tertiary care hospital. The limitation was the small sample size and short study period. Like other Indian studies polyarticular was the commonest with a male predominent followad closely by ERA. In future more data need to be collected over prolonged period, to show the pattern of JIA from Eastern India.

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