Introduction
The most prevalent chronic rheumatic condition in children, juvenile idiopathic arthritis (JIA), has an unclear cause. Estimating that 20% of JIA populations have Polyarthritis, approximately 85% of the polyarthritis populations have Rheumatoid Factor (RF)- Negative.1, 2 Annual incidence & prevalence for RF negative polyarthritis can be estimated as 1 to 4 per 100,000 and 21 to 37 per 100,000 respectively. 3 RF negative Polyarthritis girls are affected 4 times more frequently than the boys.4 JIA includes a number of distinct subgroups and typically manifests as peripheral arthritis. There are many different clinical states on the illness spectrum. It has been demonstrated that both endogenous and external antigens with heightened inflammatory responses are crucial in the pathophysiology of the illness. The patient's daily activities and productivity are restricted by chronic inflammation. In order to be diagnosed with JIA, a patient must have the disease's inception before the age of 16 years where there is biphasic peak of onset, one at 1-4 years & other at later childhood & adolescence and have arthritis that has persisted for more than six weeks.4, 5, 6 Juvenile rheumatoid arthritis (JRA) and juvenile chronic arthritis (JCA) are other names that have been used for the same clinical entity. 7 There is symmetric involvement of joints.
Case Report
Physical & clinical examination
Fixed Flexion Deformity (FFD) of 20 degrees & Fixed Abduction Deformity of 15 degrees present on Right Hip
Management
Roentgenographic examination
On Xray of Hand :- Soft tissue swelling, osteopenia, Loss of articular cartilage & destructive changes in the distal & proximal interphalangeal (i.e. DIP & PIP) joints and metacarpophalangeal (MCP) joints (Figure 3)
On Xray of Wrist:- Erosions at the radial articular surface & metacarpal bases with crenated margins with destruction and fusion of carpal bones in the wrist (Figure 3)
On Xray of Cervical Spine:- Ankylosis, Loss of normal cervical lordosis, Narrowing & erosion of neural arch joints (Figure 4)
On Xray of Bilateral Hip with Pelvis:- Uniform joint space narrowing with Destruction of femoral head & acetabula, fusion of Sacro-Iliac joint (Figure 5)
On Xray of Bilateral Knee Joint:- Soft tissue swelling , Osteopenia , Joint space narrowing (Figure 6)
Treatment
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Disease - Modifying Anti-Rheumatic Drugs (DMARDs): 10, 9, 11
Tab. Folic Acid 5 mg: 1 tab once daily except on the day of taking Methotrexate. 10, 8
Tab. Deflazacort 6mg: 8, 9, 121 tab (12mg) once daily after food * 4 monthsThen, 1 tab (6mg) once daily after food * 4 monthsThen, 1 tab (6mg) on alternative day after food * 4 months & then STOP.* Before starting DMARD therapy, LFT, KFT, CXR (PAV), Virology were done.* After every 12 weeks of above treatment LFT, KFT are being repeated to monitor the side-effects of DMARDs.
Follow – Up
The patient is reviewed at 4 weeks interval to know the disease progression. On Follow-up at 4 weeks, there is decreased ESR (40 mm AEFH), CRP Negative. The symptoms has subsided than before. The patient is also reviewed on every 12 weeks intervals to rule out progression to Sero positive adult Rheumatoid arthritis factor. The patient is also being monitored for the adverse effects of the drugs causing hepatic & renal impairment because of DMARDs.
Conclusion
The importance of early recognition of clinical features in the diagnosis allows correct treatment, planning & reduces the chance of disease progression & complications in future, thus ensuring a better quality of life to the patient. It allows aggressive treatment to prevent irreversible joint damage. As a result, it helps patient to live a healthy life.