PAFLAR Guidelines for Polyarticular Juvenile Idiopathic Arthritis Save

The Pediatric African League against Rheumatism (PAFLAR) initiative has published its guidelines for the diagnosis and treatment of polyarticular juvenile idiopathic arthritis (pJIA).

The ACR last published recommendations for JIA (for Non-Systemic Polyarthritis, Sacroiliitis, and Enthesitis) were in n 2019.

These consensus based (Delphi method) guidelines were based on 15 key clinical questions according to the PICO (Patient/Population, Intervention, Comparison, Outcome) approach. They surveyed a voting panel made up of twenty pediatric rheumatologists and adult rheumatologists who are experts and interested in pediatric rheumatology. 

A total of 15 recommendation points were identified, divided into two parts: five recommendations for diagnosis and ten recommendation is the most common rheumatologic disease of childhood.   

Below are the proposed guidelines for children with polyarticular JIA.

Overarching Principles

• Diagnosis of polyarticular JIA should be based on the clinical assessment of an expert rheumatologist to facilitate the diagnosis and exclude other differential diagnosis
• Musculoskeletal ultrasound could be used in diagnosis, monitoring the disease activity, and assessment of response to treatment
• The aim of the treatment is to reach clinical and ultrasound remission or low disease activity based on a shared decision between the rheumatologist and the child parents
• Management of polyarticular JIA may need multiple successive therapies either csDMARDs or bDMARDs, modified according to the disease activity, response to treatment, safety issues, comorbidities and progression of structural damage

Guidelines

1. Polyarticular JIA should be suspected, and the child should be referred to a pediatric rheumatologist if child was presented with chronic arthritis affecting 5 or more joints
2. Plain X-rays, MSUS, and MRI are important radiological investigations which help in the diagnosis of polyarticular JIA
3. Diagnosis of polyarticular JIA is based on diagnosis of expert pediatric rheumatologist after clinical examination and using laboratory and radiological evaluation guided by the 2001 ILAR Classification Criteria for JIA in children who have arthritis affecting 5 or more joints during the first 6 months of disease
4. Polyarticular JIA should be differentiated from other inflammatory and non-inflammatory arthropathies in children
5. Uveitis may occur in RF-negative poly arthritis especially in ANA positive children
6. The main treatment target is to achieve clinical and/or ultrasound remission, with the alternative target of low disease activity is recommended
7. Non-pharmacologic interventions could be used to optimize supportive care in JIA patients using physical therapy, occupational therapy nutrition, and surgical intervention
8. NSAIDs could be used as first-line for pain relief and symptom management, and corticosteroids could be used in polyarticular JIA as a bridge therapy, intra-articular injection in resisted joints, or as IV infusion (in severe resisted cases)
9. Starting with csDMARDs (MTX is the first choice) should be considered as first-line in patients with polyarticular JIA
10. The choice of second-line management depends on disease activity, response to initial treatments, individual patient characteristics and prognostic factors. Escalation of MTX dose or using combined csDMARDs should be considered, biological therapy could be used as second line of treatment in presence of poor prognostic factors
11. Biological DMARDs should be adopted as third-line management after failure of csDMARDs as mono or combined therapy
12. In moderate or severe active polyarticular JIA who do not respond to csDMARDs, biological therapy is recommended in the form of: anti-TNFs, tocilizumab, abatacept and tofacitinib. The usage of biosimilars in African countries provides an important opportunity to treat more JIA children with biologic drugs due to lower cost and similar efficacy
13. High-risk polyarticular JIA patients who should proceed to 2nd line or 3rd line immediately are those who have poor prognostic factors
14. The child should be assessed clinically, radiographically, and functionally to assess disease activity and response to treatment
15. Stop or withdraw medication depends upon response to the medication, tolerance of the administration regimen, and other patient factors