How to manage chest pain in a pediatric patient with juvenile rheumatoid arthritis (JRA)?

Management of Chest Pain in Juvenile Rheumatoid Arthritis

Chest pain in a child with JRA requires immediate evaluation for life-threatening serositis (pericarditis, pleuritis, myocarditis), which occurs primarily in systemic-onset JRA and demands urgent cardiac assessment with echocardiography and consideration of escalating immunosuppressive therapy.

Immediate Assessment and Risk Stratification

Identify the JRA Subtype

• Systemic-onset JRA carries the highest risk for serious chest pain etiologies including pericarditis, pleuritis, and myocarditis, which are recognized extra-articular manifestations of this subtype 1, 2
• Polyarticular and oligoarticular subtypes rarely present with serositis, making musculoskeletal chest wall pain more likely in these patients 1

Urgent Cardiac Evaluation

• Obtain echocardiogram immediately if systemic-onset JRA is present, as pericarditis can progress to cardiac tamponade and represents a medical emergency 1
• Assess for fever spikes, pericardial friction rub, muffled heart sounds, and signs of cardiac compromise (tachycardia, hypotension, jugular venous distension) 1, 2
• Myocarditis, though less common, can occur in systemic-onset disease and requires ECG and troponin evaluation 1

Evaluate for Pleuritis

• Examine for pleuritic chest pain (sharp, worse with deep breathing), decreased breath sounds, and pleural friction rub 1
• Chest radiograph is indicated to assess for pleural effusion in systemic-onset patients with chest pain 1, 2

Treatment Algorithm Based on Etiology

For Confirmed Serositis (Pericarditis/Pleuritis)

• Escalate immunosuppressive therapy immediately rather than relying on NSAIDs alone, as serositis indicates inadequately controlled systemic disease 3
• IL-1 and IL-6 inhibitors are strongly recommended over conventional synthetic DMARDs for systemic JIA with inadequate response to NSAIDs and/or glucocorticoids 3
• Consider short-term systemic glucocorticoids for acute severe serositis while initiating or escalating biologic therapy 3
• Avoid long-term glucocorticoid monotherapy; biologic DMARDs or csDMARDs are strongly recommended over prolonged glucocorticoid use 3

For Musculoskeletal Chest Wall Pain

• NSAIDs remain first-line for chest wall pain related to costochondral joint involvement or muscle inflammation 3
• Assess whether current disease-modifying therapy is adequate; chest wall involvement may indicate undertreated polyarticular disease requiring methotrexate or biologic escalation 3
• Physical therapy can address chest wall muscle tightness and improve respiratory mechanics 4

Pain Assessment and Monitoring

Standardized Pain Measurement

• Use Visual Analog Scale (VAS) for children ≥8 years to quantify chest pain intensity (0-100 scale: 10-30 mild, 40-60 moderate, 70-100 severe) 5
• For younger children (≥4 years), employ Faces Pain Scale-Revised (FPS-R) for developmentally appropriate assessment 5
• Assess pain at each visit to monitor treatment response and guide therapy escalation decisions 5

Multidimensional Assessment for Persistent Pain

• Consider Bath Adolescent Pain Questionnaire (BAPQ) for children ≥8 years with chronic chest pain to capture functional impact beyond intensity alone 5
• Parent proxy forms are available and valuable when the child cannot provide reliable self-report 5

Critical Pitfalls to Avoid

Delayed Recognition of Serositis

• Never attribute chest pain to musculoskeletal causes alone in systemic-onset JRA without excluding pericarditis and pleuritis through imaging 1, 2
• Subclinical pericarditis may be present without obvious clinical signs; maintain low threshold for echocardiography 1

Inadequate Disease Control

• Chest pain from serositis or extensive joint involvement signals treatment failure requiring immediate escalation rather than symptomatic management alone 3
• An adequate trial of methotrexate is 3 months, but changing or adding therapy is appropriate if minimal response after 6-8 weeks 3

NSAID Masking

• High-dose NSAIDs may partially suppress serositis symptoms, creating false reassurance while underlying inflammation persists 2
• Do not rely on NSAID response alone to rule out serious pathology in systemic-onset disease 2

Treatment Escalation Pathway

For Inadequate Response to Current Therapy

• Biologic DMARDs are strongly recommended if inadequate response to NSAIDs/IAGCs and at least one csDMARD in oligoarticular or polyarticular disease 3
• For systemic JIA with persistent symptoms, switch to or add IL-1 or IL-6 inhibitors rather than continuing inadequate conventional therapy 3
• Early treatment intensification is critical to prevent permanent damage and improve long-term outcomes 3