What is the best course of management for a 19-year-old female with a history of Parry Romberg syndrome, currently experiencing ankle pain, knee fatigue, and shoulder pain without swelling, who has been taking ibuprofen (nonsteroidal anti-inflammatory drug)?

Management of Polyarticular Joint Pain in a Young Adult with Parry-Romberg Syndrome

Immediate Clinical Assessment Required

This patient requires urgent rheumatologic evaluation to rule out inflammatory arthritis, particularly given the bilateral ankle involvement, knee fatigue, and shoulder pain in the context of an autoimmune condition (Parry-Romberg syndrome). 1

The key concern here is distinguishing between mechanical joint pain versus new-onset inflammatory arthritis, as Parry-Romberg syndrome has documented rheumatologic associations 1. The absence of swelling does NOT exclude inflammatory arthritis—many inflammatory conditions present initially with arthralgia before frank synovitis develops 2.

Diagnostic Workup

Obtain the following laboratory studies immediately:

• Inflammatory markers: ESR and CRP to assess for systemic inflammation 2
• Autoantibody panel: ANA, RF, anti-CCP to evaluate for rheumatoid arthritis or other connective tissue disease 2
• Creatine kinase (CK): To rule out myositis, which can present with joint pain and fatigue 2
• Complete blood count and comprehensive metabolic panel: Baseline assessment 2

Imaging considerations: Plain radiographs of affected joints (ankles, knees, shoulder) to assess for structural changes, with ultrasound or MRI if inflammatory arthritis is suspected 2.

Immediate Management Strategy

Continue NSAIDs with Monitoring

Continue ibuprofen at an adequate anti-inflammatory dose (up to 2400 mg/day divided) for 4-6 weeks while awaiting rheumatologic evaluation 3. The patient has already initiated NSAID therapy, which is appropriate first-line treatment 2.

• Monitor for gastrointestinal side effects, particularly if prolonged use is needed 2
• Consider gastroprotection if risk factors present (though age <75 years is lower risk) 2

If NSAIDs Provide Insufficient Relief

Consider low-dose prednisone 10-20 mg daily for 2-4 weeks if NSAIDs are ineffective 3. This approach is supported for inflammatory joint pain when NSAIDs alone are insufficient.

Escalation for Moderate-to-Severe Symptoms

If pain worsens or functional impairment increases:

• Temporarily hold any potential myotoxic medications (though none mentioned in this case) 3
• Increase prednisone to 20 mg daily, with potential escalation to 1 mg/kg/day if symptoms persist 3
• Expedite rheumatology referral for consideration of disease-modifying therapy if inflammatory arthritis is confirmed 2

Critical Differential Diagnoses to Exclude

Inflammatory Arthritis Associated with Autoimmune Disease

Parry-Romberg syndrome has documented associations with autoimmune conditions 1. The bilateral ankle involvement with shoulder pain suggests:

• Reactive arthritis pattern: Oligoarthritis affecting large joints (ankles, knees) 2
• Early rheumatoid arthritis: Polyarticular involvement, though typically more symmetrical 2
• Polymyalgia-like syndrome: Proximal fatigue (knees, shoulder) with elevated inflammatory markers but normal CK 2

Myositis

Weakness versus pain is the critical distinction 2:

• Myositis presents with proximal muscle weakness (difficulty standing, lifting arms) 2
• This patient describes pain and fatigue, not true weakness 2
• Normal CK would effectively rule out myositis 2

Mechanical/Degenerative Causes

Less likely given age (19 years) and acute onset (2 days), but consider:

• Overuse injury or trauma (obtain detailed activity history) 2
• Crystal arthropathy (gout/pseudogout)—uncommon at this age but possible 2

Red Flags Requiring Urgent Intervention

Seek immediate medical attention if any of the following develop:

• True muscle weakness (inability to stand from sitting, lift arms)—suggests myositis requiring urgent treatment 2
• Severe, uncontrolled pain—may require hospitalization for pain control 2
• Systemic symptoms: Fever, significant fatigue, weight loss—suggests active systemic inflammation 2
• Chest pain or dyspnea—myositis can involve cardiac muscle 2

Parry-Romberg Syndrome Considerations

While Parry-Romberg syndrome is typically a self-limiting facial hemiatrophy 4, 5, 1, the documented rheumatologic associations mean this patient has increased risk for:

• Autoimmune arthritis 1
• Scleroderma overlap (documented case association) 4
• Other systemic autoimmune manifestations 1

The 3-year remission of facial symptoms does NOT preclude new systemic manifestations 1.

Follow-Up Timeline

• Rheumatology appointment within 1-2 weeks if inflammatory markers elevated or symptoms persist despite NSAIDs 2
• Re-evaluation in 4-6 weeks if symptoms resolve with NSAIDs alone 3
• Immediate return if red flag symptoms develop 2

Common Pitfalls to Avoid

Do not dismiss this as simple overuse or mechanical pain given the autoimmune history and polyarticular involvement 2, 1. The absence of visible swelling does not exclude inflammatory arthritis—many patients have arthralgia before developing frank synovitis 2.

Do not delay rheumatologic evaluation if inflammatory markers are elevated or symptoms persist beyond 4-6 weeks of NSAID therapy 2, 3.

Do not use intra-articular corticosteroid injections as initial therapy for polyarticular involvement—this is reserved for monoarticular or oligoarticular disease 2.