Can familial Mediterranean fever (FMF) cause chest pains with dyspnea?

Familial Mediterranean Fever Can Cause Chest Pain with Dyspnea

Yes, chest pain with dyspnea can occur in Familial Mediterranean Fever (FMF) due to pleuritis and, in rare cases, pericardial involvement including cardiac tamponade.

Clinical Manifestations of Chest Pain in FMF

FMF is characterized by recurrent attacks of fever and serosal inflammation, which can affect various body sites including the pleura and pericardium. When the pleura is involved, patients may experience:

• Pleuritic chest pain (sharp pain that worsens with breathing)
• Dyspnea (shortness of breath)
• Reduced chest expansion
• Pleural effusion (detectable on imaging)

Pleuritis in FMF

Pleuritis is a well-documented manifestation of FMF attacks 1. During these episodes:

• Pain is typically unilateral but can be bilateral
• Symptoms usually last 12-72 hours (typical duration of FMF attacks)
• Pleural effusions may develop and can be detected on chest imaging
• Symptoms often resolve spontaneously but can recur

Pericardial Involvement

Though less common than pleuritis, pericardial involvement can occur in FMF and may present as:

• Chest pain with radiation to shoulders or back
• Dyspnea that worsens when lying flat
• Tachycardia
• In severe cases, cardiac tamponade (a rare but documented complication) 2

Diagnostic Considerations

When a patient with known or suspected FMF presents with chest pain and dyspnea:

1. Differentiate from other causes: The EULAR guidelines emphasize that when suspecting an FMF attack with chest symptoms, clinicians should "always consider other possible causes" 1

2. Monitor acute phase reactants: Check C-reactive protein (CRP) and serum amyloid A (SAA) levels, which are typically elevated during attacks

3. Imaging: Consider chest radiography or other relevant imaging to detect pleural effusion or pericardial involvement

4. Recurrent pattern: Note that recurrent, self-limiting episodes of pleuritic chest pain that resolve within 1-3 days are characteristic of FMF 3

Management Approach

For FMF patients experiencing chest pain with dyspnea:

1. Continue colchicine: Maintain regular colchicine dosing during attacks (3 mg daily in adults, 2 mg daily in children) 1, 4

2. Symptomatic relief: Use NSAIDs (except aspirin) for pain management during attacks 1, 4

3. Monitor for complications: Particularly in severe cases with significant dyspnea, monitor for development of large effusions or cardiac tamponade 2

4. Consider treatment escalation: For patients with frequent or severe pleuropericardial attacks despite optimal colchicine therapy, consider IL-1 inhibitors like canakinumab 5

Special Considerations

• Colchicine resistance: In patients with recurrent chest symptoms despite adequate colchicine therapy, consider adding biological agents, particularly IL-1 inhibitors 6

• Differential diagnosis: Recurrent pleural effusion may be the first manifestation of FMF before other classic symptoms appear 3

• Monitoring: Regular follow-up every 6 months is recommended to assess disease activity and response to treatment 1

FMF should be considered in the differential diagnosis of recurrent pleuritic chest pain with dyspnea, especially in patients of Mediterranean descent (Jewish, Armenian, Turkish, and Arab populations) 7. Early recognition and appropriate treatment can prevent recurrent episodes and complications.