Introduction: Protracted Febrile Myalgia (PFM) is a rare complication of familial mediterranean fever (FMF) that presents as an excruciating and debilitating muscular pain. The natural history of the attack lasts 6-8 weeks and is characterized by extreme myalgia and high fever, accompanied by elevated inflammatory markers and normal levels of muscle enzymes. Response to analgesics is universally poor. Current management includes oral corticosteroids (prednisone dose of 1-2mg/kg/d) and nonsteroidal anti-inflammatory drugs.
Objective: To describe our experience with pulse glucocorticoids (10mg/kg/d) for the treatment of PFM.
Methods: A retrospective case series of children diagnosed with PFM and treated with pulse glucocorticoids during 2011-2014.
Results: Eight children with PFM were hospitalized in our department during the study period. Four of them had been misdiagnosed and transferred to our medical center from other hospitals. In 5/8 children PFM was the presenting symptom of FMF. Six children were treated with pulse glucocorticoids of 10mg/kg/d for 3 days. All six children experienced a dramatic improvement within hours after completing the first pulse. Inflammatory markers decreased promptly. Steroid tapering was done in the outpatient clinic. All eight children were eventually diagnosed as having FMF with one or two M694V mutation.
Conclusion: PFM is a rare yet significant complication of FMF which causes a severe debilitating clinical syndrome necessitating prolonged hospitalization. A three days` pulse of glucocorticoids results with swift and dramatic clinical and laboratory improvement and accelerated ambulation. The optimal dosage and duration of the pulses as well as steroid withdrawal protocols needs further research.
