Rakiba Belkhir, MD; Luc Moulonguet-Doleris, MD; Eric Hachulla, MD, PhD; Jacques Prinseau, MD; Alain Baglin, MD; Thomas Hanslik, MD, PhD
Potential Financial Conflicts of Interest: None disclosed.
Belkhir R, Moulonguet-Doleris L, Hachulla E, Prinseau J, Baglin A, Hanslik T. Treatment of Familial Mediterranean Fever with Anakinra. Ann Intern Med. 2007;146:825-826. doi: 10.7326/0003-4819-146-11-200706050-00023
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Published: Ann Intern Med. 2007;146(11):825-826.
Background: About 5% to 10% of patients with familial Mediterranean fever (FMF) have no clinical response to colchicine therapy, and no treatment recommendations exist for these cases (1). Interleukin-1 (IL-1) is a major proinflammatory cytokine that is increased in activity by pyrin, which is elevated in FMF (2, 3). Thus, blockade of IL-1 could in theory control the disease.
Objective: To describe a woman with FMF who was refractory to colchicine treatment but who responded clinically to anakinra therapy, an IL-1–receptor antagonist.
Case Report: A 68-year-old woman with FMF was treated with colchicine since her teens. The diagnosis was based on her personal and family history. She was homozygous for the M694V mutation of the MEFV gene. Despite treatment with colchicine, 1 mg/d, she had recurrent episodes of fever, abdominal pain, and polyarthralgia, which notably altered her quality of life. She had anemia attributed to chronic inflammation. Her serum creatinine level was 176.8 µmol/L (2 mg/dL), and she had no proteinuria. A biopsy of her accessory salivary glands disclosed amyloid A amyloidosis. After an increase in fever, asthenia, and abdominal pain, the dosage of colchicine was increased to 1.5 mg/d, without improvement of inflammatory symptoms. The high colchicine dosage led to severe toxic neuromyopathy. Treatment was suspended, which resulted in a worsening of her inflammatory symptoms. The patient developed almost permanent fever, abdominal pain, and back pain. Her C-reactive protein level increased to 118 mg/L, and her serum amyloid A level increased to 864 mg/L (normal level, <15 mg/L). The patient was confined to bed.
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