Anti-IL-1 treatment for secondary amyloidosis in an adolescent with FMF and Behcet's disease


BİLGİNER Y., AKTAY AYAZ N., ÖZEN S.

CLINICAL RHEUMATOLOGY, cilt.29, sa.2, ss.209-210, 2010 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 29 Sayı: 2
  • Basım Tarihi: 2010
  • Doi Numarası: 10.1007/s10067-009-1279-8
  • Dergi Adı: CLINICAL RHEUMATOLOGY
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Sayfa Sayıları: ss.209-210
  • Anahtar Kelimeler: Anakinra, Amyloidosis, Behcet disease, FMF, FAMILIAL MEDITERRANEAN FEVER
  • Hacettepe Üniversitesi Adresli: Evet

Özet

Familial Mediterranean fever (FMF) is associated with mutations in the gene coding for pyrin which lead to accentuated innate immune responses resulting in increased production of IL-1. We present a teenager who had severe FMF and Beh double dagger et's disease and developed moderate proteinuria. Renal biopsy showed secondary amyloidosis. Anakinra was started at 1 mg/kg/day subcutaneously along with colchicine treatment. The clinical response was excellent. Acute phase reactants decreased. The level of proteinuria and renal functions remained stable and the hypoalbuminemia returned to normal. Her clinical and laboratory symptoms returned when anakinra had to be stopped at 6 months. Thus, the drug was restarted and she is now clinically in excellent condition a year after the start of therapy. She has normal renal functions, normal serum proteins, and normal acute-phase reactants. However, recently, after 18 months of anakinra treatment, her proteinuria gradually increased and albumin levels decreased. We suggest that anti-IL-1 treatment is beneficial for the suppression of inflammation; however, long-term studies are needed to understand whether progressive renal disease will be prevented.