Anti-IL-1 treatment for secondary amyloidosis in an adolescent with FMF and Behçet's disease.
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 Behçet'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.
Department of Pediatrics, Nephrology and Rheumatology Unit, Hacettepe University School of Medicine, Sihhiye, 06100, Ankara, Turkey.
Yelda
Y
Bilginer
Nuray Aktay
NA
Ayaz
Seza
S
Ozen
D000293:Adolescent; D000686:Amyloidosis / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology*; D018501:Antirheumatic Agents / Q000627:therapeutic use; D001528:Behcet Syndrome / Q000150:complications* / Q000188:drug therapy; D003078:Colchicine / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D010505:Familial Mediterranean Fever / Q000150:complications* / Q000188:drug therapy; D005260:Female; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use*; D011507:Proteinuria / Q000188:drug therapy / Q000209:etiology; D016896:Treatment Outcome
D018501:Antirheumatic Agents; D053590:Interleukin 1 Receptor Antagonist Protein; D003078:Colchicine