Infliximab therapy for familial Mediterranean fever-related amyloidosis: case series with long term follow-up.
Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent self-limited attacks of fever and polyserositis. Reactive amyloid A amyloidosis is the most devastating complication of FMF, and amyloidosis continues to occur in the colchicine era in untreated and noncompliant patients. Unfortunately, there is no proven effective treatment for established amyloidosis. In this report, we present four FMF-related amyloidosis patients that were treated with long term infliximab therapy with the longest duration of follow-up, together with the literature review. Infliximab was very effective in controlling gastrointestinal system findings and protracted arthritis, and it also had a favorable impact on the clinical findings of nephrotic syndrome in these patients. In conclusion, by controlling debilitating complaints of amyloidosis with infliximab, quality of life increases in these patients, and they get rid of recurrent hospitalizations and return to school or work.
Department of Pediatric Nephrology, Ankara University School of Medicine, Ankara, Turkey.
Z Birsin
ZB
Özçakar
Selçuk
S
Yüksel
Mesiha
M
Ekim
Fatoş
F
Yalçınkaya
D000293:Adolescent; D000686:Amyloidosis / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000008:administration & dosage*; D000911:Antibodies, Monoclonal / Q000008:administration & dosage*; D002648:Child; D003078:Colchicine / Q000008:administration & dosage*; D004359:Drug Therapy, Combination; D010505:Familial Mediterranean Fever / Q000150:complications*; D005260:Female; D005500:Follow-Up Studies; D006074:Gout Suppressants / Q000008:administration & dosage; D006801:Humans; D000069285:Infliximab; D008297:Male; D016896:Treatment Outcome
D000894:Anti-Inflammatory Agents, Non-Steroidal; D000911:Antibodies, Monoclonal; D006074:Gout Suppressants; D000069285:Infliximab; D003078:Colchicine