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AA type renal amyloidosis secondary to FMF: a long-term follow-up in two patients
Aysun Aybal Kutlugün1, Tolga Yildirim, Mahmut Altindal
1Department of Nephrology, Faculty of Medicine, Hacettepe University, Ankara, Turkey. draysunaybal@yahoo.com
Abstract:
Renal amyloidosis, which leads to renal failure, is the most important long-term complication of familial Mediterranean fever (FMF). Resolution of nephrotic syndrome secondary to amyloidosis in FMF following colchicine treatment has rarely been reported. We describe two patients with FMF and nephrotic syndrome. These patients were treated with colchicine 1.5 mg/day and had a complete remission of nephrotic syndrome with a stable clinical course over 30 years. To our knowledge, our patients have the longest follow-up time without proteinuria.
Insights
Familial Mediterranean fever (FMF) patients with amyloidosis-induced nephrotic syndrome achieved complete remission with long-term colchicine treatment. This case series reports the longest follow-up without proteinuria in FMF renal amyloidosis.
Area of Science:
- Nephrology
- Rheumatology
- Genetics
Background:
- Renal amyloidosis is a severe complication of Familial Mediterranean Fever (FMF), often leading to end-stage renal disease.
- Nephrotic syndrome in FMF patients typically indicates advanced amyloidosis and carries a poor prognosis.
Observation:
- Two patients with FMF-induced renal amyloidosis and nephrotic syndrome were treated with colchicine.
- The treatment regimen involved a daily dose of 1.5 mg of colchicine.
Findings:
- Both patients achieved complete remission of nephrotic syndrome, evidenced by the resolution of proteinuria.
- The patients maintained a stable clinical course with no proteinuria for over 30 years, representing the longest reported follow-up.
Implications:
- Long-term colchicine therapy can induce sustained remission in FMF-associated nephrotic syndrome and renal amyloidosis.
- These findings highlight the efficacy of colchicine in managing severe renal complications of FMF, potentially altering the disease's long-term trajectory.
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