Colchicine in the treatment of renal amyloidosis secondary to familial Mediterranean fever

Ashot Sarkissian1, Marina Papazian1, Armen Sanamyan2 and Ernst Leumann3

1 Division of Nephrology, Medical Centre ‘Arabkir’, Yerevan, Armenia 2 Republican Centre of Paediatric Pathology, Yerevan, Armenia 3 Division of Nephrology, University Children's Hospital, Zurich, Switzerland

Sir,

We have read with interest the Letter to the Editor by Simsek and colleagues [1] reporting the efficacy of colchicine in a nephrotic paediatric patient with amyloidosis due to familial Mediterranean fever (FMF) with resolution of proteinuria. Several studies have clearly shown the effect of colchicine in the prevention of amyloidosis, but once the kidney is involved it is disputed whether this treatment could still alter the progression of renal disease. There are anecdotal reports on the successful treatment at the late stage of renal amyloidosis, however these are mainly on adults [2,3] and very few on paediatric patients [4,5]. Reversal of the nephrotic syndrome was noticed by Hojberg and Mertz [4] in two brothers (aged 6 and 10 years) with FMF and amyloidosis proven by rectal biopsy: one of them had complete remission. Majeed et al. [5] observed the disappearance of proteinuria in two children with FMF. However, these two patients were not nephrotic, and no biopsy had been performed, so the authors could only assume, but not prove, that they indeed had renal amyloidosis.

We have observed three paediatric patients who seem to support the observation of Simsek et al. [1]. All three—two girls and one boy, aged 11–15 years—presented only at the nephrotic stage of FMF with serum albumin 8–20 g/l and with proteinuria of 3–9 g/24 h; serum creatinine was still in the normal range (51–63 µmol/l). Amyloidosis was confirmed by renal biopsy in each of them. Therapy with colchicine (1.8 mg/day) was initiated and was continued during the follow-up period of 55–69 months. These three patients did not only maintain good renal function, but in addition had full reversal of the nephrotic syndrome within 3–16 months with disappearance of proteinuria (trace to 0.066 g/l) at 26–40 months. They remained proteinuria free during the further follow-up of 15–30 months.

Thus, there still is a chance of reversal of the nephrotic syndrome when therapy with colchicine is initiated only at the nephrotic stage of renal amyloidosis. However, this response is not predictable. Indeed, we have only seen a moderate (or no) response to colchicine administration in several other cases who started treatment similarly late. Obviously, treatment with colchicine should be started as early as possible in the course of FMF.

References

  1. Simsek B, Islek I, Simsek T, Küçüködük S, Cengiz K. Regression of nephrotic syndrome due to amyloidosis secondary to familial Mediterranean fever following colchicine treatment. Nephrol Dial Transplant2000; 15: 281–282[Free Full Text]
  2. Zemer D, Livneh A, Langevitz P. Reversal of the nephrotic syndrome by colchicine in amyloidosis of familial Mediterranean fever. Ann Intern Med1992; 116: 426
  3. Rosenbaum M, Rosner I. Regression of amyloidosis with colchicine in familial Mediterranean fever in an Ashkenazi patient. Clin Exp Rheumatol1995; 13: 126[ISI][Medline]
  4. Hojberg AS, Mertz H. Nefrotisk syndrom ved familiaer middelhavsfeber—effekt of colchicinbehandling. Ugeskr Laeger1995; 157: 4035–4037[Medline]
  5. Majeed HA, Ravashdeh M, El-Shanti H, Qubain H, Khuri-Bulos N, Shahin HM. Familial Mediterranean fever in children: the expanded clinical profile. Q J Med1999; 92: 309–318[Abstract/Free Full Text]
  6. Sarkissian A, Papazian M, Sanamiam A, Babloyan A. Does colchicine alter the course of amyloidosis in children with familial Mediterranean fever? Pediatr Nephrol1998; 12: 76




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