This protocol covers the management of AA amyloidosis occurring in the setting of an autoinflammatory syndrome — specifically familial Mediterranean fever (FMF) — in patients whose initial treatment has not met its therapeutic goals.
The patient has AA amyloidosis associated with familial Mediterranean fever. The clinical priority is to suppress systemic inflammation so that serum SAA levels normalise and amyloidosis remission can be achieved.
Colchicine was used as the first-line approach. It did not achieve control of systemic inflammation — serum SAA levels remained significantly elevated — and remission of the associated AA amyloidosis was not reached. This unmet response is the criterion that escalates care to the current protocol.
DOI: 10.2147/CLEP.S39981
High-dose colchicine (1.5–2 mg/day) is effective in controlling systemic inflammation in autoinflammatory syndromes, such as FMF, and has been able to induce remission of associated AA amyloidosis.
Moreover, anti-IL-1 antibodies, such as anakinra or canakinumab, which have already proven effective as first-line therapies in several autoinflammatory syndromes, may also prove effective in patients with refractory FMF, in whom clinical remission with high-dose colchicine has not been achieved, patients in whom significantly elevated serum SAA levels are still detectable despite treatment with colchicine, patients with colchicine intolerance, and patients in whom the clinical picture is suggestive of an overlap between FMF and vasculitis (predominantly with concomitant polyarteritis nodosa, or Henoch–Schönlein purpura).
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