A comparison of clinical findings of familial Mediterranean fever patients with and without amyloidosis

Abstract

Objective: This study investigates the clinical and demographic characteristics of familial Mediterranean fever (FMF) patients with and without amyloidosis.

Patients and methods: The clinical data of 503 patients with FMF (females:males 250:253) were reviewed. Fifty of these patients had amyloidosis (f:m 23:27).

Results: The ages of attack onset in patients with and without amyloidosis were 7.8+/-6.2 and 11.1+/-8.5, respectively (P<0.05). The time between disease onset and diagnosis was longer in patients with amyloidosis than those without (187.6+/-99.4 months and 132.5+/-110.2 months, respectively, P<0.001). More patients in the amyloidosis group had positive family histories of FMF (68% vs 54%, P<0.05). The frequencies of chest pain (78% vs 51%, P<0.001), arthritis ( 80% vs 60%, P<0.01), and erysipelas-like erythema (44% vs 16%, P<0.001) were higher in the amyloidosis group.

Conclusion: In the amyloidosis group, FMF-related manifestations of chest pain, arthritis, and erysipelas-like erythema are more frequent. Our results also support that long periods between disease onset and diagnosis are associated with a high risk of developing amyloidosis.