A comparison of clinical findings of familial Mediterranean fever patients with and without amyloidosis.
OBJECTIVE
This study investigates the clinical and demographic characteristics of familial Mediterranean fever (FMF) patients with and without amyloidosis.


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.


CONCLUSIONS
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.
Department of Rheumatology, Division of Internal Medicine, Istanbul University Faculty of Medicine, Istanbul, Turkey. kcefle@hotmail.com
Ayse
A
Cefle
Sevil
S
Kamali
Mehmet
M
Sayarlioglu
Murat
M
Inanc
Lale
L
Ocal
Orhan
O
Aral
Meral
M
Konice
Ahmet
A
Gul
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000686:Amyloidosis / Q000453:epidemiology / Q000209:etiology* / Q000473:pathology; D001168:Arthritis / Q000150:complications / Q000453:epidemiology / Q000473:pathology; D002637:Chest Pain / Q000150:complications / Q000209:etiology / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D004886:Erysipelas / Q000150:complications / Q000453:epidemiology / Q000473:pathology; D004890:Erythema / Q000150:complications / Q000453:epidemiology / Q000473:pathology; D010505:Familial Mediterranean Fever / Q000150:complications* / Q000453:epidemiology / Q000473:pathology; D005260:Female; D006801:Humans; D007223:Infant; D007676:Kidney Failure, Chronic / Q000453:epidemiology / Q000209:etiology / Q000473:pathology; D008297:Male; D011507:Proteinuria / Q000175:diagnosis / Q000209:etiology; D013997:Time Factors; D014421:Turkey / Q000453:epidemiology