Decrease in the rate of secondary amyloidosis in Turkish children with FMF: are we doing better?

Abstract

Familial Mediterranean fever (FMF) is the most common autoinflammatory disease in the world. The most serious complication of FMF is the development of secondary amyloidosis. Besides genetic factors, environment has been implicated in the development of this complication. The main objective of this study is to analyze whether there has been a substantial decrease of secondary amyloidosis in Turkey and possible effective factors. For this purpose, clinical features of the patients diagnosed with secondary amyloidosis between the years 1978 and 1990 were compared with those diagnosed between 2000 and 2009. Severity scores were determined by the use of a scoring system modified for children. Median ages of the group diagnosed between 1978 and 1990 (n = 115; 12.1% among a total of 947 renal biopsies) and diagnosed after 2000 (n = 19; 2% among a total of 974 renal biopsies) were 12 and 13 years, respectively. There were no significant differences between the two patient groups according to gender, age, age of onset, disease duration, and disease severity. There was, however, a clear decrease in the percentage of biopsies with secondary amyloidosis from 12.1% (1978-1990) to 2% (after 2000; p < 0.001). Our results have shown that there has been a significant decrease in the rate of secondary amyloidosis in Turkey. The main reason for this decrease is better medical care with increased awareness and treatment of the disease. However, we suggest that the improvement of infectious milieu may possibly have had a positive effect on the course of this monogenic disease, since inflammatory pathways related to innate immunity are deregulated.

Changing epidemiology of AA amyloidosis: clinical observations over 25 years at a single national referral centre

“…Akse-Onal and colleagues (6) A Swedish study identifying patients from hospital discharge and outpatient registers between 2001 and 2008 estimated an incidence of AA amyloidosis of 0.2 per 100 000, based on RA death rates (7). In 2013 an epidemiological study of systemic amyloidosis in England was conducted using combined data from the United Kingdom (UK) National Amyloidosis Centre (NAC) patient database and English death certificate data from the Office of National Statistics (8).…”

An Overview of the Amyloidosis in Children with Rheumatic Disease

“…FMF is the overall most common autoinflammatory disease and has prevalences as high as 1/ 1,000-1/250 among Jews, Turks, Armenians, and Arabs (37). The most seri o u s complication of the disease is the development of AA type amyloidosis, first diagnosed by Mamou and Cattan in 1952 (38).…”

The Rate of Awareness for Familial Mediterranean Fever for Urban Dwellers in Turkey

“…[4] However, to our knowledge, there is no study that has evaluated the awareness of Turkish people in general regarding FMF. We presumed that the information status of a given population concerning a frequent disease like FMF would affect the early diagnosis and adequate treatment.…”

“…This can be attributed to the increased awareness of physicians with regard to FMF. [4] Familial Mediterranean fever was highlighted as a health problem due in large part to a study published in 1993 that reported on this disorder in Turkey. [5] In 1998, the high prevalence rate of FMF at 1/1.073 in a field study placed it as one of the most frequent genetic diseases in Turkey.…”

“…[3] The main factor for the dramatic decrease in the cases of renal amyloidosis in Turkey is thought to be due to better medical education and practice among Turkish physicians which has led to the increased use of colchicine. [4] Unfortunately, only the education of physicians is not enough to prevent complications due to FMF. The general population should also be educated for the signs and symptoms of this frequent genetic disease.…”