Caused by mutations in the
• Provides instructions for making pyrin
(marenostrin), which is found in white blood cells
• Regulates process of inflammation
• Primarily affects populations originating in the
• Armenian, Arab, Turkish, Jewish peoples
• Affects from 1/250 to 1/1000 people in these
• Inherited in autosomal recessive pattern
• Recessive gene
• Recurrent episodes of painful inflammation of
the abdomen, chest or joints
• First episode usually occurs in childhood or
• Episodes last 12-72 hours on average
• Episodes can vary in severity
• Length of time between attacks varies
• A buildup of protein deposits may occur on
organs and body tissues
• Without preventative treatments, kidney
failure can occur
• Recurrent short episodes
• Inflammation and serostis
• Pericarditis and Meningitis (less common)
• Amyloidosis as the first clinical manifestation
of FMF in an otherwise asymptomatic
• Recurrent febrile episodes accompanied by
peritonitis, synovitis, or pleuritis
• Recurrent erysipelas-like erythema
• Repeated laparotomies for "acute abdomen" with no
• Amyloidosis of the AA type that characteristically
develops after age 15 years in untreated
individuals, even in those who do not have a history of
recurrent inflammatory attacks
• Favorable response to continuous colchicine treatment
• FMF in a first-degree relative
• At-risk ethnic group
• Test looks for gene mutations
• Minimal Criteria
– FEVER plus one or more major signs and one
– FEVER plus two minor signs
• No known cure
• Colchicine precents attacks
– Administer colchicine therapy daily in patients at risk
of developing amyloidosis
– The regimen for acute attacks in patients not taking
daily colchicine is 0.6 mg every hour for 4 doses, then
0.6 mg every 2 hours for 2 doses and then 0.6 mg
every 12 hours for 4 doses.
• Nonsteroidal anti-inflammatory drugs
• Possible renal transplantation
• Treatment can prevent long-term damage to
• Some children initially need psychological
support to cope.
• Seek support from community members
(family, friends, teachers, employers)
because episodes may result in an inability
to carry out daily responsibilities
• Use treatment (colchicine) to avoid attacks
and help you live a normal life
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• “Disease Characteristics." National Center for Biotechnology Information. U.S.
National Library of Medicine, n.d. Web. 14 Mar. 2014.
• "Familial Mediterranean Fever." - Genetics Home Reference. Genetics Home
Reference, 10 Mar. 2014. Web. 12 Mar. 2014.
• Hashkes, Philip. "Familial Mediterranean Fever (Juvenile) | American College of
Rheumatology | ACR." Familial Mediterranean Fever (Juvenile) | American
College of Rheumatology | ACR. American College of Rheumatology, Mar.
2013. Web. 14 Mar. 2014.
• Meyerhoff, John O. "Familial Mediterranean Fever Treatment &
Management." Familial Mediterranean Fever Treatment & Management.
Medscape, 13 Feb. 2014. Web. 14 Mar. 2014
• Shohat, Mordechai. "Familial Mediterranean Fever." Familial Mediterranean Fever.
U.S. National Library of Medicine, 26 Apr. 2012. Web. 14 Mar. 2014.