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Familial Mediterranean fever

Familial Mediterranean fever is an inherited condition characterized by recurrent episodes of painful inflammation in the abdomen, chest, or joints

Prevalence

1-5 / 10 000

33,100-165,500

US Estimated

51,350-256,750

Europe Estimated

Age of Onset

ICD-10

E85.0

Inheritance

Autosomal dominant

Autosomal recessive

Mitochondrial/Multigenic

X-linked dominant

X-linked recessive

5 Facts you should know

FACT

1

An inherited condition characterized by recurrent episodes of painful inflammation in the abdomen, chest, or joints

FACT

2

While all ethnic groups are susceptible to FMF, it usually occurs in people of Mediterranean origin

FACT

3

Ninety percent of all patients have their first attack before they are 18 years old

FACT

4

Attacks develop over 2–4 hours and last anywhere from 6 hours to 4 days

FACT

5

Amyloid A protein is produced in very large quantities during attacks, and at a low rate between attacks, and accumulates mainly in the kidney

Familial Mediterranean fever is also known as...

Familial Mediterranean fever is also known as:

  • Periodic peritonitis
  • Recurrent polyserositis
  • Benign paroxysmal peritonitis

What’s your Rare IQ?

Without treatment, which of the following is known to occur to patients with Familial Mediterranean fever?

Common signs & symptoms

Abdominal pain

Arthralgia

Constipation

Fever

Myalgia

Nausea and vomiting

Current treatments

The goal of treatment for familial Mediterranean fever (FMF) is to control symptoms because there is no cure for the condition.

Treatment of an acute episode may include:

Intravenous saline

for hydration

Nonsteroidal anti-inflammatory drugs (NSAIDs)

for fever and inflammatory episodes

NSAIDs, paracetamol or dipyrone

for pain relief

Routine treatment of end-stage renal disease

(kidney failure), including renal transplantation