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Health condition · Clinically reviewed

Familial Mediterranean fever, short attacks, lifelong colchicine and protected kidneys.

The most common inherited periodic fever syndrome - and one of the most treatable, when the diagnosis is made early and colchicine is taken every day.

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Why trust this guide

  • 01

    Clinically reviewed

    Written by our editorial team and reviewed by a registered UK clinician before publication.

  • 02

    Sourced from guidance

    Checked against EULAR, PRES, the UK National Amyloidosis Centre and peer-reviewed sources you can see at the end.

  • 03

    Current for 2026

    Reflects modern UK practice including lifelong colchicine, IL-1 inhibition for colchicine-resistant disease and specialist commissioned amyloid pathways.

Key facts

FMF at a glance.

The essentials, in plain English - what it is, how it presents and how it is treated in the UK today.

  • What it is

    The most common monogenic autoinflammatory syndrome - recurrent, self-limiting attacks of fever and serositis driven by pyrin dysregulation.

  • Genetics

    Autosomal recessive, caused by mutations in the MEFV gene. Most common in Turkish, Armenian, Sephardic Jewish, Arabic, Italian, Greek and North African populations, and now recognised globally.

  • Onset

    Usually childhood - around 90% of people have their first attack before age 20.

  • Classic attack

    A short 1 to 3 day episode of fever above 38 degrees plus peritonitis, pleuritis, monoarthritis or erysipelas-like leg rash, with normal health between attacks.

  • Foundation therapy

    Lifelong colchicine 0.5 to 2 mg daily - prevents attacks and, crucially, prevents AA amyloidosis.

  • Main complication

    Secondary AA amyloidosis, particularly renal disease with proteinuria and progressive CKD. Managed by specialist commissioned amyloid centres.

Why this guide matters

Recognised early, controlled for life.

FMF is often missed for years because attacks look like an acute abdomen, pleurisy or joint infection. A pattern-based approach and lifelong colchicine change the outcome.

  • Pattern beats a single episode

    Short, stereotyped attacks with complete recovery between them is the diagnostic signature - one attack alone is rarely enough.

  • Colchicine is the whole strategy

    Taken lifelong, at the right dose, it prevents attacks and prevents AA amyloidosis - the most important complication.

  • IL-1 inhibition rescues the rest

    For colchicine-resistant FMF, anakinra and canakinumab are highly effective and available through specialist commissioned services.

How the diagnosis is made

From recurrent attacks to a confirmed diagnosis.

The steps a UK GP, rheumatologist or immunologist will normally follow, in order - so you know what to expect and why.

  1. 01

    Assessing

    History and ethnicity

    Recurrent, stereotyped attacks lasting 1 to 3 days, ancestry from a Mediterranean population and a positive family history all raise suspicion.

  2. 02

    Assessing

    Attack diary

    A written log of duration, fever, sites of pain and triggers helps the specialist match features to the Tel Hashomer and Livneh criteria.

  3. 03

    Assessing

    Rule out an acute abdomen

    FMF peritonitis can mimic appendicitis or a surgical abdomen - repeated self-limiting episodes with normal imaging is a key clue and helps avoid unnecessary surgery.

  4. 04

    Confirming

    Inflammatory markers in attack

    CRP, ESR, serum amyloid A (SAA), IL-6 and IL-1 beta rise sharply during an attack and normalise between episodes.

  5. 05

    Confirming

    MEFV genetic testing

    Targeted MEFV sequencing through specialist genetics services confirms the diagnosis. Available at specialist commissioned centres such as Great Ormond Street and Guy’s and St Thomas’.

  6. 06

    Confirming

    Exclude other periodic fevers

    TRAPS, Muckle-Wells, PFAPA, hyper-IgD and cryopyrin-associated periodic syndromes (CAPS) can look similar - a specialist autoinflammatory service will run the differential.

  7. 07

    Protecting

    Amyloidosis screen

    Urinalysis, protein-creatinine ratio and referral to a specialist commissioned amyloid centre (Royal Free National Amyloidosis Centre, Papworth) if proteinuria or unexplained renal decline is found.

Typical pathway: first suspicion to a settled long-term plan in weeks, once specialist review begins.

Symptoms

What an FMF attack actually looks like.

Short episodes of fever plus one or more serosal sites - peritoneum, pleura, joint or skin - resolving completely between attacks.

  • Recurrent fever

    Short, sharp episodes of fever above 38 degrees lasting 1 to 3 days, then full resolution.

  • Peritonitis and abdominal pain

    Severe, generalised abdominal pain that can mimic an acute abdomen. A common reason for unnecessary appendicectomy before diagnosis.

  • Pleuritis and chest pain

    Sharp, one-sided pleuritic pain, worse on breathing, that settles within a few days.

  • Monoarthritis of a large joint

    A hot, swollen knee, ankle or hip during an attack, usually one joint at a time.

  • Erysipelas-like erythema

    A well-demarcated red patch on the shin, ankle or dorsum of the foot - classic for FMF.

  • Attacks last 1 to 3 days

    Stereotyped, short and self-limiting - with completely normal health between attacks.

  • Childhood onset

    Most people have their first attack before age 20 - adult-onset FMF is recognised but less common.

  • Red flag - proteinuria

    New proteinuria, nephrotic syndrome or progressive CKD suggests AA amyloidosis and needs urgent specialist review.

Treatment

How FMF is treated in the UK.

Lifelong colchicine at the right dose is the foundation. IL-1 inhibitors rescue colchicine-resistant disease, and amyloidosis is managed by specialist commissioned centres.

  • Colchicine

    The mainstay of care - 0.5 to 2 mg daily, lifelong. Prevents attacks in around two-thirds of people and prevents AA amyloidosis. See our colchicine clinic.

  • Anakinra (IL-1 inhibitor)

    Daily subcutaneous IL-1 receptor antagonist for colchicine-resistant or colchicine-intolerant FMF. Specialist commissioned.

  • Canakinumab (IL-1 inhibitor)

    Monthly subcutaneous anti-IL-1 beta antibody, licensed for colchicine-resistant FMF. Highly effective and specialist commissioned.

  • Rilonacept

    IL-1 trap used in selected colchicine-resistant patients, in specialist commissioned autoinflammatory clinics.

  • TNF and IL-6 inhibitors

    Tocilizumab and TNF inhibitors are used selectively where IL-1 blockade is not enough, particularly with amyloidosis.

  • Amyloidosis management

    Colchicine is the primary prevention. Established AA amyloidosis is managed by specialist commissioned amyloid centres such as the Royal Free.

  • Supportive care

    Hydration, simple analgesia and rest during attacks. Long-term NSAIDs are avoided where possible to protect the kidneys.

  • MDT and support

    Rheumatology, immunology, nephrology, genetics and patient organisations such as FMF UK work together across the pathway.

Explore related services: colchicine clinic, anakinra and canakinumab clinic, rituximab infusion clinic.

What this guide is based on

The sources behind every claim on this page.

European and UK specialist society guidance and specialist commissioned service standards, current at the time of last review.

Key references

Guidelines and standards we relied on.

A quiet reminder

This guide is for information, not medical advice.

Your GP, rheumatologist or immunologist knows your history and can tell you which parts apply to you. If in doubt, get seen.

  • EULAR. Recommendations for the management of familial Mediterranean fever.

  • PRES / SHARE. Evidence-based recommendations for the diagnosis and management of autoinflammatory diseases in children.

  • Royal Free London / UK National Amyloidosis Centre. Guidance on AA amyloidosis diagnosis and treatment.

  • NHS England. Specialised commissioning for autoinflammatory diseases and amyloidosis.

  • Livneh A et al. Criteria for the diagnosis of familial Mediterranean fever.

Red flags

When FMF needs urgent attention.

Most people with FMF are stable on colchicine. These are the situations that need a fast specialist opinion.

  • New or worsening proteinuria

    A late but critical sign of AA amyloidosis - warrants urgent specialist assessment at an amyloid centre.

  • Nephrotic syndrome

    Heavy proteinuria, oedema and low albumin in someone with FMF is amyloidosis until proven otherwise.

  • Progressive CKD

    Unexplained decline in kidney function needs prompt nephrology review and screening for amyloid.

  • Prolonged or atypical attack

    An episode lasting longer than 3 to 4 days, or with new features, should prompt review to exclude other causes.

  • Recurrent surgical abdomen

    Multiple normal laparotomies for undiagnosed abdominal pain in a person from a Mediterranean background - think FMF.

  • Poor response to colchicine

    Ongoing attacks despite maximal tolerated colchicine defines colchicine-resistant FMF - IL-1 inhibition is indicated.

  • Colchicine toxicity

    Severe diarrhoea, cytopenia or myopathy - especially with renal impairment or interacting drugs (macrolides, statins) - is a medical emergency.

  • Pregnancy planning

    Colchicine is continued in pregnancy to prevent flares and amyloidosis - specialist input is important.

  • Suspected other periodic fever

    If the pattern does not fit FMF, consider TRAPS, CAPS, hyper-IgD or PFAPA and refer to a specialist autoinflammatory service.

Consider other periodic fevers: TRAPS and Muckle-Wells syndrome.

Living with it

A lifelong condition, with a clear plan.

Four things that make the biggest difference year to year - daily colchicine, an attack diary, kidney monitoring and asking about IL-1 inhibitors when attacks persist.

A quiet reminder

Consistency beats intensity, every time.

Small, steady habits - kept up for decades - do more than a heroic month that does not last.

  1. 01 Daily

    Take colchicine every day

    Even between attacks and even when you feel completely well. It is prevention as much as treatment - and it protects your kidneys.

  2. 02 Track

    Keep an attack diary

    Note date, duration, fever, site of pain and triggers. It helps your team judge control and spot patterns early.

  3. 03 Monitor

    Check your kidneys regularly

    Annual urinalysis, protein-creatinine ratio and kidney function - the earliest way to catch amyloidosis before symptoms.

  4. 04 Escalate

    Ask about IL-1 inhibitors if attacks continue

    If colchicine at the highest tolerated dose is not enough, anakinra or canakinumab through a specialist commissioned autoinflammatory service can transform control.

Frequently asked

Everything we get asked about FMF.

Quick answers on genetics, colchicine, IL-1 inhibitors and amyloidosis.

  • What is familial Mediterranean fever?

    FMF is an inherited autoinflammatory condition caused by mutations in the MEFV gene. It causes short, recurrent attacks of fever with abdominal pain, chest pain, joint pain or a red rash on the lower legs. Between attacks, people are usually completely well. It is the most common monogenic autoinflammatory syndrome and is treatable.

  • How is FMF inherited?

    FMF is autosomal recessive. That means two copies of a MEFV mutation are usually needed to cause typical disease - one inherited from each parent. Genetic testing is arranged through specialist genetics services and helps confirm the diagnosis and inform family members.

  • Who gets FMF?

    FMF is most common in people of Turkish, Armenian, Sephardic Jewish, Arabic, Italian, Greek and North African ancestry, but it is increasingly recognised in other populations worldwide. Ethnicity supports the diagnosis but does not make or exclude it on its own.

  • Why is colchicine so important?

    Colchicine prevents attacks in the majority of people with FMF and, more importantly, prevents AA amyloidosis - the most serious long-term complication. It is taken lifelong, usually 0.5 to 2 mg daily, and continues through pregnancy under specialist guidance.

  • What happens if colchicine is not enough?

    Colchicine-resistant FMF is defined as ongoing attacks despite the maximum tolerated dose. IL-1 inhibitors such as anakinra (daily) or canakinumab (monthly) are highly effective and are prescribed through specialist commissioned autoinflammatory services in the UK.

  • What is AA amyloidosis and why does it matter?

    AA amyloidosis is a build-up of serum amyloid A protein in organs, most often the kidneys. It can cause proteinuria, nephrotic syndrome and progressive kidney failure. Regular urine and blood monitoring, lifelong colchicine and, where needed, IL-1 blockade or referral to the UK National Amyloidosis Centre at the Royal Free are how we prevent and manage it.

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