Familial Mediterranean fever: an animated, cited guide

This page needs JavaScript for its animations. The outline and key answers are below, and every claim in the full version carries a numbered citation.

Not medical advice. FMF Companion is an educational synthesis of published literature. It does not replace a clinician.

What is in it

What FMF is Who gets it, the numbers, and what an attack looks like.
The gene and inheritance MEFV, pyrin, the common variants and an animated Punnett square.
Pyrin, animated How pyrin guards RhoA, and what FMF variants change.
An attack, hour by hour 72 hours of fever, pain, CRP and SAA, site by site.
Colchicine inside the cell From the pill to the neutrophil to the microtubule.
Colchicine over months Why the benefit builds slowly, and what it prevents.
Dosing, safety and interactions What the guidelines and the label publish.
AA amyloidosis Serum amyloid A to fibril to kidney, over years.
Diagnosis and criteria Tel Hashomer, Livneh and the Eurofever/PRINTO calculator.
The differential TRAPS, MKD, CAPS, PFAPA, Behçet and the surgical abdomen.
The treatment ladder Colchicine first, then IL-1 blockade.
Living with FMF Pregnancy, surgery, school, triggers and monitoring.
Toward a cure What a cure would need, and the evidence for each route.
History and people 1908 to the discovery that pyrin senses RhoA.
Flashcards, quiz and glossary Revision for students.
Sources Every reference behind the app.

Part 4 · Treatment & the future

Living with FMF

FMF is managed across years, not visits. Press play to walk a single year around the ring: routine checks arrive on schedule, and the toggles drop real-life situations into the same year, each with what the published literature says about it. Everything here is what studies and guidelines report, not advice.

Add to this year

Through the year

Schematic year. Intervals follow published guidance, not any one clinic.

What the trigger evidence actually shows

Grow bars

Questionnaire percentages are what patients report, which is not the same as what causes attacks. The case-crossover design compares each patient with themselves, so it is the stronger evidence. ^118,209,279

Diet is the weakest link in this literature: a review found only 7 eligible studies, with conflicting results on fatty and salty food and a possible role for wheat, and no robust trial showing that any diet prevents attacks. ¹⁶⁷

School, work and mood

Shade the rows

FMFcontrols

Group findings from separate studies, each with its own population. ^151,236,251

Prognosis, monitoring and people

The monitoring checklist in the 2016 guideline

Response, toxicity and adherence reviewed every 6 months, more often in the first year or when acute-phase markers are raised, and yearly once stable.
Tests listed: SAA and/or CRP, blood count, liver enzymes, kidney function, CPK, and urinalysis for proteinuria.
Urinalysis at every clinic visit, to catch proteinuria early.
Colchicine is reduced if liver enzymes go above twice the upper limit of normal.
High SAA values are shared with the patient, deliberately, to support adherence.
The 2024 update sets no fixed interval, and asks for monitoring more often at diagnosis and when disease is uncontrolled, against a minimum outcome set. ^199,203,222

Questions people bring to a clinic visit

Plain-words versions of what the guidelines themselves say should be discussed. They are prompts for a conversation with a clinician, not a script and not advice.

What is my SAA or CRP between attacks, and is it in the normal range?
Was there any protein in my urine today?
Am I on the dose the guidance would call maximum tolerated for me, and is there room to go up?
How many attacks have I had in the last 3 months, counted rather than remembered?
If attacks continue at this dose, what would the next step be?
What should happen to my treatment if I plan a pregnancy, or need an operation, or need vaccinations?
How often should I be seen, and which tests each time?

In plain wordsTwo numbers do most of the work at a visit: how many attacks since last time, and what the inflammation markers say between them.