Approach to Recurrent Abdominal Pain with Fever
Familial Mediterranean Fever • Recurrent Polyserositis • Autoinflammatory Disease • MRCP (UK) PACES
A structured MRCP (UK) PACES approach to patients presenting with recurrent abdominal pain and fever. Learn how to recognise Familial Mediterranean Fever (FMF), distinguish it from other causes of recurrent abdominal pain and investigate patients using a logical clinical framework.
At a Glance
Red Flags
PACES Approach
Recurrent Abdominal Pain Framework
Think inflammatory cause
FMF
Polyserositis
FMF flare
Common clue
AA Amyloidosis
Familial Mediterranean Fever Clues
Overview
Recurrent abdominal pain with fever should prompt consideration of inflammatory, infectious and surgical causes. In young patients with self-limiting attacks of peritonitis, pleuritis or arthritis—particularly those of Mediterranean ancestry or with a positive family history—Familial Mediterranean Fever (FMF) should be suspected. FMF is an inherited autoinflammatory disorder caused by mutations in the MEFV gene, leading to recurrent episodes of serosal inflammation. Early recognition and lifelong colchicine therapy are essential to reduce attacks and prevent AA amyloidosis, the major long-term complication. :contentReference[oaicite:1]{index=1}
Structured History
Characterise the Abdominal Pain
- Age at onset and frequency of attacks
- Sudden or gradual onset
- Site and radiation of pain
- Severity and duration of episodes
- Complete resolution between attacks
- Previous admissions with similar symptoms
Associated Symptoms
- High fever during attacks
- Nausea or vomiting
- Constipation during attacks followed by diarrhoea
- Loss of appetite
- Pleuritic chest pain
- Breathlessness
Features Suggesting Polyserositis
- Recurrent pleuritic chest pain
- Acute monoarthritis affecting knees or ankles
- Painful swollen joints
- Scrotal or testicular pain
- Erysipelas-like rash over the lower limbs
- Episodes resolving spontaneously within a few days
Important Background History
- Mediterranean ancestry
- Family history of similar illness
- Previous appendicectomy or laparotomy
- Previous diagnosis of recurrent peritonitis
- History of infertility or recurrent miscarriage
- Previous episodes of proteinuria
Screen for Complications
- Leg swelling
- Foamy urine
- Reduced urine output
- Weight loss
- Progressive fatigue
- Symptoms of chronic kidney disease
Examination
- Record temperature and vital signs
- Assess hydration status
- Inspect the abdomen for previous surgical scars
- Look for abdominal guarding or signs of peritonism
- Auscultate for bowel sounds
- Listen for a pleural rub
- Examine knees, ankles and wrists for acute synovitis
- Inspect both lower legs for an erysipelas-like erythematous rash
- Assess for peripheral oedema suggesting nephrotic syndrome
- Perform urine dipstick for proteinuria if available
Differential Diagnosis
Investigations
Baseline Blood Tests
- Full blood count
- CRP and ESR
- Serum Amyloid A (where available)
- Renal profile
- Liver function tests
- Fibrinogen
Urine Assessment
- Urinalysis for proteinuria
- Urine protein-creatinine ratio
- Microscopy for haematuria
Confirmatory Investigations
- MEFV gene mutation analysis
- Chest imaging if pleural involvement is suspected
- Ultrasound abdomen during acute attacks where appropriate
- Joint ultrasound if significant synovitis is present
Assessment for Complications
- Renal biopsy when amyloidosis is suspected
- Echocardiography if pericarditis is suspected
- Pulmonary imaging for pleural effusions
Diagnosis
Recurrent Abdominal Pain with Fever due to Familial Mediterranean Fever
Familial Mediterranean Fever is an autosomal recessive autoinflammatory disorder caused by pathogenic variants in the MEFV gene. It is characterised by recurrent self-limiting episodes of fever, peritonitis, pleuritis and arthritis. Without treatment, persistent inflammation may lead to AA amyloidosis and progressive renal disease. :contentReference[oaicite:1]{index=1}
Management
Explaining to the Patient
"Your symptoms suggest an inherited inflammatory condition called Familial Mediterranean Fever. It causes repeated episodes of inflammation affecting the lining of the abdomen, chest and joints, leading to attacks of pain and fever. Although the attacks usually settle on their own, long-term treatment is important because it helps prevent future attacks and protects the kidneys from a serious complication called amyloidosis."
Examiner's Corner
What is Familial Mediterranean Fever?
- Familial Mediterranean Fever (FMF) is an inherited autoinflammatory disorder caused by pathogenic variants in the MEFV gene.
- It results in recurrent episodes of sterile inflammation involving serosal surfaces, producing fever, abdominal pain, pleuritis and arthritis.
- Attacks usually last between 12 and 72 hours and patients are typically well between episodes.
Why is colchicine so important?
- Colchicine reduces the frequency and severity of inflammatory attacks.
- It is the most effective treatment for preventing AA amyloidosis.
- Treatment is lifelong, even when attacks become infrequent.
- Patients who remain symptomatic despite adherence should be assessed for colchicine resistance and referred for specialist review.
What is AA Amyloidosis?
- The most serious long-term complication of FMF.
- Persistent inflammation leads to deposition of serum amyloid A protein in tissues.
- The kidneys are most commonly affected, causing proteinuria, nephrotic syndrome and progressive chronic kidney disease.
- Regular urine testing for proteinuria is essential during follow-up.
How do you differentiate Familial Mediterranean Fever from other periodic fever syndromes?
| Condition | Typical Features |
|---|---|
| Familial Mediterranean Fever | Short attacks of fever with peritonitis, pleuritis or arthritis; excellent response to colchicine |
| TRAPS | Longer attacks, migratory rash and myalgia |
| Hyper-IgD Syndrome | Childhood onset with cervical lymphadenopathy and diarrhoea |
| PFAPA Syndrome | Periodic fever with aphthous ulcers, pharyngitis and cervical adenitis |
Common Viva Questions
- Which populations are most commonly affected by FMF?
- What is the function of the MEFV gene?
- What is polyserositis?
- Why do many patients undergo unnecessary appendicectomy?
- How is FMF confirmed?
- What is the most important long-term complication?
- When should biologic therapy be considered?
Important Clinical Pearls
- Think FMF whenever a young patient has recurrent self-limiting abdominal pain with fever.
- Always ask about Mediterranean ancestry and family history.
- Patients are usually completely well between attacks.
- Proteinuria may be the first clue to developing AA amyloidosis.
- Early lifelong colchicine dramatically improves long-term outcomes.
Pathfinder Pearls
Common Pitfalls
- Repeatedly treating attacks as acute appendicitis without considering FMF.
- Failing to ask about ethnicity or family history.
- Missing pleuritis or arthritis because attention is focused solely on abdominal pain.
- Not screening for proteinuria during follow-up.
- Stopping colchicine once symptoms improve.
- Overlooking AA amyloidosis until advanced renal disease develops.
- Neglecting genetic counselling for affected families.
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