PERIODIC FEVER

Definition

Periodic fever is a condition characterized by recurrent episodes of fever occurring at regular or irregular intervals, without an apparent infectious cause. It may be idiopathic, genetic, or associated with autoinflammatory syndromes.

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✅ Epidemiology

• Mostly affects children, but may also occur in adults.

• It is rare and often underdiagnosed.

• Genetic forms (e.g., FMF, PFAPA) are more common in certain populations (e.g., Mediterranean, Middle Eastern regions).

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✅ Pathophysiology (Explanation of the Disease)

• Malfunction of the innate immune system.

• The body triggers inflammatory responses even in absence of infection.

• Pro-inflammatory cytokines, such as interleukin-1 (IL-1), are often involved.

• In some syndromes, genetic mutations disrupt proper inflammatory regulation.

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✅ Causes and Risk Factors

• Genetic causes (hereditary autoinflammatory syndromes):

• PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis)

• FMF (Familial Mediterranean Fever)

• TRAPS (TNF Receptor-Associated Periodic Syndrome)

• HIDS (Hyper IgD Syndrome or Mevalonate Kinase Deficiency)

• Environmental factors are usually not implicated.

• Family history can be a significant risk indicator.

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✅ Symptoms

• Recurrent episodes of high fever (≥38.5°C / 101.3°F)

• Each episode typically lasts 3–7 days, resolving spontaneously

• Asymptomatic intervals between episodes

• Associated symptoms vary depending on the syndrome:

• Cervical lymphadenopathy

• Pharyngitis (especially in PFAPA)

• Mouth ulcers

• Abdominal or joint pain

• Skin rashes (in some genetic forms)

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✅ Diagnosis

• Clinical diagnosis: based on medical history and pattern of fever episodes.

• Exclusion of other causes (infections, autoimmune diseases, malignancies).

• Blood tests during episodes: elevated CRP, ESR, leukocytosis.

• Genetic testing: to confirm autoinflammatory syndromes (e.g., FMF, TRAPS).

• Keeping a fever diary is helpful to track the periodicity.

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✅ Therapy

Treatment depends on the specific form:

🔹 PFAPA:

• Single dose of corticosteroids (e.g., prednisone) during episodes → rapid resolution

• Tonsillectomy may be considered in some cases (controversial)

🔹 FMF, TRAPS, HIDS:

• Colchicine (FMF) → prevents flare-ups

• Biological drugs (e.g., IL-1 inhibitors like anakinra or canakinumab) → in severe or resistant cases

• NSAIDs for symptomatic relief

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✅ Nursing Care

• Monitor body temperature during and between episodes

• Observe for associated symptoms (rash, pain, localized inflammation)

• Patient and family education:

• Recognize early signs of a flare-up

• Correct use of medications (especially corticosteroids and biologics)

• Psychological support: anxiety due to chronic nature of the disease

• Ensure hydration during febrile episodes

• Monitor for side effects of therapies (e.g., colchicine: gastrointestinal issues)

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✅ Advice and Prevention

• No true prevention strategies exist, especially for genetic forms.

• Keeping a fever diary helps with monitoring and diagnosis.

• Regular follow-ups with a pediatrician or rheumatologist are essential.

• Avoid self-medicating with antibiotics during fever (usually ineffective).

• In case of genetic diagnosis, genetic counseling is advised for the family.