Understanding Recurring Fevers: An Overview
When fevers return repeatedly over time, it often suggests an underlying health issue different from a typical infection. A fever that keeps recurring, especially in a cyclical pattern, is medically termed a periodic fever. These fevers are the main characteristic of periodic fever syndromes or systemic autoinflammatory diseases (SAIDs), a group of disorders where the innate immune system is dysregulated, leading to episodes of inflammation without infection. Understanding this terminology is crucial for proper diagnosis and management.
Types of Periodic Fever Syndromes
Periodic fever syndromes are a diverse group, often genetic, primarily affecting children but also present in adults. Episode frequency, duration, and accompanying symptoms vary by syndrome.
Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA) Syndrome
PFAPA is a common periodic fever syndrome in children, usually starting before age five. It involves recurrent fevers (lasting 3-7 days) with mouth sores, sore throat, and swollen neck lymph nodes, often in a predictable pattern every 3-6 weeks. It is generally benign and may resolve in adolescence.
Familial Mediterranean Fever (FMF)
FMF, the most common genetically diagnosed recurrent fever syndrome, affects people of Mediterranean and Middle Eastern descent. Caused by a MEFV gene mutation, it leads to fever, painful inflammation in the abdomen, chest, and joints, and sometimes a rash. Untreated FMF can result in amyloidosis.
Tumor Necrosis Factor Receptor-Associated Periodic Syndrome (TRAPS)
TRAPS is a genetic disorder with potentially long-lasting fever episodes. Symptoms include fever, muscle pain, painful red skin rashes, and eye issues.
Hyperimmunoglobulin D Syndrome (HIDS)
Also known as Mevalonate Kinase Deficiency (MKD), this rare genetic condition often begins in infancy. Fevers are often accompanied by flu-like symptoms, abdominal pain, diarrhea, joint pain, rash, and swollen lymph nodes. Episodes can be triggered by stress, vaccinations, or minor infections.
Differential Diagnosis: Distinguishing Recurring Fevers
It is important to distinguish periodic fever syndromes from other causes of recurrent fever. Diagnosis involves history, physical exam, and tests.
Comparison of Fever Types
| Feature | Periodic Fever Syndrome | Relapsing Fever | Recurring Infection |
|---|---|---|---|
| Cause | Genetic or autoinflammatory | Bacterial infection (e.g., Borrelia bacteria) | Viral, bacterial, or fungal infection |
| Pattern | Predictable or irregular episodes, interspersed with well periods. | A characteristic cycling of fever episodes lasting days, separated by symptom-free weeks. | Multiple, separate infectious illnesses, often without a strict pattern. |
| Accompanying Symptoms | Mouth sores, rashes, joint/muscle pain, swollen glands. | Headache, muscle pain, chills. | Symptoms vary widely depending on the infection (e.g., sore throat, cough, fatigue). |
| Response to Treatment | May respond to anti-inflammatory medications, steroids, or biologics. | Responds to antibiotics. | Responds to appropriate treatment (e.g., antivirals, antibiotics) for each infection. |
The Diagnostic Process
Diagnosing a periodic fever syndrome involves careful observation and ruling out other conditions. This includes tracking fever episodes, blood tests for inflammation markers, genetic testing for hereditary syndromes, and excluding infections, autoimmune diseases, and malignancies.
Treatment and Management of Periodic Fevers
Treatment for periodic fever syndromes targets symptoms and prevents complications, varying by the specific syndrome.
Treatment Options
Treatment options include NSAIDs for symptom management, corticosteroids to stop fever episodes (especially in PFAPA), colchicine for FMF to prevent attacks and amyloidosis, biologics for severe cases, and sometimes tonsillectomy for PFAPA.
Management Strategies
Management also includes keeping detailed records of episodes, ensuring rest and hydration during fevers, and managing stress, which can trigger episodes.
Conclusion
A recurring fever can indicate a periodic fever syndrome, an autoinflammatory condition. Accurate diagnosis requires clinical observation, excluding other causes, and often genetic testing. Treatment aims to control symptoms and prevent complications. It is important to consult a healthcare provider, such as a pediatric rheumatologist, for evaluation of recurring fevers. For more information on autoinflammatory conditions, visit the National Institutes of Health website.
