Recurrent Fever Syndromes (aka Periodic Fever Syndromes): Symptoms, Causes, Treatment

What are the symptoms of recurrent fever syndromes?

Recurrent fever syndromes are characterized by episodes of fever that occur repeatedly over time, often with periods of normal temperature between episodes. Symptoms can vary depending on the specific syndrome and its underlying causes, but common symptoms include:

• Fever: Recurrent episodes of elevated body temperature, often higher than 100.4°F (38°C). The fever may be intermittent or cyclical, and the temperature can vary from mild to high.
• Chills and Excessive Sweating: Patients may experience chills during the onset of a fever and profuse sweating as the fever subsides.
• Fatigue: Generalized weakness and tiredness are common, particularly during or after fever episodes.
• Headache: Persistent or recurrent headaches may accompany fever episodes.
• Muscle Pain and Joint Pain: Body aches and muscle or joint pain can occur, often associated with fever and inflammation.
• Abdominal Pain: Some recurrent fever syndromes, such as periodic fever syndromes, can cause abdominal pain or discomfort.
• Rashes: Certain syndromes, such as Still’s disease, may present with rash along with recurrent fever.
• Swelling: Swelling in various parts of the body, such as lymph nodes or joints, may be observed in some recurrent fever syndromes.
• Gastrointestinal Symptoms: Nausea and vomiting, or diarrhea can occur with some recurrent fever syndromes.
• Behavioral Changes: In some cases, fever episodes may be associated with irritability, mood changes, or changes in behavior, particularly in children.

The specific symptoms and their severity can vary depending on the underlying condition causing the recurrent fever. Examples of recurrent fever syndromes include:

• Familial Mediterranean Fever (FMF)
• Still’s Disease (Systemic Juvenile Idiopathic Arthritis)
• Hyper-IgD Syndrome
• Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis (PFAPA) Syndrome
• Cyclic Neutropenia

If you or someone you know is experiencing recurrent fever, it is important to seek medical evaluation to determine the underlying cause and appropriate management.

What are the causes of recurrent fever syndromes?

Recurrent fever syndromes can arise from a variety of causes, including genetic, infectious, and inflammatory conditions. Here are some common causes:

Genetic and Autoinflammatory Conditions

• Familial Mediterranean Fever (FMF): A genetic disorder characterized by recurrent episodes of fever, abdominal pain, and inflammation, often caused by mutations in the MEFV gene.
• Still’s Disease (Systemic Juvenile Idiopathic Arthritis): A type of arthritis that causes recurrent fevers, rash, and joint inflammation. The exact cause is not fully understood but involves immune system dysfunction.
• Hyper-IgD Syndrome: A genetic disorder associated with recurrent fever, elevated levels of immunoglobulin D (IgD), and episodes of abdominal pain. It is caused by mutations in the MVK gene.
• Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis (PFAPA) Syndrome: A condition that causes recurrent fevers along with mouth ulcers, sore throat, and swollen lymph nodes. The cause is not entirely clear, but it may involve an immune system dysfunction.
• Cyclic Neutropenia: A rare genetic disorder that causes periodic drops in neutrophil levels, leading to recurrent infections, fever, and mouth ulcers.

Infectious Causes

• Recurrent Infections: Some individuals may experience repeated fevers due to chronic or recurring infections, such as bacterial, viral, or fungal infections. Conditions like tuberculosis or chronic sinusitis can also cause recurrent fever.
• Endocarditis: Infection of the heart valves can cause recurrent or persistent fever, along with other symptoms like heart murmurs and fatigue.

Inflammatory and Autoimmune Conditions

• Systemic Lupus Erythematosus (SLE): An autoimmune disorder that can cause recurrent fever, joint pain, skin rashes, and other systemic symptoms.
• Rheumatic Fever: A complication of untreated streptococcal throat infection that can cause fever, joint pain, and inflammation.
• Behçet’s Disease: A rare autoimmune condition characterized by recurrent oral ulcers and genital ulcers, eye inflammation, and fever.

Other Causes

• Cancer: Certain cancers, such as leukemia and lymphoma, can present with recurrent fevers as a symptom, often accompanied by other signs like weight loss or night sweats.
• Medications: Some medications can induce recurrent fever as a side effect or allergic reaction.
• Unknown Etiology: In some cases, the cause of recurrent fever may not be immediately identifiable, leading to the diagnosis of idiopathic recurrent fever.

Diagnosing the underlying cause of recurrent fever syndromes often involves a thorough medical evaluation, including detailed patient history, physical examination, and a range of diagnostic tests. If you or someone you know is experiencing recurrent fever, seeking medical advice is crucial for proper diagnosis and treatment.

What is the treatment for recurrent fever syndromes?

Treatment for recurrent fever syndromes depends on the underlying cause of the fevers. Here are common approaches based on different types of recurrent fever syndromes:

Genetic and Autoinflammatory Conditions

• Familial Mediterranean Fever (FMF): Treatment typically involves colchicine, a medication that helps prevent fever episodes and inflammation. Long-term management may also include lifestyle adjustments and monitoring for potential complications.
• Still’s Disease (Systemic Juvenile Idiopathic Arthritis): Management may include nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroids, and disease-modifying antirheumatic drugs (DMARDs) to control inflammation and fever. Biologic agents may also be used in more severe cases.
• Hyper-IgD Syndrome: Treatment often involves colchicine to reduce the frequency and severity of fever episodes. Supportive care and monitoring are also essential.
• PFAPA Syndrome: Treatment may include corticosteroids to manage acute fever episodes and, in some cases, tonsillectomy may be considered if the condition is severe and persistent.
• Cyclic Neutropenia: Management focuses on supportive care and may include granulocyte colony-stimulating factor (G-CSF) to stimulate white blood cell production and reduce the frequency of neutropenic episodes.

Infectious Causes

• Antibiotic Therapy: For recurrent fevers caused by bacterial infections, targeted antibiotics are prescribed based on the specific infection.
• Antiviral or Antifungal Medications: If recurrent fevers are due to viral or fungal infections, appropriate antiviral or antifungal treatments are used.
• Management of Chronic Infections: Chronic infections may require long-term or repetitive treatment strategies to control symptoms and prevent recurrence.

Inflammatory and Autoimmune Conditions

• Systemic Lupus Erythematosus (SLE): Treatment includes corticosteroids and immunosuppressive drugs to manage inflammation and control autoimmune activity. Disease-modifying drugs may also be used.
• Rheumatic Fever: Management involves antibiotics to eradicate streptococcal infection and anti-inflammatory medications to control symptoms and prevent complications.
• Behçet’s Disease: Treatment often includes corticosteroids and immunosuppressive drugs to control inflammation and symptoms.

Other Causes

• Cancer: Treatment for cancers associated with recurrent fever may include chemotherapy, radiation therapy, or targeted therapies, depending on the type and stage of the cancer.
• Medication-Induced Fever: Discontinuing or adjusting the offending medication under medical supervision can help resolve fever caused by drug reactions.
• Supportive Care: In cases where the cause of recurrent fever is idiopathic or not fully understood, supportive care including pain management, hydration, and monitoring for complications is essential.

General Strategies

• Monitoring and Follow-Up: Regular follow-up with healthcare providers is crucial to monitor the effectiveness of treatment and make necessary adjustments.
• Lifestyle Modifications: For some conditions, lifestyle changes such as maintaining good hygiene, avoiding known triggers, or adjusting activity levels can help manage symptoms.
• Patient Education: Educating patients and their families about the condition and how to manage symptoms can be an important part of treatment.

The treatment plan should be tailored to the individual’s specific diagnosis and needs. Consulting with a healthcare professional for a comprehensive evaluation and personalized treatment plan is essential for effective management of recurrent fever syndromes.