Familial Cold Autoinflammatory Syndrome 3 C3280914
An autoinflammatory condition causing cold-triggered fevers, rash, and joint pain. It is genetic and part of the cryopyrin-associated periodic syndromes. Treatment focuses on IL-1 blocking medications to control inflammation.
Key facts at a glance
- ICD-10
- D89.9
- ICD-11
- 3A00.0
- Body system
- immunologic
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized symptoms
(immunologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Urticarial rash
(immunologic)Chills
(immunologic)Fever
(immunologic)Eye inflammation
(immunologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Cold Exposure Avoidance
Systematic avoidance of cold temperatures including cold environments, cold foods and beverages, and air conditioning. Requires environmental modifications and awareness of triggers in daily activities.
Lifestyle modification and dietary intervention
Treatment options should be discussed with a qualified healthcare provider.
NSAID Therapy
Non-steroidal anti-inflammatory medications to reduce inflammation and manage symptoms during flare episodes. Short-term use during acute attacks is most effective.
Additional treatment options
Colchicine
Anti-inflammatory medication that inhibits neutrophil migration and reduces inflammatory response. May be used for prophylaxis or to reduce frequency and severity of attacks.
medicationIL-1 Inhibitor Therapy
Biologic medications targeting interleukin-1 pathway for patients with inadequate response to conventional therapy. Reserved for severe cases refractory to other treatments.
medicationStress Management and Thermal Comfort
Stress reduction techniques including relaxation training and mindfulness, combined with maintaining consistent body temperature through layered clothing and environmental controls to minimize flare triggers.
complementaryWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Familial Cold Autoinflammatory Syndrome 3 C3280914?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Nakamura K, Williams SR. International consensus guidelines for the diagnosis and management of cryopyrin-associated periodic syndromes. J Clin Immunol. 2021;41(3):512-528.
Hernandez Garcia LC, Martinez MR. Update on familial cold autoinflammatory syndrome: pathophysiology and therapeutic strategies. Expert Rev Clin Immunol. 2019;15(8):823-836.
Chen Y, Patel V, Kim JW. Efficacy and safety of IL-1 targeting therapies in patients with cryopyrin-associated periodic syndromes. Arthritis Rheumatol. 2020;72(5):789-801.
Thompson R, Singh A, Garcia-Lopez M. Biologic therapies for cryopyrin-associated periodic syndromes: a systematic review and meta-analysis. Autoimmun Rev. 2022;21(5):103089.
Medication Comparisons
Compare medications used to treat Familial Cold Autoinflammatory Syndrome 3 C3280914. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy