Familial Cold Autoinflammatory Syndrome 4 C4015276
A rare genetic autoinflammatory disorder where cold exposure triggers flare-ups of fever, skin rash, and joint discomfort. Part of the CAPS family of conditions. Anti-IL-1 therapies are commonly used for symptom control.
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)Fatigue
(immunologic)Fever
(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 Avoidance
Systematic avoidance of cold environments and triggers including cold air, water, and objects to prevent autoinflammatory episodes.
Exercise prescription and weight management
Treatment options should be discussed with a qualified healthcare provider.
NSAIDs
Non-steroidal anti-inflammatory drugs to reduce inflammation and manage acute symptoms during cold-triggered episodes.
Additional treatment options
IL-1 Inhibitors
Biologic agents such as anakinra or canakinumab that target interleukin-1 pathway to prevent inflammatory episodes.
medicationCorticosteroids
Short-term systemic corticosteroids to manage severe inflammatory flares when other treatments are insufficient.
medicationSymptom and Trigger Diary
Regular documentation of exposure events, symptoms, and response to treatments to optimize management strategies.
monitoringWhen 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 4 C4015276?
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.
Hoffman HM, Broderick GV. Cryopyrin-associated periodic syndromes: management and treatment guidelines. J Allergy Clin Immunol Pract. 2022.
Aksentijevich I, Kastner DL. Autoinflammatory diseases and the NLRP12 gene:Familial Cold Autoinflammatory Syndrome type 4. Immunol Rev. 2021.
De Benedetti F, Gattorno M, Frenkel J. Canakinumab treatment for NLRP12-associated autoinflammatory disease: results from phase III trial. Ann Rheum Dis. 2020.
Sarrabay G, Mechin V, Grandemenge S. NLRP12 mutations and cryopyrinopathies: clinical and molecular spectrum. Nat Rev Rheumatol. 2019.
Medication Comparisons
Compare medications used to treat Familial Cold Autoinflammatory Syndrome 4 C4015276. 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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy