Short answer · Medically reviewed summary · Last updated: 2026-05-08
TNF Receptor Associated Periodic Syndrome (TRAPS) is classified under the ICD-10-CM code E85.0 (Hereditary amyloidosis, unspecified) as there is no unique code specifically for this autoinflammatory condition. Under the older ICD-9-CM classification system, it was categorized as 277.30 (Amyloidosis, unspecified), as the risk of secondary AA amyloidosis is a major clinical concern in patients with TNF Receptor Associated Periodic Syndrome. What is the clinical significance of these codes for TRAPS? Because TNF Receptor Associated Periodic Syndrome is a rare autoinflammatory disorder, it does not have a dedicated ICD-10 code.
ICD10 code of TNF Receptor Associated Periodic Syndrome and ICD9 code
ICD-10 and ICD-9 codes for TNF Receptor Associated Periodic Syndrome, with classification details for clinicians, coders and patients.
TNF Receptor Associated Periodic Syndrome (TRAPS) is classified under the ICD-10-CM code E85.0 (Hereditary amyloidosis, unspecified) as there is no unique code specifically for this autoinflammatory condition. Under the older ICD-9-CM classification system, it was categorized as 277.30 (Amyloidosis, unspecified), as the risk of secondary AA amyloidosis is a major clinical concern in patients with TNF Receptor Associated Periodic Syndrome.
What is the clinical significance of these codes for TRAPS?
Because TNF Receptor Associated Periodic Syndrome is a rare autoinflammatory disorder, it does not have a dedicated ICD-10 code. Clinicians often use E85.0 to capture the systemic nature of the condition, particularly the risk of protein deposition. Accurate coding is essential for managing the long-term inflammatory burden associated with TNF Receptor Associated Periodic Syndrome.
How is TNF Receptor Associated Periodic Syndrome diagnosed?
Diagnosis of TNF Receptor Associated Periodic Syndrome is primarily clinical, supported by molecular genetic testing. It is caused by autosomal dominant mutations in the TNFRSF1A gene. Key diagnostic features include:
- Prolonged fever episodes lasting longer than one week.
- Migratory skin rashes and intense muscle pain (myalgia).
- Periorbital edema (swelling around the eyes).
- Elevated acute-phase reactants (CRP, SAA) during flares.
Is there support for patients with this condition?
Living with a rare diagnosis can feel isolating. At DiseaseMaps.org, we currently connect 4 members who share their personal experiences with TNF Receptor Associated Periodic Syndrome. Connecting with others who understand the unique challenges of this autoinflammatory disease can provide significant emotional relief and practical insights into symptom management.
Next steps
- Consult with a rheumatologist or an immunologist who specializes in autoinflammatory diseases.
- Undergo genetic counseling to discuss the autosomal dominant inheritance pattern of TNF Receptor Associated Periodic Syndrome.
- Join a patient support group or the DiseaseMaps community to connect with other families.
- Monitor for signs of AA amyloidosis, a rare but serious complication, through regular clinical follow-ups.
Medical disclaimer: This information is for educational purposes only and should not replace professional medical advice, diagnosis, or treatment.
References
- NIH Genetic and Rare Diseases Information Center (GARD): TNF Receptor-Associated Periodic Syndrome
- Orphanet: ORPHA896 (TNF receptor-associated periodic syndrome)
- OMIM: #142680 (TNF Receptor-Associated Periodic Syndrome; TRAPS)
- Autoinflammatory Alliance: Patient resources for TRAPS
