TNF receptor 1-associated periodic fever syndrome
Findings
No curated finding names TNF receptor 1-associated periodic fever syndrome yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
A periodic fever syndrome, characterized by recurrent fever, arthralgia, myalgia and tender skin lesions lasting for 1 to 3 weeks, associated with skin, joint, ocular and serosal inflammation and complicated by secondary amyloidosis.
Definition from the Mondo Disease Ontology (MONDO:0007727), read 2026-09-29. CC BY 4.0.
- Inheritance
- Autosomal dominant inheritance
HPO, annotations 2026-09-02
Features
52 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Recurrent feverHPOHP:0001954
- 157 of 183 reported patients
- Very frequent (80% to 99% of cases)
- Abdominal painHPOHP:0002027
- 132 of 183 reported patients
- Very frequent (80% to 99% of cases)
- DiarrheaHPOHP:0002014
- Very frequent (80% to 99% of cases)
- Elevated circulating C-reactive protein concentrationHPOHP:0011227
- Very frequent (80% to 99% of cases)
- Elevated erythrocyte sedimentation rateHPOHP:0003565
- Very frequent (80% to 99% of cases)
- ErysipelasHPOHP:0001055
- 7 of 158 reported patients
Show the remaining 40
- ArthritisHPOHP:0001369
- Frequent (30% to 79% of cases)
- ConstipationHPOHP:0002019
- Frequent (30% to 79% of cases)
- ErythemaHPOHP:0010783
- Frequent (30% to 79% of cases)
- Increased total leukocyte countHPOHP:0001974
- Frequent (30% to 79% of cases)
- Intestinal obstructionHPOHP:0005214
- Frequent (30% to 79% of cases)
- LymphadenopathyHPOHP:0002716
- Frequent (30% to 79% of cases)
Genes
1 gene
Germline variants in the gene encoding each protein, as classified by GenCC submitters, each in their own words. Limited, disputed and refuted submissions are listed too.
- TNFRSF1AHGNC:11916
- Definitive · Ambry Genetics · Autosomal dominant · 2018
- Definitive · ClinGen · Autosomal dominant · 2025
- Definitive · Illumina · Autosomal dominant · 2020
- Definitive · G2P · Autosomal dominant · 2019
- Strong · Labcorp Genetics (formerly Invitae) · Autosomal dominant · 2022
- Strong · Laboratory for Molecular Medicine · Autosomal dominant · 2020
- Strong · PanelApp Australia · Autosomal dominant · 2025
- Supportive · Orphanet · Autosomal dominant · 2021
Where it sits
Other names
13 names
Resolves to: TNF receptor 1-associated periodic fever syndrome
- Also called
- familial Hibernian feverFHFHibernian fever, familialTNF receptor 1-associated periodic syndromeTNF receptor-associated periodic syndromeTRAPSTRAPS syndrometumor necrosis factor receptor 1 associated periodic syndrometumor necrosis factor receptor 1-associated periodic syndromeTumor Necrosis Factor Receptor-Associated Periodic Syndrometumour necrosis factor receptor 1 associated periodic syndrometumour necrosis factor receptor 1-associated periodic syndrometumour necrosis factor receptor-associated periodic syndrome