autoinflammatory syndrome
MONDO:0019751Mondo
Findings
No curated finding names autoinflammatory syndrome yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
A group of disorders of the innate immune system characterized by attacks of seemingly unprovoked inflammation without significant levels of either autoantibodies or autoreactive T cells more characteristic of autoimmune disease.
Definition from the Mondo Disease Ontology (MONDO:0019751), read 2026-09-29. CC BY 4.0.
Genes
8 genes
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.
- DPP9HGNC:18648
- Strong · PanelApp Australia · Autosomal dominant · 2025
- GIMAP6HGNC:21918
- Strong · PanelApp Australia · Autosomal recessive · 2025
- IL36RNHGNC:15561
- Strong · PanelApp Australia · Semidominant · 2025
- UNC93B1HGNC:13481
- Strong · PanelApp Australia · Autosomal dominant · 2025
- FGL2HGNC:3696
- Moderate · PanelApp Australia · Autosomal recessive · 2025
- PMVKHGNC:9141
- Moderate · PanelApp Australia · Autosomal recessive · 2025
- Limited · Ambry Genetics · Autosomal recessive · 2025
- PSTPIP1HGNC:9580
- Moderate · Ambry Genetics · Autosomal dominant · 2025
- PTPN1HGNC:9642
- Limited · Ambry Genetics · Autosomal dominant · 2025
Where it sits
- A kind of
- Narrower terms (37)
- adult-onset Still disease
- autoinflammation-PLCG2-associated antibody deficiency-immune dysregulation
- autoinflammatory disease, multisystem, with immune dysregulation, X-linked
- autoinflammatory disease, systemic, with vasculitis
- autoinflammatory disease, X-linked
- autoinflammatory syndrome due to TBK1 deficiency
- autoinflammatory syndrome of childhood
- autoinflammatory syndrome with immunodeficiency
- autoinflammatory syndrome with pyogenic bacterial infection and amylopectinosis
- autoinflammatory syndrome, familial, Behcet-like
- CEBPE-associated autoinflammation-immunodeficiency-neutrophil dysfunction syndrome
- cherubism
- chronic recurrent multifocal osteomyelitis
- early-onset pulmonary and cutaneous vasculitis
- F12-associated cold autoinflammatory syndrome
- granulomatous autoinflammatory syndrome of childhood