Familial Mediterranean fever

ICD-11 code : 4A60.0;

Preferred Label : Familial Mediterranean fever;

ICD-11 definition : FMF is an autoinflammatory disease associated with mutations in pyrin resulting in enhanced IL1 beta production. This results in clinical attacks of inflammation in the form of fever and serositis in the form of peritoneal, pleural or synovial inflammation along with increased acute phase reactants.;

ICD-11 synonym : Hereditary amyloid nephropathy; periodic familial polyserositis; periodic polyserositis; Armenian disease; FMF - [familial mediterranean fever]; Periodic disease; familial recurrent polyserositis; Riemann periodic disease; periodic fever; periodic familial peritonitis; paroxysmal polyserositis; familial non-neuropathic amyloidosis;

ICD-11 acronym : FMF;

Details

Origin ID

1373335705;

Automatic exact mappings (from CISMeF team)
- Hereditary amyloidosis with primary renal involvement [ORDO Disease]
- Nephrosis [ICPC-3 diagnosis]
- Periodic fever [HPO term]
Currated CISMeF NLP mapping
- Familial Mediterranean Fever [NCIt concept]
- Familial Mediterranean fever [ORDO Disease]
- Familial Mediterranean fever [ICD-9 code]
- Familial Mediterranean fever (disorder) [SNOMED CT concept]
- Familial mediterranean fever [MedDRA Preferred Term]
- Familial mediterranean fever [OMIM Phenotype]
- Familial recurrent polyseritis [PASCAL descriptor]
- Hereditary periodic fever (disorder) [SNOMED CT concept]
- Periodic fever [MedDRA LLT]
- familial Mediterranean fever [DO Concept]
- familial mediterranean fever [MeSH Descriptor]
- familial mediterranean fever [MeSH concept]
- familial mediterranean fever [Disease (Nov.)]
- periodic disease [SNOMED Notion]
False automatic mappings
- Familial recurrent polyserositis [MedDRA LLT]
- relapsing fever [MeSH Descriptor]
ICD-10 Mapping
- Other specified disorders involving the immune mechanism, not elsewhere classified [ICD-10 Sub-category]
See also inter- (CISMeF)
- Familial non-neuropathic amyloidosis (disorder) [SNOMED CT concept]
- MEFV innate immuity regulator, pyrin [ORDO Gene]
- Non-neuropathic heredofamilial amyloidosis [ICD-10 Sub-category]
Validated automatic mappings to NTBT
- Other specified disorders involving the immune mechanism, not elsewhere classified [ICD-10 Sub-category]

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