ICD-11 code : FB82.3;
Preferred Label : Relapsing polychondritis;
ICD-11 definition : Relapsing polychondritis is a multisystem inflammatory disease of unknown aetiology
affecting the cartilage. The disease is characterised by intermittent or fluctuant
inflammatory manifestations due to inflammation of the cartilaginous structures, resulting
in tissue damage and tissue destruction. Chondritis of auricular, nasal, tracheal
cartilage predominates in this disease, suggesting response to tissue-specific antigens
such as collagen II and cartilage matrix protein (matrillin-1). In about one third
of patients, RP is associated with vasculitis (from isolated cutaneous leucocytoclastic
vasculitis to systemic polyangiitis) and autoimmune rheumatic diseases (mainly rheumatoid
arthritis and systemic lupus erythematosus). Haematological malignant diseases, gastrointestinal
disorders and endocrine diseases may also occur. Functional and anatomical evaluation
for upper and lower airway disease is essential in evaluation and management of the
disease.;
Origin ID : 1412888287;
UMLS CUI : C0032453;
Currated CISMeF NLP mapping
- ICD-10 Mapping
- Semantic type(s)
- UMLS correspondences (same concept)
Relapsing polychondritis is a multisystem inflammatory disease of unknown aetiology
affecting the cartilage. The disease is characterised by intermittent or fluctuant
inflammatory manifestations due to inflammation of the cartilaginous structures, resulting
in tissue damage and tissue destruction. Chondritis of auricular, nasal, tracheal
cartilage predominates in this disease, suggesting response to tissue-specific antigens
such as collagen II and cartilage matrix protein (matrillin-1). In about one third
of patients, RP is associated with vasculitis (from isolated cutaneous leucocytoclastic
vasculitis to systemic polyangiitis) and autoimmune rheumatic diseases (mainly rheumatoid
arthritis and systemic lupus erythematosus). Haematological malignant diseases, gastrointestinal
disorders and endocrine diseases may also occur. Functional and anatomical evaluation
for upper and lower airway disease is essential in evaluation and management of the
disease.
