Relapsing polychondritis (Q44617): Difference between revisions
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A policondrite recidivante é uma doença inflamatória multissistêmica de etologia desconhecida que acomete a cartilagem. É caracterizada por manifestações inflamatórias intermitentes ou flutuantes, devidas a inflamação nas estruturas cartilaginosas, resultando em dano e destruição teciduais. Condrite das cartilagens auricular, nasal e traqueal predominam nessa doença, o que sugere uma resposta inflamatória a antígenos teciduais específicos, como o colágeno do tipo II e a proteína de matriz cartilaginosa (matrilin-1). Em aproximadamente um terço dos pacientes, a PR está associada a vasculite (desde vasculite leucocitoclástica cutânea isolada até poliangeite sistêmica) e doenças reumáticas autoimunes (principalmente a artrite reumatoide e o lúpus eritematoso sistêmico). Neoplasias hematológicas malignas, transtornos gastrointestinais e doenças endócrinas podem ocorrer. A avaliação funcional e anatômica para doenças de vias aéreas superiores e inferiores é essencial na avaliação e manejo desta doença. | |||
| description / en | description / en | ||
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. | |||
Revision as of 12:10, 13 August 2026
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.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | FB82.3 |
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| English | Relapsing polychondritis |
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. |
