Knowra Relapsing polychondritis Relapsing polychondritis Relapsing polychondritis is a rare inflammatory disease that repeatedly attacks cartilage and other tissues. It can damage the ears, nose, joints, eyes, and airways.
Cartilage : Cartilage is a flexible connective tissue that supports structures and cushions joints. Its vulnerability to recurrent inflammation defines the disease’s characteristic pattern.
Auricular chondritis : Auricular chondritis is inflammation of the cartilage of the external ear. A red, painful pinna that spares the earlobe is a hallmark presentation.
McAdam criteria : The McAdam criteria are clinical features used to identify relapsing polychondritis. They organize diagnosis around characteristic cartilage, eye, hearing, and vestibular findings.
VEXAS syndrome : VEXAS syndrome is an adult-onset inflammatory disease caused by acquired UBA1 mutations in blood-forming cells. It can mimic relapsing polychondritis, particularly in older men with systemic inflammation and blood abnormalities.
Autoimmunity : Autoimmunity occurs when the immune system attacks the body's own tissues. Immune-mediated inflammation is thought to drive cartilage injury, although the precise cause remains unknown.
Nasal chondritis : Nasal chondritis is inflammation of cartilage in the nose. Repeated attacks can damage the nasal bridge and produce a saddle-nose deformity.
Damiani and Levine criteria : The Damiani and Levine criteria are diagnostic rules for relapsing polychondritis that incorporate treatment response and histology. They broaden clinical classification beyond the original McAdam criteria.
Myelodysplastic syndrome : Myelodysplastic syndrome is a group of bone marrow disorders that impair normal blood-cell production. It is associated with relapsing polychondritis and may signal an underlying hematologic disorder.
Tracheobronchomalacia : Tracheobronchomalacia is weakness and excessive collapse of the trachea or bronchi during breathing. Airway cartilage damage can leave the trachea and bronchi weak and prone to collapse.
Laryngotracheal involvement in relapsing polychondritis : Laryngotracheal involvement is inflammation and damage affecting the larynx, trachea, or bronchi. Airway disease can cause hoarseness, breathlessness, or life-threatening obstruction.
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