Relapsing polychondritis

Author: Dr Mark Duffill, Dermatologist, Hamilton, New Zealand, 2008.

Synonyms:

Meyenburg Altherr Uehlinger syndrome, Meyenburg disease, Chronic polychondritis, Systemic chondromalacia, Atrophic polychondritis, Polychondropathia

Categories:

Other inflammatory disorder, Immune disorder, Systemic disorder

Subcategories:

Autoimmune, Collagen type 2 autoantibodies, Inflammation of cartilage, Involvement of ear and nose

ICD-10:

M94.1

ICD-11:

FB82.3

SNOMED CT:

72275000, 121237000

What is relapsing polychondritis?

Relapsing polychondritis is a rare autoimmune disorder characterised by recurrent episodes of inflammation and destruction of cartilaginous tissues.

Relapsing polychondritis most commonly presents as inflammation of the cartilage of the ears and nose.

Inflammation of the cartilage of the ears, with red ears resembling infectious cellulitis, is the most common finding. The ear lobes, containing no cartilage, are classically spared by the inflammation. Chronic disease may result in a flabby, droopy ear or in a cauliflower ear.
Involvement of the nose may result in saddle nose deformity.
Acute involvement of the cartilage of the trachea (windpipe) may cause the collapse of the airway with obstruction and subsequent lung infections.
Arthritis may affect one joint or more than one joint, most often the cartilaginous junctions of the ribs.
Other manifestations may include damage to the heart valves, nervous system, eye and kidney disease.