Relapsing polychondritis

What is relapsing polychondritis?

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

What are the clinical features of relapsing polychondritis?

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.

Relapsing polychondritis

Relapsing polychondritis

Relapsing polychondritis

What is the cause of relapsing polychondritis?

Antibodies to type 2 collagen in cartilage are found. As with other autoimmune diseases, exactly why these antibodies form is not known.

Polychondritis of the ear and/or nose can be a feature of VEXAS syndrome, an acquired X-linked autoinflammatory syndrome seen in middle-aged men.

What investigations should be undertaken?

A blood screen shows signs of inflammation. Antibody to type 2 collagen may be measured by certain specialist laboratories.

A tissue biopsy shows inflammation in the cartilage.

How is relapsing polychondritis treated?

Systemic corticosteroids (such as prednisone) remain the mainstay of treatment. Other treatments that are sometimes prescribed include:

• Dapsone
• Colchicine
• Azathioprine
• Methotrexate
• Cyclophosphamide
• Hydroxychloroquine
• Ciclosporin
• Infliximab.