Relapsing Polychondritis is a rare multisystem episodic autoimmune inflammatory disorder affecting cartilaginous and other connective tissues – characterised by lymphocytic infiltrate and inflammation in cartilage – humoral reactivity to collagen may play a role. Chondritis occurs in the cartilage in ear, nose, laryngotracheal, costal and articular cartilage – has recurrent and progressive episodes that result in destruction of the cartilage. Cause is unknown, but affecting cartilage suggest that a tissue specific antigen is involved. Associated with HLA-B27. Most common between ages of 40 and 60. M=F. Potentially fatal (tracheal collapse and obstruction of respiratory pathways)
Most common presenting features are acute pain, erythema and swelling of cartilaginous portion of external ears. Also can have respiratory involvement (develops in 50% poor prognosis), cardiovascular (20%), eye, renal (up to 10%) and skin symptoms (develop in 25% and may precede cartilaginous involvement).
A large number also have another rheumatological condition (usually rheumatoid arthritis or SLE) and/or haematological disorder.
Arthritis episodic and asymmetrical eventually develops in 75%. Arthritis is non-erosive and non-deforming – most commonly in ankle – also affects wrist, MCP’s, elbows and MPJ’s. Peripheral arthritis is associated with a poorer prognosis. Occasionally articular symptoms are the presenting feature.
Diagnosis is based on clinical and pathological features. Mean reported delay between initially seeking medical care and diagnosis is 2.9 years .
Involvement of the Foot:
Arthritis in ankle and metatarsophalangeal joint - non-deforming and non-erosive
Peripheral vascular disease
Initially with NSAID’s and/or colchicine. Later with corticosteroids and immunosuppressives.
Earlier literature puts survival of 70% at 4yrs; more recently 94% at 8 yrs.