Relapsing Polychondritis

Wikis > Rheumatology > Connective Tissue Disorders > Relapsing Polychondritis

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)

Relapsing Polychondritis

Typical ear inflammation in relapsing polychondritis.

Clinical features:
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

Treatment:
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.

Comments are closed.