Relapsing polychondritis is a rare autoimmune disease that causes episodic inflammation and degeneration of cartilaginous structures like the ears, nose, and respiratory tract. It is characterized by recurrent chondritis of both ears (McAdam's sign), nonerosive arthritis, ocular inflammation, laryngeal/tracheal chondritis, and hearing loss/tinnity/vertigo from cochlear damage. Diagnosis is based on clinical criteria including involvement of multiple cartilaginous sites and response to steroids. High doses of prednisone are typically used to induce remission, with lower doses for maintenance to prevent recurrences. Immunosuppressants may also be used to reduce steroid

1. Relapsing polychondritis
Definition; Relapsing polychondritis is a rare disease of episodic inflammation & degeneration
involving multiple cartilaginous structures usually those of the upper respiratory tract,as well as
connective tissue at various other sites. Other names polychondropathy,systemic chondromalacia,
chronic atropic polychondritis.
Pathology
All types of cartilage may be involved. Initially neutrophil infiltration & later infiltration by
histocytes ,plasma cells & lymphocytes .
Other protreoglycan-rich structures,such as the eyes &the cardiovascular system, may also undergo
similar inflammation & degeneration.
Aetiology
Autoimmune.
Diagnosis;
Diagnostic sign for relapsing polychondritis ( Mc Adam’s sign)
Recurrent chondritis of both auricles;
Nonerosive inflammatory polyarthritis
Chondritis of nasal cartilages,
Ocular inflammation including conjunctivitis, keratitis, scleritis/episcleritis and/or uveitis,
Chondritis of the respiratory tract involving laryngeal and/or tracheal cartilage,
Cochlear and/or vestibular damage manifested by neurosensory hearing loss ,tinnitus and/or
vertigo.
These were modified by Damiani& Levine to the following criteria:
Three or more of Mc Adam’s sign-histologic confirmation not necessary;
One or more of Mc Adam’s sign with positive histologic confirmation
Involvement of two or more separate anatomic locations with response to steroid and/or
dapsone.
D/D
Perichondritis
Wegner’s granulomatosis
T-cell lymphomas

2. Tuberculosis
Sarcoidosis
Outcomes
The disease tends to follow a course of acute exacerbation & remissions. Long-term consequences
are largely the result of cartilaginous destruction.
Management options;
High doses 30-60mg prednisolone/daily may be required to induce remission & maintenance 5-10mg
to suppress recurrences
Immunosuppressants have been utilized in an attempt to reduce steroid doses particularly
azathioprine or methotrexate. Dapsone has been advocated by some authors.
The use of anti-CD4 monoclonal antibody & oral minocycline have recently been reported.
Best clinical practice;
The diagnosis of polychondritis is primarily a clinical one.
Steroids would appear to be first option.
Assuming an autoimmune aetiology, others immunosuppressants would be logical.