Relapsing Polychondritis :-
Characterized by inflammation of cartilage affecting the ears, nose and laryngotracheobronchial tree.
HLA DR 4 association
SYSTEMIC VASCULITIS is the most common association, followed by Rheumatoid arthritis, and SLE.
Histologically earliest abnormality of hyaline and elastic cartilage is noted.
Both cell mediated and humoral immunity plays an important role in the pathogenesis.
Antibodies to Type II collagen and to Matralin 1 and immune complexes are detected in sera of patients.
Matralin 1 is a non collageneous protein present in the extracellular matrix in cartilage ( higher concentration in trachea and also in nasal septum)
Auricular chrondritis is the most frequent manifestation of Relapsing Polychondritis followed by Arthritis.
Features of VASCULITIS are seen in 25 % of patients and can affect any size vessel.