Cartilage-specific autoimmunity in animal models and clinical aspects in patients - focus on relapsing polychondritis

Abstract

Relapsing polychondritis is an autoimmune disease in which an inappropriate immune response destroys cartilage. Cartilage of the ears, larynx and nose rather than spine and joint cartilage is affected by a chronic relapsing and erosive inflammation. Several animal models for relapsing polychondritis have been published in which immunization with various cartilage proteins induces a variety of chondritis symptoms that mimic those seen in patients. In this review we describe the collagens, matrilin-1 and cartilage oligomeric matrix protein as potential autoantigens able to trigger the tissue-specific immune response seen both in patients and in animal models for relapsing polychondritis and related autoimmune diseases.

Figure 1

Patient with RP presenting nasal chondritis and saddle-nose deformity. Reproduced courtesy of Dr S Foster, Immunology and Uveitis Service, Massachusetts Eye & Ear Infirmary, Boston, MA, USA.

Figure 2

Sections of cartilaginous tissues from a female LEW.1F rat immunized with matrilin-1 [3]. An erosive inflammation is detected in the nasal septum (a) and in the laryngeal part of the respiratory tract (b) with an influx consisting mainly of neutrophils but also of lymphocytes, macrophages and eosinophils. Hematoxylin and erythrosine staining. Reproduced with the permission of the American Society for Clinical Investigation Inc.