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

Hansson AS, Holmdahl R - Arthritis Res. (2002)

Bottom Line: 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.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.

Affiliation: Department of Clinical Immunology, Sahlgrenska University Hospital, Gothenburg, Sweden. ann-sofie.hansson@vgregion.se

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.

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.
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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.

Mentions: The most characteristic symptom is an inflammation of the external ear, subsequently leading to 'cauliflower' ears with sometimes severe tissue deformation [4,5]. Large and peripheral small joints are affected by inflammation in an asymmetric, episodic and migratory manner. Contrary to RA, no rheumatic factor is detected in sera from RP patients and the arthritis is considered to be non-erosive, although erosions may appear in late stages of the disease. Inflammation and erosion of cartilage in the nasal septum is common and occasionally results in the appearance of a 'saddle-nose' deformity (Fig. 1). In half of the RP patients the cartilage of the tracheolaryngeal tract is affected. This is a potentially fatal symptom caused by a collapse of tracheal rings and bronchi and/or by inflammatory obstruction of the airways. These patients experience dyspnea, cough, stridor and (in some cases) symptoms from severe cyanosis. One fifth of the patients develop complications from nephritis, which is commonly associated with extrarenal vasculitis. Noncartilaginous structures such as the skin and cardiovascular system are commonly affected by inflammation as well, the latter accounting for a fatal outcome in some patients. All symptoms appear in episodes and are most often found as manifestations from chondritis at specific sites.

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

Hansson AS, Holmdahl R - Arthritis Res. (2002)

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.
© Copyright Policy
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.
Mentions: The most characteristic symptom is an inflammation of the external ear, subsequently leading to 'cauliflower' ears with sometimes severe tissue deformation [4,5]. Large and peripheral small joints are affected by inflammation in an asymmetric, episodic and migratory manner. Contrary to RA, no rheumatic factor is detected in sera from RP patients and the arthritis is considered to be non-erosive, although erosions may appear in late stages of the disease. Inflammation and erosion of cartilage in the nasal septum is common and occasionally results in the appearance of a 'saddle-nose' deformity (Fig. 1). In half of the RP patients the cartilage of the tracheolaryngeal tract is affected. This is a potentially fatal symptom caused by a collapse of tracheal rings and bronchi and/or by inflammatory obstruction of the airways. These patients experience dyspnea, cough, stridor and (in some cases) symptoms from severe cyanosis. One fifth of the patients develop complications from nephritis, which is commonly associated with extrarenal vasculitis. Noncartilaginous structures such as the skin and cardiovascular system are commonly affected by inflammation as well, the latter accounting for a fatal outcome in some patients. All symptoms appear in episodes and are most often found as manifestations from chondritis at specific sites.

Bottom Line: 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.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.

Affiliation: Department of Clinical Immunology, Sahlgrenska University Hospital, Gothenburg, Sweden. ann-sofie.hansson@vgregion.se

ABSTRACT

Background: 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.

View Similar Images In: Results  - Collection
View Article: Pubmed Central -  PubMed
Show All Figures - Show MeSH
getmorefigures.php?pmc=128937&rFormat=json&query=null&req=5