A new animal model for relapsing polychondritis, induced by cartilage matrix protein (matrilin-1).
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Relapsing polychondritis (RP) differs from rheumatoid arthritis (RA) in that primarily cartilage outside diarthrodial joints is affected. The disease usually involves trachea, nose, and outer ears. To investigate whether the tissue distribution of RP may be explained by a specific immune response, we immunized rats with cartilage matrix protein (matrilin-1), a protein predominantly expressed in tracheal cartilage. After 2-3 weeks, some rats developed a severe inspiratory stridor. They had swollen noses and/or epistaxis, but showed neither joint nor outer ear affection. The inflammatory lesions involved chronic active erosions of cartilage. Female rats were more susceptible than males. The disease susceptibility was controlled by both MHC genes (f, l, d, and a haplotypes are high responders, and u, n, and c are resistant) and non-MHC genes (the LEW strain is susceptible; the DA strain is resistant). However, all strains mounted a pronounced IgG response to cartilage matrix protein. The initiation and effector phase of the laryngotracheal involvement causing the clinical symptoms were shown to depend on alphabeta T cells. Taken together, these results represent a novel model for RP: matrilin-1-induced RP. Our findings also suggest that different cartilage proteins are involved in pathogenic models of RP and RA. (+info)
Autoreactivity against matrilin-1 in a patient with relapsing polychondritis.
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Relapsing polychondritis (RP) is a rare inflammatory disease of cartilage. Chondritis of the auricular, nasal, and tracheal cartilages predominates in this disease, suggesting a response to a tissue-specific antigen. One potential antigen is matrilin-1, a cartilage matrix protein found uniquely in the tracheal, auricular, and nasal cartilage of adults. We describe herein a patient with RP who had both a humoral and a cellular immune response directed toward the cartilage matrix protein matrilin-1. (+info)
Tracheobronchial involvement in relapsing polychondritis.
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Relapsing polychondritis (RPC) is a multisystem disorder of chondromalacia involving any cartilage. Respiratory tract involvement is the greatest threat to life. We report a patient with stenosis of the subglottic trachea and left main bronchus who suddenly ceased breathing. As this patient did not have any other clinical features of RPC, the diagnosis was difficult. CT showed circumferential worm-eaten-like thickening suggesting a deformity and edema of the tracheal mucosa. Biopsy of the tracheal and thyroid cartilage revealed mild cartilage degeneration and infiltration with inflammatory cells. Therefore, the patient was diagnosed as having RPC. She is currently well 24 months after Montgomery T tube intubation with systemic steroids. Narrowing of the left main bronchus has not worsened. (+info)
Autologous stem-cell transplantation in refractory autoimmune diseases after in vivo immunoablation and ex vivo depletion of mononuclear cells.
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Autoimmune diseases that are resistant to conventional treatment cause severe morbidity and even mortality. In the present study we demonstrate that complete remissions can be achieved in refractory polychondritis and systemic lupus erythematosus (SLE), even at advanced stage, with the use of autologous stem-cell transplantation (SCT). Remissions persisted after reconstitution of the immune system. In the treatment of advanced systemic sclerosis (SSc), stable disease may be achieved with autologous SCT. (+info)
Orbital mucosa-associated lymphoid tissue (MALT)-type lymphoma in a patient with relapsing polychondritis.
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Relapsing polychondritis is characterized by recurrent inflammation of the cartilaginous tissues of the ears, nose, peripheral joints, and the tracheobronchial tree. The eye is also a frequent target organ in relapsing polychondritis, and proptosis is a well-recognized manifestation of eye involvement. Similar to other rheumatologic diseases, an association of relapsing polychondritis with malignancy has been reported. We describe a patient with relapsing polychondritis who presented with exophthalmos. When treatment directed toward control of her underlying disease was only partially effective, further investigation revealed that she had an orbital mucosa-associated lymphoid tissue (MALT)-type B cell lymphoma. We hypothesize that the lymphoma resulted from malignant transformation of the relapsing polychondritis-induced inflammatory pseudotumor and emphasize that neoplastic disease should be considered in the differential diagnosis in patients with relapsing polychondritis presenting with exophthalmos. (+info)
Active aortitis in relapsing polychondritis.
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Relapsing polychondritis (RP) is a rare inflammatory multiorgan disorder affecting cartilaginous structures and other connective tissues. Serious cardiovascular complications have been reported in patients with RP, the most frequent being aortic or mitral regurgitation and aortic aneurysms. Aortitis is a very rare complication. An unusual case of active aortitis in a patient with RP, despite intensive immunosuppressive treatment, is described with a special emphasis on the pathological findings. (+info)
Pulmonary fibrosis with intractable pneumothorax: new pulmonary manifestation of relapsing polychondritis.
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Relapsing Polychondritis is a rare disease which causes the repetitive inflammation of cartilage and connective tissues. Although the large airway is sometimes involved and the stenosis of them often influences the prognosis of the patients, there have been few reports concerning the manifestation of the peripheral lung. A 60-year-old man with pulmonary fibrosis was admitted to a regional hospital due to sudden deafness, and then he suffered from relapsing polychondritis. During the steroid therapy, he also suffered from bilateral pneumothoraces. His computed tomogram revealed many bilateral bullae, emphysematous changes, and fibrotic changes in bilateral lungs. The mechanism of generating peripheral pulmonary manifestations is also discussed. (+info)
Identification of type II collagen peptide 261-273-specific T cell clones in a patient with relapsing polychondritis.
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OBJECTIVE: To characterize and clone T cells specific for type II collagen (CII) in a patient with relapsing polychondritis (RP) and to establish whether the immunodominant epitope of CII determined in HLA transgenic mice is used in the human autoimmune response to CII. METHODS: T cell responses to CII were examined in a patient with RP, who was heterozygous for the HLA-DR allele DRB1*0101/DRB1*0401. T cell clones were established from this patient and characterized for peptide specificity, class II restriction, cytokine production, and staining with HLA-DRB1*0401 class II tetramers. RESULTS: A response to CII and the peptide 255-273 was present in this patient. T cells specific for the CII epitope 261-273 were cloned. Evaluation of these clones demonstrated a response to CII 261-273 in the context of both DR alleles. HLA-DR4 CII tetramer did not demonstrate staining of either CII-specific DRB1*0401-restricted T cell clones or a polyclonal population of CII-reactive T cells from this individual. CONCLUSION: T cells directed against CII were present in this patient with RP. Also, T cell clones isolated from this individual were found to be specific for the CII peptide 261-273 and were restricted to either the DRB1*0101 or the DRB1*0401 allele. These findings establish that a T cell response directed against CII is present in this patient with RP and that the CII peptide 261-273 plays a role in the human immune response to CII. (+info)