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Results for “Polychondritis, Relapsing”

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At least 19 recordsLinked to original sources
PubMed2001

Orbital mucosa-associated lymphoid tissue (MALT)-type lymphoma in a patient with relapsing polychondritis.

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.

Adult↗
PubMed2002

Multiple cardiovascular complications in a patient with relapsing polychondritis.

Relapsing polychondritis is an uncommon disease of unknown etiology, usually manifested by inflammatory changes of cartilaginous tissues. Cardiovascular complications are rare but have been associated with adverse prognosis. Aortitis, vasculitis of large- and medium-sized arteries with aneurysm formation, valvulitis, pericarditis, and atrioventricular conduction disturbances have been reported as late complications of relapsing polychondritis. We describe a 42-year-old man who developed all the known cardiovascular complications of relapsing polychondritis except for clinically evident pericarditis. This case illustrates the multiple, varied, and potentially fatal cardiovascular complications that can occur with this disorder. Patients with relapsing polychondritis should be monitored closely for development of such complications.

Adult↗
PubMed1998

Relapsing polychondritis.

Relapsing polychondritis, an uncommon, chronic, multisystem disorder characterized by recurrent episodes of inflammation of cartilaginous tissues, can be life-threatening, debilitating, and difficult to diagnose. This review is based on the authors' experience with 36 patients with relapsing polychondritis who were followed from 1980 to 1997, 30 patients located elsewhere who completed a detailed questionnaire and interview, and a perusal of English-language textbooks and papers located by a systematic search of the MEDLINE database. Relapsing polychondritis can present in a highly ambiguous fashion; therefore, in the authors' series, the mean delay from the time medical attention was sought because of symptom onset until diagnosis was 2.9 years. Although prednisone was the main form of treatment, methotrexate seemed to be of additional value. Survival was much more favorable than previously thought. Greater awareness of relapsing polychondritis would probably lead to earlier diagnosis and better outcomes.

Adrenal Cortex Hormones↗
PubMed2006

Multiple cardiovascular involvement in a case of relapsing polychondritis.

Relapsing polychondritis is a multisystem disease characterized by recurrent inflammation of the cartilaginous tissue. Cardiovascular manifestations of relapsing polychondritis are rare but are the second most common cause of death in these patients. We report a case of relapsing polychondritis who underwent aortic valve replacement uneventfully but presented six months later with myocardial infarction due to bilateral coronary ostial stenosis.

Adult↗
PubMed1990

Vasculitis and relapsing polychondritis.

Relapsing polychondritis is a systemic disease of unknown etiology with predominate manifestations of multiorgan cartilaginous inflammation. Although relapsing polychondritis occurs predominantly as a separately defined clinical complex, a significant number of patients may suffer from another rheumatic disease. Vasculitic syndromes are the most commonly observed disorders associated with relapsing polychondritis.

Humans↗
PubMed1993

Autoantibodies to cartilage collagens in relapsing polychondritis.

Relapsing polychondritis is a systemic disease associated with a destruction of cartilage in various parts of the body. Sera from six patients with relapsing polychondritis and one patient with microscopic polyarteritis nodosa as well as from six controls were analyzed by immunoblotting and ELISA. All patients had autoantibodies against native collagens II and IX. The serum from one patient showed a strong reaction with all three collagen chains of the high molecular weight fraction of collagen IX after denaturation; sera from four patients showed autoantibodies against alpha 2 (XI) and sera from three patients showed autoantibodies against the covalently cross-linked gamma component of collagen XI. The presence of autoantibodies against collagens II, IX, and XI, which form the major fibrillar scaffold in cartilage and mediate the interaction of collagen fibrils and proteoglycan, suggests that autoantibodies against cartilaginous collagen may play a crucial role in the pathogenesis of relapsing polychondritis and microscopic polyarteritis nodosa.

Adult↗
PubMed2002

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

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.

Animals↗
PubMedDate not supplied

[Relapsing polychondritis].

Relapsing polychondritis is a rare disease of unknown origin consisting in recurrent inflammatory episodes of cartilaginous structures as well as other organs such as the eye, the inner ear and the kidney. Symptoms of autoimmune or rheumatic disorders frequently precede those typical of relapsing polychondritis and thus the contemporaneous presence of these diseases is a common observation. Consequently some diagnostic concerns connected to the great variability of the early symptoms and also to the lack of a semiotic test do exist. It is widely recognized that a serious involvement of the tracheo-bronchial cartilage the lumen diameter with a consequent heavy dyspnoea which reduces occurs in relapsing polychondritis. In these cases the study of the thorax area by means of CT was of fundamental importance. Furthermore, histological analysis of bioptic specimens from tissues only apparently not affected, such as the pinna of the ears, better defines the nature of the disease. The Authors present a case report, characterized by the presence of a saddle-nose deformity as well as a dyspnoea caused by a lumen stenosis related to tracheo-bronchial cartilage chondritis.

Autoimmune Diseases↗
PubMed2000

Relapsing polychondritis.

Relapsing polychondritis is a rare inflammatory disease of unknown aetiology characterized by recurrent inflammation and destruction of cartilaginous structures and connective tissue. Current data provide increasing support for an autoimmune basis, but its cause remains unknown. Individuals of any race, gender, or age may be affected, but it is most commonly seen between the ages of 40 and 60 years. Although relapsing polychondritis occurs predominantly as a separately defined clinical complex, a significant number of patients may suffer from another underlying rheumatic and/or haematological disorder; vasculitic syndromes are the most commonly observed disorders associated with relapsing polychondritis. Common clinical features are auricular, nasal and respiratory tract chondritis with involvement of organs of special sense, such as the eyes and audiovestibular apparatus. Polyarthritis and vasculitic involvement are also common. Corticosteroids are still the agents of choice although several other anti-inflammatory drugs can be used in order to allow tapering of the steroid dose or to achieve a lower maintenance dose for refractory cases.

Adrenal Cortex Hormones↗
PubMed2003

Auricular chondritis in NOD.DQ8.Abetao (Ag7-/-) transgenic mice resembles human relapsing polychondritis.

Relapsing polychondritis is a multisystem autoimmune disease involving cartilage destruction but no known causative antigen. HLA-DQ8 has been associated with various autoimmune diseases in humans. To study the role of DQ8 in autoimmune diseases, we have generated transgenic mice expressing DQ8 (DQA1*0301, DQB1*0302) in a NOD background lacking endogenous class II molecules (Abetao). Upon immunization with type II collagen (CII), 85% of NOD.DQ8 mice develop severe experimental polychondritis, auricular chondritis, and polyarthritis, with clinical and histological similarities to relapsing polychondritis (RP) in humans. CII-immunized mice mount a T cell response and produce Ab's to type IX collagen (CIX) and self-CII. Transgene-negative littermates do not develop any serological and clinical manifestations following immunization. B10.DQ8 transgenic mice develop polyarthritis and Ab's to CII only. The susceptibility to auricular chondritis in NOD.DQ8 mice can be attributed to response to CIX. A higher number of activated cells, CD4+CD44(hi)CD62L(lo), and lower regulatory cells CD4+CD152+CD25+ were observed in NOD.DQ8 mice compared with B10.DQ8 mice. The NOD.DQ8 mice provide a model of RP with a high disease incidence and multiple organ involvement to investigate putative autoantigen and regulatory cells involved in disease pathogenesis. An experimental model restricted by the human class II molecule will be valuable when studying the role of various collagens in immunologic and pathologic responses in human RP.

Animals↗
PubMed1989

Classical rheumatoid arthritis associated with relapsing polychondritis.

Relapsing polychondritis is rare in rheumatoid arthritis (RA). We report a case of classical RA with relapsing polychondritis. Nasal septal chondritis resulted in cartilage collapse and a characteristic saddle nose deformity. HLA typing of the patient showed the presence of HLA-DR4 antigen.

Adult↗
PubMed1994

Use of metallic stents in relapsing polychondritis.

Relapsing polychondritis is a rare multisystem disease. We describe the presentation and treatment of a patient with relapsing polychondritis and review the literature. This patient had involvement of the tracheobronchial tree requiring insertion of metallic stents.

Aged↗
PubMed1999

When is a biopsy justified in a case of relapsing polychondritis?

Relapsing polychondritis (RP) is a relatively rare rheumatic condition of unknown aetiology. It is characterized by recurrent episodic inflammation of cartilaginous structures (nose, ear and trachea). The clinical diagnosis of polychondritis can frequently be made with confidence in the absence of histological confirmation. A 61-year-old diabetic man, with bilateral relapsing aural inflammation, left ear deafness with tinnitus and pain at the sternocostal junctions is reported. After clinical diagnosis of relapsing polychondritis steroid therapy was started. An ear cartilage biopsy was performed confirming the clinical diagnosis. Subsequently soft tissue infection occurred at the operation site. The abscess was drained and oral ciprofloxacin was given with complete resolution of the infection over 30 days. As the infection is the main cause of death in these patients, we analyse whether biopsy is absolutely necessary for the diagnosis of RP in some patients.

Biopsy↗
PubMed1978

Antibodies to type II collagen in relapsing polychondritis.

Relapsing polychondritis is a disorder of unknown cause characterized by the destruction of cartilage. To test the hypothesis that immunologic mechanisms are involved in the pathogenesis of relapsing polychondritis, we analyzed the serum of 15 patients for the presence of antibodies to cartilage. Antibodies to Type II (cartilage) collagen were found in the serum of five patients at the time of acute symptoms. No antibodies were detected either to cartilage proteoglycan or to other collagen types. The antibodies were detected at the onset of the disease and their titers appeared to correlate with severity of disease. Circulating immune complexes were also detected in the serum of these patients. Our findings support an immunologic involvement in this condition.

Acute Disease↗
PubMed1999

Anti-labyrinthine antibodies in a patient with relapsing polychondritis.

Relapsing polychondritis is a rare inflammatory disease that causes destruction of cartilaginous tissue in various anatomical regions. We report here about a 55-year-old female patient with relapsing polychondritis that involved the right auricle, both audiovestibular organs and both eyes. The patient presented with persisting inflammation of the right auricle, sudden lower-frequency hearing loss, acute moderate vertigo with nausea and mild ocular symptoms. Immunofluorescence assays were used for the detection of antibodies against the cochlea and the vestibular organ and demonstrated the presence of circulating antibodies against the audiovestibular organ. No staining for anti-corneal IgG was detected. Improvement of clinical disease was achieved by treatment with systemic steroids and vasodilator drugs, and long-term medication with low-dose corticosteroids.

Anti-Inflammatory Agents↗
PubMed1996

Steroid sparing effect of methotrexate in relapsing polychondritis.

Relapsing polychondritis is a rare inflammatory disorder causing recurrent inflammatory reactions in the cartilaginous structures. It often worsens as prednisone is tapered. We describe 3 biopsy proven patients with relapsing polychondritis in whom methotrexate was useful as a steroid sparing agent in the management of auricular chondritis.

Adult↗
PubMed1982

Pulmonary function in patients with relapsing polychondritis.

Relapsing polychondritis is a disease characterized by progressive inflammation of cartilagenous structures including those of the glottis, trachea, or central bronchi. We performed detailed physiologic and radiologic studies of the respiratory tract in five patients with respiratory involvement due to relapsing polychondritis. We found that the maximal expiratory and inspiratory flow-volume loop and airway resistance together can provide useful clues as to the presence, site, and fixed or dynamic nature of the upper airway obstruction in these patients. However, in patients with fixed upper airway obstruction or compound lesions, computer tomography of the respiratory tract, cinetracheography, or laryngotracheograms at different lung volumes were required to identify the site of the obstruction and to clarify the dynamic nature of the obstruction.

Adult↗