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PubMed2000

Relapsing polychondritis in childhood--case report and short review.

Relapsing polychondritis (RP) is a disease of unknown etiology and it is characterized by inflammation of the cartilage. While the clinical picture of RP in adults is well described, RP in childhood is poorly documented. We describe a young girl presenting with acute dyspnea, stridor and polyarthritis. The diagnosis of RP was made 2 years after first presentation, when auricular chondritis occurred. Based on a MEDLINE search, reports on RP in childhood were reviewed. The frequency of chondritis and systemic manifestations of RP in children was compared to data in adults and found to be very similar. RP in childhood can be a life-threatening and debilitating disease.

Adult↗
PubMedDate not supplied

Computed tomography of relapsing polychondritis.

The CT findings of a patient with relapsing polychondritis involving the larynx, tracheobronchial tree, and nasal cartilage are described. In the proper clinical setting, a characteristic constellation of findings when noted on CT can aid in differentiating this rare inflammatory disease from other causes of airway compromise. CT is the most useful imaging modality, because cartilage and soft tissue components are well visualized. If diagnosed early, appropriate treatment may prevent life-threatening airway obstruction.

Bronchial Diseases↗
PubMedDate not supplied

Relapsing polychondritis: a pediatric case.

A pediatric case of relapsing polychondritis is reported. Diagnostic features were recurrent episodes of painful inflammation of ear cartilage, episcleritis, and atopic disease. A good therapeutic response was obtained with systemic corticosteroids, dapsone, and methotrexate.

Child↗
PubMed1999

A new animal model for relapsing polychondritis, induced by cartilage matrix protein (matrilin-1).

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.

Animals↗
PubMed2003

A case of relapsing polychondritis associated with myelodysplastic syndrome with erythroid hypoplasia/aplasia.

Relapsing polychondritis (RP) is a rare multisystem disorder. Myelodysplastic syndrome (MDS) with erythroid hypoplasia/aplasia is a rare form of myelodysplasia. Several cases of RP associated with MDS have recently been described. However, RP associated with MDS with erythroid hypoplasia/aplasia has never been reported. There was only one case report of polymyalgia rheumatica associated with MDS with erythroid hypoplasia/aplasia. In this study, we report a 79-year-old patient with RP, who developed MDS subtype refractory anemia (RA) with erythroid hypoplasia/aplasia, a very characteristic subtype of MDS.

Aged↗
PubMed2004

Relapsing polychondritis: reversible airway obstruction is not always asthma.

Relapsing polychondritis (RP) is a rare disease characterized by recurrent inflammation of cartilaginous and other proteoglycan-rich tissues. Respiratory tract involvement is a common cause of morbidity and mortality in RP. We describe a patient whose clinical features at onset of disease were typical of asthma. Later, the patient developed symptoms and signs characteristic of RP. Tracheobronchomalacia necessitated airway support by stenting. The possibility that airway obstruction in the initial stages of RP is due to airway inflammation and that early, aggressive immunosuppressive treatment of RP may delay or prevent irreversible cartilaginous destruction and airway collapse are discussed.

Airway Obstruction↗
PubMed2000

[Clinical analysis of 5 cases of relapsing polychondritis].

OBJECTIVE: To describe the clinical manifestation of relapsing polychondritis(RP) and evaluate the medical therapy of RP, especially management of airway stenosis with intervening method. METHOD: Retrospective analysis of 5 cases of RP treated in our hospital since 1995 with reviewing of large series of cases in reported literatures. RESULT: RP is a rare multisystem disorder characterized by recurrent inflammation and progressive degeneration of cartilaginous structure and connective tissue. Medical treatment with corticosteroids, immunosuppressive drugs and dapsone could not interrupt the progression of the disease. Involvement of the respiratory tract is life-threatening and intervening therapy such as tracheostomy, endotracheal intubation or tracheobronchial stents is needed, one case of relieving severe dyspnea caused by extensive tracheobronchial collapse in RP with self-expanding metallic stents was presented. CONCLUSION: It is important to make the diagnosis of RP according to the clinical features and carry out medial treatment at early stage, positive airway pressure support and intervening methods to prevent the airway collapse should be considered when the laryngotracheobronchial involvement aggreviates.

Adrenal Cortex Hormones↗
PubMed2002

[Anesthesia in a patient with relapsing polychondritis].

A 46-year-old female with relapsing polychondritis (RP) was scheduled for surgical repair of saddle-nose and peri-chondritic ear. RP is a rare systemic, inflammatory, and destructive disease of the cartilaginous structures leading to multiple functional disorders in the affected organs. Preoperatively, her chest radiogram of the trachea showed narrowing in diameters which altered about 2 mm with expiration and inspiration. Propofol administered intravenously was given as induction and anesthesia was maintained with nitrous oxide-oxygen-sevoflurane with spontaneous ventilation using laryngeal mask airway. The operation was over uneventfully. Anesthesiologic management requires careful preoperative evaluation of vital organ functions, in particular, respiration.

Anesthesia, Inhalation↗
PubMedDate not supplied

[Relapsing polychondritis: apropos a case with significant tracheal involvement].

Relapsing polychondritis is an infrequently recognized disease. Its etiology is unknown. It is characterized by recurrent and potentially destructive inflammatory lesions involving cartilaginous structures, the cardiovascular system and some sensory organs. We report a patient with important tracheal stenosis, vestibulo cochlear dysfunction and polyarthritis. We present a literature review.

Adult↗
PubMed1991

[Relapsing polychondritis. A case report with particular cardiac involvement].

Relapsing polychondritis is a rare systemic disease, characterized by recurrent inflammation of articular cartilaginous tissues, with frequent involvement of other organs and systems. While the cause remains unknown, an autoimmune pathogenesis appears likely; the natural history is not completely defined. The authors describe an interesting case with cardiovascular involvement and a good response to cyclosporin A.

Adult↗
PubMed1987

[Immunology and deafness. Manifestations of relapsing polychondritis in the ENT area. A case report].

Relapsing polychondritis is a rare episodic systemic disorder characterised by recurrent, widespread, and potentially destructive inflammatory lesions involving cartilaginous structures, the cardiovascular system, and organs of special sense, such as the eyes and ears. Although the aetiology is unknown and its pathogenesis is incompletely understood, experimental evidence strongly suggests that immunological mediated mechanisms are operative. The following case report adds some considerations to the discussion about the immunopathology of the inner ear.

Deafness↗
PubMed1979

Relapsing polychondritis associated with cutaneous vasculitis.

A patient with relapsing polychondritis associated with cutaneous vasculitis is described in detail. Clinically, a systemic chondritis complicated by conjunctivitis, iritis, otitis media, tracheal stenosis, sinusitis, and cutaneous vasculitis were observed in this case. Corticosteroids have been effective in controlling the clinical symptoms as well as the vasculitis.

Female↗
PubMed1993

Preferential cellular and humoral immune reactivities to native and denatured collagen types IX and XI in a patient with fatal relapsing polychondritis.

We describe a patient with histologically confirmed relapsing polychondritis, an episodic systemic disorder. Although the etiology is unknown and its pathogenesis is incompletely understood, there is evidence strongly suggesting immunologically mediated mechanisms. Enzyme linked immunosorbent assays, immunoblotting and cellular immune responses using lymphocyte proliferation assays showed strong parallel humoral and cellular immune reactivities against collagens type IX and XI. There was also a considerable response to collagen type II which, however, was less pronounced compared to collagen type IX and was directed to native epitopes. Our findings demonstrate a highly distinct immune response to minor matrix collagens in a destructive cartilage disease and thus strongly argue against nonspecific anticollagen immune reactions simply representing epiphenomena resulting from cartilage damage.

Aged↗
PubMed1998

[A case of relapsing polychondritis with IgA nephropathy].

We report a case of a 37-year-old man with relapsing polychondritis and IgA nephropathy. He visited our hospital with high fever and the swelling of his ears and eyelids. His symptoms and the results of the biopsy of his right auricle fulfilled the Damiani's criteria. Laboratory examination on admission showed an increase of serum IgA level, a presence of immune complex, remarkable hematuria (grade III) and proteinuria (grade II). Most of his symptoms were improved by the administration of antibiotic and NSAIDs, however, urinary findings still remained unchanged. The biopsy of his right kidney led to a diagnosis of IgA nephropathy (group II). Although relapsing polychondritis is known to associate rarely with renal involvement, it is very rare to associate with IgA nephropathy. This case indicates that immune disorders including IgA nephropathy should be investigated in patients with relapsing polychondritis.

Adult↗
PubMed2005

Anesthetic management of the parturient with relapsing polychondritis.

PURPOSE: To present the anesthetic management of a parturient with relapsing polychondritis (RP) and to discuss the anesthetic implications of RP. CLINICAL FEATURES: A 28-yr-old primiparous woman with known RP, spondyloarthropathy and fibromyalgia presented for urgent Cesarean delivery for breech presentation and prodromal labour. Her pregnancy had been complicated by a hospital admission for an exacerbation of her RP as manifested by hoarseness, increased pain and tenderness of her left ear and nasal bridge cartilages, sinusitis with bloody nasal discharge and increased pain and tenderness of the anterior tracheal rings. Epidural anesthesia was administered for the Cesarean delivery. Her intraoperative and postoperative course was uneventful. Close cooperation among obstetricians, anesthesiologists and rheumatologists resulted in a successful outcome. CONCLUSION: Relapsing polychondritis is a syndrome with important anesthetic implications. Multidisciplinary cooperation is essential in managing these high risk parturients.

Adult↗
PubMed1986

Pulmonary function in relapsing polychondritis.

The pulmonary mechanics and bronchoscopic findings in 5 patients with relapsing polychondritis were studied to evaluate the mechanism of obstruction. Two of the patients did not have clinical symptoms referable to the respiratory tract: pulmonary function was normal in 1 patient and was suggestive of mild restriction in the other. Three patients had dyspnea; pulmonary function studies revealed expiratory and inspiratory obstruction in all 3. The maximal flow-static recoil curves demonstrated that the expiratory obstruction was due predominantly to airway abnormality and not to loss of elastic recoil forces of the lung. The bronchoscopic appearance of the extrathoracic airway during quiet breathing did not accurately reflect pulmonary function results. One patient had a visually normal upper airway but greatly reduced maximal inspiratory flow rates, whereas another patient had pronounced narrowing of the upper airway but only a modest reduction of maximal inspiratory flow rates. The bronchoscopic appearance of the intrathoracic airway during quiet breathing did relate well to maximal expiratory flow rates. We conclude that in our group of patients the predominant mechanism of expiratory obstruction in relapsing polychondritis is due to airway abnormality. Although the bronchoscopic and radiographic findings are useful, spirometry is more important in determining functional abnormality.

Adrenal Cortex Hormones↗
PubMed2006

[A case of aseptic meningitis caused by relapsing polychondritis].

We report a patient of relapsing polychondritis (RP) with antecedent aseptic meningitis. A 65-year-old man has developed headache and fever. Neurological examination showed meningeal signs, and cerebrospinal fluid (CSF) examination revealed meningeal inflammation which contained 450 polymorphonuclear cells/microl, 302 mononuclear cells/microl, and 0 red cells/microl, with 79 mg protein/dl. Serologic testing for autoimmune disease as well as the culture and cytology of CSF were negative. He admitted our hospital as having aseptic meningitis and experienced antibiotic therapy. However, his pyrexia continued and he developed repeating visual and hearing impairment reacting to steroid. Three months later, he became behaviorally deaf, and bilateral auricular chondritis occurred with nonerosive seronegative inflammatory polyarthritis. The result of condral biopsy was consistent with the diagnosis of RP showing cartilage surrounded by an intense inflammatory cell response with a decreased number of chondrocytes. A clinical diagnosis was made and prednisolone 60 mg/day was begun with the result of resolution of the auricular chondritis, and slight improvement of his deafness. Aseptic meningitis is a rare complication of RP. Only one report detailed RP patient who had preceding meningitis. RP is a potentially lethal disease resulting from suffocation by airway collapse, the complications of a cardiac large vessel, and so on. For improvement of a life prognosis, an early diagnosis and treatment are indispensable. Although RP is a rare discovery, it is necessary that RP should be taken into consideration and be differentiated as a cause of relapsing aseptic meningitis.

Aged↗