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PubMed2003

Relapsing polychondritis.

Relapsing polychondritis (RP) is a rare, inflammatory disease with multisystem involvement and it should be considered in the aetiology of sudden respiratory distress. A 49-year-old woman was admitted to the emergency service of Osmangazi Hospital in acute respiratory distress. She had a tracheostomy following a diagnosis of laryngeal stenosis 10 years earlier. She was managed thereafter at another hospital with a diagnosis of asthma. At admission she was in respiratory distress, had arthralgias, bilateral subconjunctival hyperaemia, periorbital oedema and skin lesions. A thoracic CT showed tracheal wall thickening. Calcification of the auricular regions were noted bilaterally. Bronchi, conjunctiva and skin lesions were biopsied with a provisional diagnosis of RP. The diagnosis of RP in this patient was based on clinical, pathological and radiological findings. Steroid therapy was begun and she was referred to a surgical centre for stenting. This case report emphasizes the need to consider the possibility of RP as a cause of sudden respiratory distress.

Female↗
PubMed2006

Endolymphatic hydrops as a cause of audio-vestibular manifestations in relapsing polychondritis.

Relapsing polychondritis (RP) is characterized by inflammation and subsequent degeneration of cartilage. We report a 61-year-old woman who had RP with audio-vestibular manifestations. She was also diagnosed as having a myelofibrosis with myeloid metaplasia (MMM). Bilateral endolymphatic hydrops (EH) was confirmed by dominant -SP/AP of the electrocochleogram (ECochG). When thalidomide and prednisolone were prescribed for the treatment of MMM, symptoms of RP -- including the inner ear dysfunction -- were ameliorated. Isosorbide, one of the osmotic diuretics commonly used for the treatment of Meniere's disease (MD) in Japan, was also effective in keeping her free from inner ear dysfunction. This is the first report to confirm the existence of EH in a patient with RP with audio-vestibular manifestations. We suppose that an immunological imbalance due to MMM, in conjunction with a specific immunogenetic background, may have played a role in the pathogenesis of RP and the formation of EH in this patient.

Acute Disease↗
PubMed2006

Proteomic surveillance of autoantigens in relapsing polychondritis.

Relapsing polychondritis (RP) is a systemic inflammatory disease, in which autoimmunity to cartilage-related components is thought to be involved in its pathogenesis. However, the autoimmune profile in RP has not been studied fully. We therefore investigated autoantibodies/autoantigens in RP comprehensively, by 2-dimensional electrophoresis (2DE), subsequent western blotting (WB) and mass spectrometry, using cell-extracted proteins as the antigen source. As a result, we detected 15 autoantigens on 2DE-WB, and further identified five of them. On average, one RP serum recognized approximately 8 out of the 15 autoantigens. Frequencies of the autoantibodies to the 5 identified antigens of tubulin alpha ubiquitous/6, vimentin, alpha enolase, calreticulin, and colligin-1/-2 were 91%, 46%, 36%, 82%, and 36%, respectively. ELISA using recombinant proteins for them revealed that frequencies of the autoantibodies to tubulin alpha ubiquitous, vimentin, alpha enolase, calreticulin, and colligin-1 were 36%, 64%, 46%, 27%, and 18%, respectively. Our data demonstrated that the autoimmune reaction was not restricted to cartilagerelated components, rather a variety of autoimmune responses occurred in patients with RP, which may be involved in the pathophysiology of RP. In addition, the proteomic approach using cell-extracted proteins would be a powerful way to investigate autoantigens.

Autoantibodies↗
PubMed2001

Active aortitis in relapsing polychondritis.

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.

Aorta↗
PubMed1978

Narrowing of the airway in relapsing polychondritis.

Relapsing polychondritis, once thought to be a very rare disease, is being recognized with increasing frequency. It is characterized by inflammation of cartilaginous structures throughout the body. In some cases, the eye and ear are involved; aortic aneurysms develop in a minority of patients. The cartilages of the airway are affected in more than 50% of all cases, and the resulting stenotic lesions can be life-threatening. Radiographic study of the airway is of great value in detection and evaluation of upper respiratory involvement.

Adolescent↗
PubMed2000

Tracheobronchial involvement in relapsing polychondritis.

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.

Airway Obstruction↗
PubMed1998

Anti-phospholipid-antibodies in patients with relapsing polychondritis.

Relapsing polychondritis (RP) is an extremely rare multisystemic disease thought to be of autoimmune origin. In order to assess if RP is associated with anti-phospholipid antibodies (aPL), clinical data and sera of 21 patients with RP were collected in a multicentre study. Concentration of anti-cardiolipin antibodies (aCL) (IgG-, IgM- and IgA-isotypes), anti-phosphatidylserine-antibodies (aPS) (IgG- and IgM-isotypes) and anti-beta-2-glycoprotein I-antibodies (a beta 2 GPI) were measured by ELISA. In eight patients aCL were found to be elevated. One patient had elevated aPS. No patient had elevated a beta 2 GPI. No patient had clinical signs and symptoms of a aPL syndrome. Interestingly, the two RP patients with the highest aPL had concomitant systemic lupus erythematosus (SLE). Therefore the presence of elevated aPL in RP is probably more closely related to an associated SLE than to RP itself. There is no convincing evidence that aPL are associated with RP.

Adolescent↗
PubMed1988

IgA nephropathy in relapsing polychondritis.

Relapsing polychondritis (RP) is a multisystem autoimmune disease characterized by the presence of antibodies to type II collagen. This collagen is found predominantly in cartilaginous tissues, vitreous humor, aorta and notochord. Involvement of the kidney is rare, only 7 cases having been recorded, and there is no type II collagen in glomeruli. Six of the previous cases had crescentic glomerulonephritis. We report here two cases of biopsy proven RP in which IgA nephropathy was seen, the first examples recorded. Both patients had hematuria and slight proteinuria, with mild impairment of renal function. The histological and immunofluorescence pattern on both biopsies was in keeping with IgA nephropathy. Both patients received steroids with diminution/disappearance of hematuria and proteinuria. In view of the potentially progressive nature of glomerular disease with RP, the renal status should be investigated in all patients with RP.

Adult↗
PubMed1992

Relapsing polychondritis.

Relapsing polychondritis is a rare disease of unknown etiology. It is characterized by progressive episodic recurring inflammation and degeneration of cartilaginous structures. The inflammation typically involves the cartilage of the ear, nose, trachea, and various peripheral joints. Treatment options most commonly involve the use of anti-inflammatory drugs and corticosteroids.

Biopsy↗
PubMed1989

[Atypical relapsing polychondritis].

Relapsing polychondritis is a disease of unknown etiology whose main characteristic is the chronic inflammation and destruction of the different cartilaginous structures of the body. A rare case is presented, the rareness being the fact that ear cartilage was not affected and that the destructive arthropathy was similar to that produced by rheumatoid arthritis.

Arthritis↗
PubMed1997

ENT manifestations of relapsing polychondritis.

Relapsing polychondritis is a rare disease probably of auto-immune etiology comprising inflammatory involvement of cartilage as well as phenomena of vasculitis. ENT manifestations are frequent and the authors present a case involving chondritis of the auricle, the nasal septum and affecting the cochleo-vestibular system as well. The diagnosis is based on the evocative clinical picture and on histological confirmation. Treatment consists of corticosteroids and immunosuppressive agents.

Anti-Inflammatory Agents↗
PubMed2001

[Asthma as presentation form of relapsing polychondritis].

Relapsing polychondritis is a systemic disease of unknown etiology characterised by relapsing inflammation affecting cartilaginous structures, cardiovascular system, eyes and ears. Respiratory involvement occurs in 56% of patients during the disease progression, but only in 14% of cases as an initial presentation. Patients develop severe symptoms due to the disease affecting the glottis and the tracheobronchial tree, which represents the cause of death in 50% of cases. The unspecificity of respiratory symptoms makes that the disease may be confounded with some other if it is not accompanied with a typical presentation, which may cause a delay in the diagnosis. Pulmonary function tests are of great importance, since an obstructive pattern not reversible after bronchodilator administration and a plateau in flow-volume curves are of great help when assessing the severity of the obstruction. High resolution computed tomography is a non-invasive test more precise than bronchoscopy in identifying tracheal and bronchi abnormalities, so it should be performed at the onset of the respiratory symptoms together with the pulmonary function tests. We present the case of a patient, whose disease started with respiratory semiology suggesting bronchial asthma, which preceded in six months the main sign nasal chondritis.

Adult↗
PubMed2002

Relapsing polychondritis.

Relapsing polychondritis (RP) is a rare disease causing inflammation and destruction of cartilage and other connective tissues. Specific laboratory aberrations are lacking. Predominant clinical manifestations include auricular chondritis, polyarthritis, nasal chondritis, ocular inflammation, audiovestibular damage, and respiratory tract chondritis. A relapsing course is characteristic. Airways are involved in 50% of patients and may cause dyspnea, stridor, wheezing, hoarseness, aphonia, and laryneal or tracheal tenderness. Airflow obstruction may result from RP involving the tracheobronchial tree; there is no interstitial or pulmonary vascular component. Collapse or failure of the trachea to dilate during inspiration is a key feature. Fast computed tomographic (CT) scanners can visualize dynamic airway collapse. Randomized, controlled trials of therapy have not been done. Corticosteroids and nonsteroidal anti-inflammatory drugs (NSAIDs) are used most commonly, but optimal regimens and duration of therapy have not been elucidated. Endobronchial stents or tracheostomy may be required for severe stenoses refractory to medical therapy.

Journal Article↗
PubMedDate not supplied

[Erythema annulare centrifugum and relapsing polychondritis].

BACKGROUND: Relapsing polychondritis is a rare systemic disease. Skin involvement occurs in 20 to 50 % of cases. Cutaneous signs are most often related to a leukocytoclastic vasculitis. Association of relapsing polychondritis with neutrophilic dermatosis have also been reported. We report the first case of an erythema annulare centrifugum-like dermatosis associated with relapsing polychondritis, with a two years delay between both conditions. CASE REPORT: A 74 year-old man was seen for papulo-erythematous centrifugal annular lesions that appeared 18 months earlier in a context of bad general conditions. Biological tests were normal. Several skin biopsies were performed, showing at the beginning features of drug reaction and then of lupus-lichen. Treatment with hydroxychloroquine, topical corticosteroids, dapsone and thalidomide were unsuccessful. In the following months, the patient developed fever and relapsing bronchitis. Suddenly, a chondritis of the ears appeared, leading to the diagnosis of relapsing polychondritis. All the cutaneous, chondritic and respiratory signs disappeared with oral steroid therapy. Two years after the diagnosis of relapsing polychondritis the patient developed refractory anemia. DISCUSSION: Cutaneous signs of relapsing polychondritis are frequent and may occur several months or years before the chondritis. They are polymorphous, but to the best of our knowledge, a clinical aspect of erythema annulare centrifugum has never been described. Our observation recalls the sometimes long delay between the cutaneous and the chondritic signs of relapsing polychondritis and the high frequency of dysmyelopoiesis in relapsing polychondritis with cutaneous involvement.

Aged↗
PubMed2002

[Cutaneous vasculitis, myelodysplasia and relapsing polychondritis].

BACKGROUND: Relapsing polychondritis is a rare autoimmune disease, characterized by recurrent inflammation of cartilaginous tissues. In some cases, many other tissues can be involved. CASE REPORT: We describe the case of a 64 year-old man with relapsing polychondritis, whose first symptoms were papulonodular and mucosal aphthous lesions. The skin biopsy revealed vasculitis without leukocytoclasic features. The diagnostic of relapsing polychondritis was made only two years later, thanks to recurrent auricular chondritis. Since the beginning, the disease was associated with a myelodysplasia. DISCUSSION: Dermatological manifestations are noticed in less than 50 p. 100 of cases during the evolution of relapsing polychondritis. An association with a myelodysplasia has already been reported, and it could be a particular form of relapsing polychondritis. Myelodysplasic syndromes are more frequently associated with relapsing polychondritis than with other vasculitis. It must be sought especially when cutaneous and mucosal manifestations exist.

Humans↗
PubMed1999

A new role for magnetic resonance imaging in the diagnosis of laryngeal relapsing polychondritis.

BACKGROUND: Relapsing polychondritis involving the upper airway is a rare cause of airflow obstruction and hoarseness. The diagnosis of relapsing polychondritis depends on clinical signs, characteristic findings on cartilage biopsy, and response to treatment. Delays in diagnosis and treatment can increase the morbidity and mortality of the disease. METHODS: We present a case report of primary laryngeal relapsing polychondritis. RESULTS: Serologic testing, direct laryngoscopy, and endoscopic biopsy could not establish the diagnosis of relapsing polychondritis. Magnetic resonance imaging (MRI) examination demonstrated findings consistent with the histopathological diagnosis obtained on open biopsy. Follow-up MRI after treatment showed resolution of the initial findings. CONCLUSION: This case demonstrates the usefulness of MRI in the diagnosis and management of relapsing polychondritis involving the upper airway.

Female↗