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Relapsing polychondritis with multifocal neurological abnormalities.

A sixty-five year old woman developed relapsing polychondritis with three of the diagnostic criteria established by McAdam et al. (1976), namely bilateral auricular chondritis, ocular inflammation and both cochlear and vestibular dysfunction. Many authors have mentioned other neurological symptoms including unilateral facial weakness. This patient is the first case described with bilateral facial weakness and cerebral manifestations. Relapsing polychondritis, an uncommon, recurrent, inflammatory disorder affecting the cartilaginous tissues of the body (Jaksch-Wartenhorst, 1923; Herman, 1981), is regularly associated with audiovestibular dysfunction (Bollet et al., 1969; Cody et al., 1971; McAdam et al., 1976; Ridgway et al., 1979). McAdam et al. (1976) and Ridgway et al. (1979) have mentioned other neurological symptoms including unilateral facial weakness. We recently observed a patient with polychondritis and multifocal neurological abnormalities. We believe this is the first case described with bilateral facial nerve palsy and with cerebral manifestations.

Diabetes Mellitus, Type 2↗

Anaesthetic management of a patient with relapsing polychondritis undergoing laparoscopic surgery.

We describe the anaesthetic management of a patient with relapsing polychondritis who underwent laparoscopic cholecystectomy. We failed to secure a patent airway with a ProSeal laryngeal mask airway, probably because of the deformity of the larynx. The glottis was small and it was only possible to pass a 5.5 mm cuffed endotracheal tube into the trachea. Positive pressure ventilation with 5 cm H2O positive end-expiratory pressure and surgery were safely performed. In relapsing polychondritis, recurrent inflammation and destruction of laryngeal and tracheobronchial cartilage causes airway obstruction, and various sizes of tracheal tubes and other airway manipulation devices should be prepared.

Adult↗

[Airway manifestations of relapsing polychondritis: treatment with cyclophosphamide and placement of bronchial stents].

We report the case of a 41-year-old female patient with relapsing polychondritis and severe respiratory involvement. The patient presented with acute respiratory failure requiring endotracheal intubation. Bronchoscopy revealed tracheal collapse and inflammatory stenoses with dynamic collapse of the major airways. We describe a multidisciplinary therapeutic approach, consisting of immunosuppressive treatment, bronchoscopic placement of self-expandable stents into the collapsing bronchi, and tracheotomy. In addition, we report the effectiveness of oral cyclophosphamide for treatment of relapsing polychondritis with severe respiratory involvement after failure of other immunosuppressive agents. The problem of severe respiratory complications in patients with relapsing polychondritis and the need for a multidisciplinary approach is discussed.

Adult↗

Relapsing polychondritis with involvement of posterior inferior cerebellar artery causing acute lateral medullary syndrome.

We present a patient with relapsing polychondritis who presented with findings of lateral medullary syndrome, known as the syndrome of Wallenberg, produced by infarction of a wedge of lateral medulla lying posterior to the inferior olivary nucleus. Angiographic examination showed complete occlusion of the right posterior inferior cerebellar artery and segmental narrowings in the basilar artery. We believe that the angiographic findings were based on an underlying vasculitis because of his relapsing polychondritis. This is the first case of relapsing polychondritis with angiographically demonstrated posterior inferior cerebellar artery involvement. Although this syndrome is usually a result of atherosclerosis, our case suggests that vasculitis must also be considered because it may respond to treatment with corticosteroids.

Journal Article↗

Relapsing polychondritis: clinical and immunogenetic analysis of 62 patients.

OBJECTIVE: In this study we describe clinical and immunogenetic findings in 62 unselected patients with relapsing polychondritis. METHODS: In a multicenter study, clinical data of 26 (41.9%) female and 36 (58.1%) male patients were collected. HLA-DR specificities were identified in 60, and the frequencies were compared with those in healthy controls. RESULTS: The median age at the time of diagnosis was 46.6 years (range 17 to 86). 58 (93.5%) patients had auricular chondritis, 31 (50.0%) ocular symptoms, 35 (56.5%) nasal involvement. Involvement of joints (53.2%), respiratory system (30.6%), skin (24.2%), cardiovascular system (22.6%), central nervous system (9.7%), and kidneys (6.5%) was found as well. 22 (35.5%) patients had associated diseases such as systemic lupus erythematosus or rheumatoid arthritis. Susceptibility to relapsing polychondritis was significantly associated with HLA-DR4 (p < 0.001). There was no difference in the frequency or distribution of DRB1*04 subtype alleles between patients and healthy controls. The extent of organ involvement was negatively associated with HLA-DR6 (p < 0.011). CONCLUSION: Immunogenetic findings as well as similarities and overlapping clinical symptoms with other autoimmune or rheumatic diseases suggest that immunological mechanisms play a major role in the pathogenesis of relapsing polychondritis.

Adolescent↗

[Laryngotracheobronchial involvement in relapsing polychondritis].

OBJECTIVE: To investigate the clinical cause, treatments and prognosis of laryngotracheobronchial involvement in relapsing polychondritis (RP). METHODS: Thirteen patients of RP with laryngotracheobronchial involvement were review retrospectively. RESULTS: From 1983 to 1998, 20 patients with RP were managed in this hospital. Of them, 13 (65%) involving the laryngotracheobronchial region were observed. Respiratory tract lesions might be diffuse or localized and occurred in larynx (46%), trachea (69%) and bronchus (69%). Corticosteroids, immunosuppressive agents and dapsone were used in these patients. Tracheotomy was performed in 4 patients with airway obstruction. Montgomery T-tube or self-expanding metal stent were used in 2 patients with tracheal collapse. Of 13 case, 2 died from respiratory complications. The 5-years survival rate was 78.6%. CONCLUSION: Respiratory tract involvement is the most severe manifestation of the relapsing polychondritis and the main cause of death despite aggressive medical therapy or tracheotomy.

Adrenal Cortex Hormones↗

Chondrosarcoma in a patient with relapsing polychondritis.

A case of a chondrosarcoma in a patient with relapsing polychondritis was presented. The association of these two unusual disorders of cartilaginous tissue has never been described before. The clinical presentation and radiologic and histologic features were discussed.

Bone Neoplasms↗

Otologic manifestations of relapsing polychondritis. Review of literature and report of nine cases.

OBJECTIVE: Relapsing polychondritis (RP) is an episodic disease most likely of autoimmune etiology, characterized by recurrent inflammation of cartilaginous structures. METHODS: Retrospective case study at two tertiary referral centers with presentation of nine patients with otologic involvement of RP, review of the spectrum of otologic disorders seen, and treatment. RESULTS: The clinical course of otologic manifestations of RP was highly variable and ranged from mild to moderate. In 6/9 patients there was an association with other autoimmune disorders. In addition to recurrent auricular chondritis, which was present in 8/9 patients, our patients had otitis externa, chronic myringitis, Eustachian tube dysfunction, conductive hearing loss, sensorineural hearing loss, and tinnitus. All patients had their diagnosis of RP made on the basis of their otologic involvement and the response to systemic corticosteroids. CONCLUSION: The diagnosis of RP is primarily clinical, but laboratory studies and biopsy may contribute as well. Once the diagnosis is suspected, the otolaryngologist should consider consultation with a rheumatologist to assist in the management of additional systemic manifestations.

Adult↗

Relapsing polychondritis--a study of four cases.

Four case reports of relapsing polychondritis, (RP), are presented, together with a literature review and management suggestions. There are approximately 211 reported cases in world literature making RP an uncommon condition associated with high morbidity and mortality rates. The key to the management of RP is based on accurate and early diagnosis though the ideal medical regimen has yet to be elucidated.

Aged↗

Relapsing polychondritis and Weber-Christian disease.

A patient with classical relapsing polychondritis in whom abdominal pain was a prominent symptom is described. Histological examination of skin and mesenteric biopsies confirmed the diagnosis of Weber-Christian disease (systemic panniculitis) suggested by clinical features. The prognostic and therapeutic implications of this unusual disease association are discussed.

Adult↗

Immunopathologic studies in relapsing polychondritis.

Serial studies have been performed on three patients with relapsing polychondritis in an attempt to define a potential immunopathologic role for degradation constituents of cartilage in the causation and/or perpetuation of the inflammation observed. Crude proteoglycan preparations derived by disruptive and differential centrifugation techniques from human costal cartilage, intact chondrocytes grown as monolayers, their homogenates and products of synthesis provided antigenic material for investigation. Circulating antibody to such antigens could not be detected by immunodiffusion, hemagglutination, immunofluorescence or complement mediated chondrocyte cytotoxicity as assessed by (51)Cr release. Similarly, radiolabeled incorporation studies attempting to detect de novo synthesis of such antibody by circulating peripheral blood lymphocytes as assessed by radioimmunodiffusion, immune absorption to neuraminidase treated and untreated chondrocytes and immune coprecipitation were negative. Delayed hypersensitivity to cartilage constituents was studied by peripheral lymphocyte transformation employing [(3)H]thymidine incorporation and the release of macrophage aggregation factor. Positive results were obtained which correlated with periods of overt disease activity. Similar results were observed in patients with classical rheumatoid arthritis manifesting destructive articular changes. This study suggests that cartilage antigenic components may facilitate perpetuation of cartilage inflammation by cellular immune mechanisms.

Adult↗

Severe endobronchial obstruction in a girl with relapsing polychondritis: treatment with Nd YAG laser and endobronchial silicon stent.

Relapsing polychondritis (RP) is an uncommon disorder of unknown aetiology characterized by inflammation and destruction of the cartilaginous structures of many organs, including the tracheobronchial tree. When untreated, there is a high mortality rate, usually from respiratory obstructive complications. An 8 year old white girl, with a previous diagnosis of RP, was referred to our department for evaluation of worsening dyspnoea. Bronchoscopy showed localized inflammatory and fibrotic alterations of the mucosa, leading to severe obstruction of the left mainstem bronchus at its origin. The condition was successfully treated by endoscopic neodymium yttrium aluminium garnet (Nd YAG) laser. Re-evaluation of the patient, 7 months later, demonstrated bronchial stenosis and malacia requiring mechanical dilatation and positioning of an endobronchial silicon stent, which was well-tolerated by the patient.

Airway Obstruction↗

[Relapsing polychondritis revealed by ENT symptoms: clinical characteristics in three patients].

Relapsing polychondritis (RP) is a recurrent, chronic and rare disease of unknown etiology, considered as a systemic vasculitis. RP is characterized by inflammation of cartilaginous structures of the ears, nose, respiratory tract and joints. RP is likely initiated by ENT symptoms. Etiology is unknown but the association with HLA-DR4 and the occurrence of antibodies to type-II collagen suggest that an immunologic mechanism is involved in its pathogenesis. Diagnosis is difficult requiring identification of elastic cartilaginous injuries. Delay before diagnosis is usually important after the first attack. Neither serum investigation nor histological confirmation are necessary to establish the RP diagnosis, and ENT symptoms are generally sufficient to achieve the diagnosis. Prognosis is linked to laryngeal, tracheal and cardiovascular involvements. An association with myelodysplasia is acknowledged. Based on these three cases and data in the literature, we review classical diagnostic criteria (McAdam), prognosis and therapeutic outcome.

Adrenal Cortex Hormones↗

[Characteristics of airway involvement in relapsing polychondritis].

OBJECTIVE: To investigate the characteristics of airway involvement in relapsing polychondritis (RP). METHODS: The clinical data, including clinical manifestations, respiratory function test, computerized tomography (CT), and bronchoscopy of 38 out of the 56 RP patients who had airway involvement, 20 males and 18 females, with the mean onset age of 45 +/- 11 (27 - 71), and 3 RP patients without airway involvement were retrospectively analyzed. Three patients out of the 16 RP patients who did not have respiratory involvement but underwent respiratory function test, CT, and bronchoscopy were used as controls. RESULTS: The symptoms of airway involvement included cough, expectoration, hoarseness, feeling of suffocation, asthma, and dyspnea. Obstructive disturbance of ventilation was found in 10 and mixed disturbance of ventilation was seen in 2 of the 18 RP patients with airway involvement. The forced expiratory volume in one second, ratio of forced expiratory volume in one second to forced vital capacity, peak expiratory flow, FEF50/FIF50, and maximal mild-expiratory flow of the patients with airway involvement were 1.4 L +/- 0.23 L, 46.0 +/- 4.86, 3.3 L/s +/- 0.67 L/s, 0.3 +/- 0.08, and 0.4 L/s +/- 0.18 L/s respectively, all significantly lower than those of the RP patients without airway involvement (2.5 L +/- 0.09 L, 83.7 +/- 2.24, 6.9 L/s +/- 0.52 L/s, 1.3 +/- 0.51, and 2.8 L/s +/- 0.73 L/s, all P = 0.01). Flow volume loop showed remarkable decrease of PEF and formation of a plateau in the expiratory phase in the RP patients with airway involvement. CT performed in 27 RP patients with airway involvement showed trachea stenosis in 11, and thickened airway wall in 8 of them. Bronchoscopy performed in 23 patients with airway involvement showed inflammation in 16, destruction of tracheobronchial cartilage in 6, collapsed tracheobronchial wall in 7, tracheal stenosis in 15, left major bronchial stenosis in 13, and right major bronchial stenosis in 12 of them. CONCLUSION: Respiratory function test is sensitive in early detection of airway involvement in RP. Bronchoscopy and CT are useful in evaluation of the severity of airway involvement in patients with RP.

Adult↗

Acute airway obstruction in relapsing polychondritis: treatment with pulse methylprednisolone.

We describe 2 adolescents with relapsing polychondritis who developed acute airway obstruction. Both were successfully treated with intravenous steroids for this complication following failure with oral steroids. Early respiratory tract involvement in younger patients seems predictive of a poor outcome and aggressive therapy with intravenous high dose steroids and/or immunosuppressive drugs appears to be indicated.

Acute Disease↗

[Relapsing polychondritis. A case report with special reference to otolaryngological manifestations].

Relapsing polychondritis is a rare disease of unknown etiology. The symptomatology is characterised by inflammatory and degenerative changes of multiple cartilaginous structures. --In this peculiar case the nose, the larynx and the cartilages of the ribs and of one hand were involved. Distinct inflammatory changes of the left vocal cord were seen. The diagnosis of the disease was assured by the presented electron microscopic studies. Treatment with high doses of corticosteroids did not prove effective. The disease process could only be controlled by in immunosuppressive combination therapy with prednisone, and cyclophosphamide. --Characteristic clinical features, diagnosis, pathogenesis and therapeutical problems are discussed.

Adult↗

[Relapsing polychondritis: report of a case and review of the literature].

Relapsing polychondritis (RP) is a rare disease of unknown etiology, characterized by episodic and progressive chondritis, ocular and audiovestibular involvement, and occasional cardiovascular abnormalities. The inflammation typically involves the cartilage of the ears, nose, trachea, larynx, ribs, joints, and Eustachian tubes. The major clinical features include auricular chondritis, arthritis, nasal chondritis, ocular inflammation, chondritis of the respiratory tract, audiovestibular damage, and cardiovascular disorders. RP occurs predominantly in Caucasians and is uncommon in Oriental patients. Two cases of RP have previously been reported in Taiwan. We report one Taiwanese patient who was diagnosed with RP according to the criteria proposed by Damiani and Levine. The patient has bilateral auricular chondritis, episcleritis, and uveitis. Successful treatment was accomplished with systemic corticosteroid and topical corticosteroid eye drops. At an outpatient visit one year and ten months after discharge, there was no evidence of recurrence.

Adult↗