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Results for “Polychondritis, Relapsing”

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PubMed1989

Thoracic epidural morphine in the palliation of chest wall pain secondary to relapsing polychondritis.

Relapsing polychondritis is a rare disease of unknown etiology characterized pathologically by degeneration of the chondrocyte and replacement with fibrous connective tissue. The following case report presents the pain management of a 34-yr-old man suffering from intractable pain secondary to relapsing polychondritis. Systemic narcotic analgesics, adjunctive drugs, and peripheral nerve blocks with local anesthetic and steroid failed to adequately control the patient's pain. Thoracic epidural morphine was used to provide excellent relief of pain. Factors in the selection of an implantable narcotic delivery system as well as practical considerations including tolerance and potential side effects of intraspinal narcotics are discussed. Ethical issues surrounding the chronic use of intraspinal narcotics in the setting of chronic benign pain are also discussed.

Adult↗

PubMed1997

Management of laryngotracheobronchial sequelae and complications of relapsing polychondritis.

Relapsing polychondritis is a rare multisystem disorder of unknown etiology characterized by recurrent inflammation and degeneration of cartilage and connective tissue. Laryngotracheobronchial complications are the most severe manifestations of the disease and present the most challenging management decisions. We present four cases of relapsing polychondritis with laryngotracheobronchial manifestations that illustrate the clinical features and review the treatment options.

Adult↗

PubMed1987

Cardiac involvement in relapsing polychondritis.

Relapsing polychondritis is a rare disease characterized by recurrent inflammatory lesions involving cartilaginous structures, the eyes, the ears and the cardiovascular system. Valvar insufficiency represents the most common of the cardiac abnormalities and appears in 8 percent of patients. We describe two patients having relapsing polychondritis with cardiac involvement. One of them required valvar substitution and died 6 months after surgery.

Aortic Valve Insufficiency↗

PubMed2006

Endobronchial stenting for respiratory complications in relapsing polychondritis.

Relapsing polychondritis is characterized by recurrent inflammation and destruction of the cartilage and connective tissue. Respiratory complications are frequently severe during the course of the disease and usually signal a poor prognosis. We report a case of a 47-year-old man with known relapsing polychondritis who presented with bilateral narrowing of the airways complicated by refractory cavitary Pseudomonas aeruginosa pneumonia. Despite an aggressive antibiotic regimen, the patient's pneumonia did not improve until bronchial stenting 4 months later.

Airway Obstruction↗

PubMed2005

Uncommon CT findings in relapsing polychondritis.

Relapsing polychondritis is a rare inflammatory disorder of cartilage with well-established clinical features and imaging characteristics. Abnormal calcification and erosion of cartilaginous structures are the traditional radiographic findings. As with any disease, aberrancies of the expected clinical presentation may lead to a delayed (or missed) diagnosis. We discuss a rare case of relapsing polychondritis in which the diagnosis was suggested on the basis of CT findings, despite uncharacteristic clinical and radiographic manifestations of the disease.

Adult↗

PubMed1997

Cardiovascular involvement in relapsing polychondritis.

Relapsing polychondritis is an inflammatory disease that characteristically involves cartilagenous tissues. Cardiovascular involvement is a fairly common complication and the second most frequent cause of mortality in this disease. The case of a man with a progressive cardiac involvement, aortic incompetence, mitral regurgitation, and finally complete atrioventricular block offered the opportunity of reviewing the cardiovascular complications in relapsing polychondritis. The most frequent abnormalities are aortic regurgitation and aortic aneurysm. Furthermore, several cases of atrioventricular block, mitral regurgitation, and acute pericarditis have been reported. For early diagnosis and treatment of these severe complications, periodic cardiovascular examination is mandatory in these patients.

Aged↗

PubMed2001

Pulmonary fibrosis with intractable pneumothorax: new pulmonary manifestation of relapsing polychondritis.

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.

Blood Cell Count↗

PubMed1985

Relapsing polychondritis.

Relapsing polychondritis is an uncommon disease consisting of inflammation of the eyes, inner ears, cardiovascular system, and cartilaginous portions of the joints, respiratory tract, and external ear. Cutaneous manifestations are the presenting feature in more than 50% of patients. These usually consist of erythema, swelling, and pain, reflecting involvement of the underlying cartilage. Direct involvement of the skin may occur as vasculitis, lesions resembling erythema nodosum, or nonspecific eruptions. The presence of circulating antibodies to type II collagen--more against native than denatured collagen--and to human fetal cartilage, and the presence of circulating immune complexes suggest a primary role for antibody in the pathogenesis of relapsing polychondritis. There is no ideal treatment, but systemic corticosteroids and dapsone seem most effective.

Adult↗

PubMed2000

Autoreactivity against matrilin-1 in a patient with relapsing polychondritis.

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.

Aged↗

PubMed1994

Stroke as initial manifestation of relapsing polychondritis.

Relapsing polychondritis (RP) is a rare vasculitide characterized by recurrent inflammation of cartilagenous structures (auricular, nasal, laryngotracheal, articular) and specific sense organs (eyes and ear). Neurological complications are exceptional. We report the case of an elderly and previously well woman who presented with inflammatory syndrome and stroke as the first manifestations of this disease.

Aged↗

PubMed2005

Aseptic meningitis and ischemic stroke in relapsing polychondritis.

Relapsing polychondritis (RP) is a rare disease characterized by recurrent inflammation of cartilaginous structures. Its involvement of central nervous system is uncommon. We report the case of a 71-year-old woman whose initial manifestations were RP and meningitis and whose subsequent attack of RP was associated with a left cerebral infarction. The patient responded to steroid therapy in both attacks of RP.

Aged↗

PubMed2006

Pneumatosis cystoides intestinalis in relapsing polychondritis.

Relapsing polychondritis (RP) is a rare systemic disease characterized by an inflammatory process involving predominantly cartilaginous structures. Pneumatosis cystoides intestinalis (PCI) in RP has not been reported previously. We report a case of PCI in RP that was successfully treated with high oxygen and antibiotics.

Adult↗

PubMed2006

A case of severe dyspnea caused by relapsing polychondritis.

Relapsing polychondritis (RP) is a multi-systemic rheumatic disease characterized by widespread and potentially destructive inflammatory lesion of the cartilage. The rarity of the disease and the lack of pathognomonic laboratory findings sometimes cause delay in diagnosis. We report a 72-year-old woman with RP. When she was referred to our hospital 4 weeks after the onset of respiratory symptoms, she was having severe breathing difficulty. Immediate tracheostomy followed by steroid therapy dramatically improved her condition of RP, although the treatment was complicated due to her other condition, depression. While airway involvement of RP can be life threatening, it is curable with steroid therapy. Otolaryngologists should keep in mind that airway obstruction could be caused by RP.

Aged↗

PubMed2004

Relapsing polychondritis.

Relapsing polychondritis (RP) is a rare multisystem autoimmune disease of unknown origin characterized by recurrent episodes of inflammation and progressive destruction of cartilaginous tissues. Elastic cartilage of the ears and nose, hyaline cartilage of peripheral joints, vertebral fibrocartilage and tracheobronchial cartilage, as well as proteoglycan-rich structures of the eye, heart, blood vessels or inner ear may all be affected. In most patients RP manifests in a fluctuating but progressive course which eventually results in a significant shortening of life expectancy. The relatively uncommon occurrence, the unknown etiopathogenesis, the ambiguous clinical pattern, as well as the variety in its course and response to therapy may all contribute to the difficulties the physician must overcome when managing RP. Beside describing the main features of RP and seven clinical cases of our own, in the present review we focus on recent findings in the etiopathogenesis and novel treatment options.

Animals↗

PubMedDate not supplied

Relapsing polychondritis.

Relapsing polychondritis is a rare disease most commonly presenting as inflammation of the cartilage of the ears and nose. Auricular chondritis, with red ears resembling infectious cellulitis, is the most common initial finding. Antibodies to type II collagen in cartilage are found, and the earlobes are classically spared. Chronic disease may result in a flabby, droopy ear, cauliflower ear, or saddle nose deformity. Acute involvement of the tracheal cartilage may cause collapse of the airway with obstruction and pulmonary infections. Arthritis may be oligoarticular or polyarticular, most often involving the costochondral junctions. Other manifestations include audiovestibular damage; heart valve disease; and neurologic, ocular, and renal disease. Corticosteroids remain the major treatment. Other therapies include nonsteroidal anti-inflammatory drugs, dapsone, colchicine, azathioprine, methotrexate, cyclophosphamide, hydroxychloroquine, cyclosporine, and infliximab.

Anti-Inflammatory Agents, Non-Steroidal↗

PubMed2004

Nonparaneoplastic limbic encephalitis with relapsing polychondritis.

Relapsing polychondritis (RP), which shows pain, swelling and destruction of the affected parts, is a rare autoimmune disorder affecting cartilage. We report a patient with RP that affected skull cartilage, who subsequently developed multifocal meningoencephalitis. The patient presented with severe recent memory disturbance, anxiety and moderate depression. MRI study showed bilateral median temporal lobe lesions including hippocampi and amygdaloidal bodies, abnormal findings that disappeared after treatment with high-dose steroids. This is thought to be the first case of RP presenting amnesic syndrome and mental disorder associated with nonparaneoplastic limbic encephalitis involving bilateral hippocampi and amygdaloidal bodies detected by MRI.

Anxiety↗

PubMed1988

Relapsing polychondritis.

Relapsing polychondritis is a progressive multisystem disorder that predominantly affects cartilage, producing an inflammatory reaction. We present case histories from four patients having mainly respiratory symptoms in whom we measured the coronal diameters of the trachea and compared them with the tracheal measurements from radiographs of patients with no respiratory symptoms. The results showed that the disease produced diffuse narrowing of the main airway in all four patients. Early detection of tracheal narrowing is important, as patients presenting with respiratory tract disease have a worse prognosis than those who develop it later in the course of their illness. Other features of the disease are also discussed with reference to the cases presented.

Adult↗

PubMed2005

Laryngotracheal involvement as the initial manifestation of relapsing polychondritis.

Relapsing polychondritis (RP) is a rare multisystemic disease characterized by recurrent inflammation of cartilaginous and noncartilaginous tissues. When laryngotracheal or bronchial cartilages are involved, the disease can be life-threatening and needs aggressive treatment. Upper airway complaints are rare as initial presentations of RP. Here, however, we present a case of RP, with initial manifestations of cough and dyspnea that were treated as bronchial asthma for 6 months. Subglottic stenosis was found in April 2003, during a bronchoscopic examination, and emergency tracheostomy was performed. Auricular and nasal chondritis and bilateral scleritis developed 3 months after tracheostomy. High doses of methylprednisolone and immunosuppressive agents were used, and active inflammation in the eyes and ears was controlled, but the patient's upper airway was completely collapsed. This case is reported with the hope of increasing awareness about the potential for early upper airway involvement in RP.

Airway Obstruction↗