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Results for “Polychondritis, Relapsing”

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At least 19 recordsLinked to original sources
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↗
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↗
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↗
PubMed1976

Multiple aortic aneurysms in relapsing polychondritis.

A patient with relapsing polychondritis and thoracic and abdominal aortic aneurysms is described. The aortic changes were due to aortitis, which primarily involved the media, with increased vascularization, perviascular infiltration of mononuclear cells, increased amounts of collagen and decreased amounts of elastic tissue and sulfated acid mucopolysaccharides. Aortic aneurysms frequently occur in relapsing polychondritis; they are usually in the ascending aorta but may be multiple and involve the abdominal aorta; involvement of the ascending aorta results in aortic regurgitation and left ventricular failure, and involvement of the abdominal aorta may be clinically silent and result in fatal rupture.

Adult↗
PubMed1978

Relapsing polychondritis with crescentic glomerulonephritis.

Relapsing polychondritis is rare and its cause is unknown. The tissues affected are those with a high glycosaminoglycan content, such as cartilage, the aorta, the sclera and cornea, and parts of the ear. Symptoms can usually be controlled with oral steroids, but when there is coexistent progressive crescentic glomerulonephritis quadruple chemotherapy may be used. Three cases of the clinical syndrome of relapsing polychondritis were studied in which rapidly progressive cresentic glomerulonephritis developed. In two the patients appeared to respond to aggressive treatment with immunosuppressive agents and anticoagulants. The multisystemic nature of the disease, the renal lesions, and the response to treatment all suggested that the condition might be related to periarteritis nodosa.

Adult↗
PubMed1976

Relapsing polychondritis treated with dapsone.

Relapsing polychondritis is a rare and sometimes fatal disease of unknown cause and pathogenesis. Results of recent studies suggest that it is an immune disorder probably caused by the production of autoantibodies to possibly the mucopolysaccharides. Perpetuation of the cartilage inflammation may be maintained by a proteolytic process. Treatment primarily has been with orally given corticosteroids. Dapsone has been shown to possibly inhibit lysosomal enzyme activity. We present a patient who, to our knowledge, is the first with relapsing polychondritis to be treated successfully with dapsone. The rapid response of the patient's condition to treatment with dapsone suggests an alternative, if not preferred, method of controlling this disease.

Dapsone↗
PubMed1992

Relapsing polychondritis: new pulmonary manifestations.

Relapsing polychondritis is purported to be an autoimmune disease characterized by inflammation of cartilaginous structures including the nose, ears, glottis, trachea, and mainstem bronchi. Arthropathy, aortopathy, scleritis, conjunctivitis, iritis, vertigo, otitis media, glomerulonephritis, and skin lesions are common manifestations. We have recently cared for an elderly nonsmoker with a history of abdominal aortic aneurysm repair and recurrent bouts of painful ear swelling who presented with a pulmonary infiltrate and pulmonary function studies suggestive of small airways disease. Subsequent transbronchial biopsy revealed no pathogens but was associated with excessive bleeding, perhaps suggestive of a vasculitic process in the lung. The pulmonary infiltrate cleared over a 2-month interval with the use of corticosteroids. Unusual in this otherwise classical case of relapsing polychondritis was that the pulmonary infiltrate and small airways disease were the prominent pulmonary manifestations and no tracheobronchial abnormalities were visualized.

Adrenal Cortex Hormones↗
PubMed2026

The utility of 18F-fluorodeoxyglucose PET/computed tomography in relapsing polychondritis: a systematic review and meta-analysis.

Relapsing polychondritis is a rare chronic autoimmune inflammation of the cartilage associated with life-threatening respiratory complications. Currently, no clear role of imaging modalities such as 18F-fluorodeoxyglucose (FDG) PET/computed tomography (CT) is defined in the literature. This systematic review and meta-analysis provide current evidence on the PET-positivity rate and utility in relapsing polychondritis. Prospective or retrospective studies with more than five patients of suspected relapsing polychondritis who underwent 18F-FDG PET/CT during their management and reported a PET-positivity rate were included. Low-sample-size studies describing chondritis due to other aetiologies or utilizing PET-based radiopharmaceuticals other than FDG were excluded. A systematic search using relevant keywords was conducted across four databases (PubMed, Embase, Scopus and Web of Science) to include studies up to 25 April 2025. The Joanna Briggs Institute critical appraisal tools were used for risk-of-bias analysis. Data were analysed using the R software package (v4.3.1; 2023). Out of 962 articles, three with a total of 97 patients were included. With a pooled PET-positivity rate of 94% [95% confidence interval (CI): 73-99%, I2 = 0%, P = 0.76] and a pooled baseline SUVmax of 4.0 (95% CI: 3.5-4.6, I2 = 32%, P = 0.23), 18F-FDG PET identified asymptomatic cartilage involvement in more than 25% patients and PET parameters correlated well with inflammatory markers. It had a higher positivity rate for inaccessible sites, such as peripheral airways, and was crucial in treatment monitoring. The pooled PET-positivity rate of 18F-FDG PET in relapsing polychondritis is high but requires prospective large-sample-size studies to explore the diagnostic accuracy and prognostic implications of 18F-FDG PET in relapsing polychondritis.

Polychondritis, Relapsing↗
PubMed1992

Early aortic valve cusp rupture in relapsing polychondritis.

Aortic regurgitation associated with relapsing polychondritis usually occurs late in the disease as a result of aortic root dilatation. A case where aortic regurgitation occurred early and was due to cusp rupture with a normal aortic root is reported. The patient required urgent aortic valve replacement within six weeks of developing a murmur despite apparent control of inflammation with immunosuppressive treatment. The possibility of cusp rupture with sudden haemodynamic deterioration should be considered in patients with relapsing polychondritis who develop aortic regurgitation.

Aortic Valve↗
PubMed1990

[Relapsing polychondritis in a patient with myelodysplastic syndrome].

Relapsing polychondritis is a rare disorder of uncertain origin characterized by recurrent inflammation of cartilage. A case of myelodysplastic syndrome (MDS) associated with relapsing polychondritis is reported. A 60-year-old man who had been diagnosed as MDS was admitted because of pain and swelling in the bilateral preauricular regions and cheek. A diagnosis of relapsing polychondritis was made by coexistence of auricular chondritis, arthropathy, ocular inflammation and audio-vestibular disturbance. He also developed ocular palsies and optic neuritis. He was treated with prednisolone, azathioprine, dapsone, and then with steroid pulse therapy. Moreover, plasmapheresis and high dose gamma-globulin therapy were undertaken. However, all these treatments were unsuccessful and he died of respiratory failure.

Azathioprine↗
PubMed1976

Treatment of relapsing polychondritis with dapsone.

Three patients with relapsing polychondritis were treated successfully with dapsone. Mounting evidence suggests an immune-related pathogenesis for relapsing polychondritis. Dapsone may be effective in certain immune-related diseases because of its inhibition of lysosomal enzymes.

Aged↗
PubMed1992

Progressive aortic valve inflammation occurring despite apparent remission of relapsing polychondritis.

We describe a patient with relapsing polychondritis in whom aortic valve inflammation developed 3 years after diagnosis, when the polychondritis had been in apparent remission for an extended period of time. Infection and cardiac involvement can be significant complications of relapsing polychondritis. Recommendations for monitoring and treatment of patients with this disease are discussed.

Adult↗
PubMed1979

Relapsing polychondritis--report of ten cases.

Relapsing polychondritis is a rare disease of unknown etiology. There are approximately 211 reported cases in the world literature. This is a report of ten cases from the Cleveland Clinic Foundation. McAdam's diagnostic criteria for R.P. were reviewed and modified. For diagnosis, all patients had to have 1. at least three or more diagnostic criteria, histologic confirmation not necessary; 2. one or more of McAdam's signs with positive histologic confirmation; or 3. chondritis in two or more separate anatomic locations with response to steroids and/or Dapsone. Chondritis of the auricles (9/10 patients) and arthropathy (8/10 patients) are the most common presenting signs. Chondritis was also seen in the nose (6/10) and the upper respiratory tract involving the larynx and trachea (4/10). Cochlear and vestibular damage and ocular inflammation were each seen in 5/10 patients. Patients were treated with steroids and/or Dapsone. Both drugs were reliable in abating episodes of activity and in decreasing recurrences. These results further support Dapsone as an alternate form of treatment for RP.

Adolescent↗
PubMed1976

Hypocomplementemic ear effusion in relapsing polychondritis.

In a case of relapsing polychondritis it was possible to aspirate a collection of subcutaneous fluid from the patient's involved ear. A determination of total hemolytic complement activity of this fluid was low, suggesting that activation of the complement system may have occurred in the course of the patient's disease and might be related to the pathogenesis of this disorder.

Complement System Proteins↗
PubMed1979

Relapsing polychondritis: unusual neurological findings and therapeutic efficacy of dapsone.

Relapsing polychondritis is a rare disease of undetermined cause. The most frequently seen symptom is redness and swelling of the cartilaginous ear, followed by cartilage inflammation elsewhere in the body. Our five cases had neurologic symptoms that posed diagnostic difficulties. Because vasculitis is not uncommon in relapsing polychondritis, it was difficult to determine whether these neurologic symptoms were attributable to relapsing polychondritis or to other unrelated causes. In our cases, specific neurologic complaints were caused by a concomitant but unrelated disease, rather than by relapsing polychondritis. We add five additional documented cases to the medical literature and confirm the therapeutic efficacy of dapsone in the treatment of this unusual disease.

Adult↗
PubMed1977

Vascular involvement in relapsing polychondritis.

Review of four cases of relapsing polychondritis (RP) seen at one hospital in the 12-year period 1963 to 1974 revealed that one patient had aortic insufficiency with large artery involvement, two others had involvement of medium and large arteries and the fourth may have had mucocutaneous vasculitis. Valvular disease has occurred in 9% of all cases of RP reported in the literature and, if vasculitis beyong the aortic root is included, 25% of cases of RP manifested inflammatory vascular disease. The frequency of pseudotumour of the orbit and cochlear-labyrinthine dysfunction is also high and may be a manifestation of vasculitis.

Aged↗