1986
DOI: 10.7326/0003-4819-104-1-74
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Relapsing Polychondritis

Abstract: To define the natural history of relapsing polychondritis, the probability of survival and causes of death were determined in 112 patients seen at one institution. By using covariate analysis, early clinical manifestations were identified that predicted mortality. The 5- and 10-year probabilities of survival after diagnosis were 74% and 55%, respectively. The most frequent causes of death were infection, systemic vasculitis, and malignancy. Only 10% of the deaths could be attributed to airway involvement by ch… Show more

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Cited by 584 publications
(210 citation statements)
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“…It can affect vessels of all sizes and large vessel vasculitis is a well known complication. The latter most frequently involves the aortic ring and the ascending aorta, though the thoracic and abdominal aorta may be affected by aneurysms or rupture [56]. The various conditions that may be associated with RP besides vasculitic disorders are other autoimmune rheumatic diseases.…”
Section: Disease Associationsmentioning
confidence: 99%
“…It can affect vessels of all sizes and large vessel vasculitis is a well known complication. The latter most frequently involves the aortic ring and the ascending aorta, though the thoracic and abdominal aorta may be affected by aneurysms or rupture [56]. The various conditions that may be associated with RP besides vasculitic disorders are other autoimmune rheumatic diseases.…”
Section: Disease Associationsmentioning
confidence: 99%
“…According to the criteria, obtaining histological confirmation of RP is not necessary if the patient has three or more of the following conditions: 1) bilateral auricular relapsing chondritis; 2) nasal chondritis; 3) ocular inflammation; 4) non-erosive inflammatory arthritis; 5) respiratory tract chondritis; and 6) cochlear and/or vestibular damage. Among patients with RP, 22-66% test positive for antinuclear antibodies, 16% test positive for rheumatoid factor (RF) (7)(8)(9) and approximately 30-70% test positive for autoantibodies against type II collagen, a component of cartilage (1), thus indicating that some autoimmune abnormalities are associated with the disease. Antitype II collagen antibodies are also detected in approximately 15% of patients with chronic arthritis.…”
Section: Discussionmentioning
confidence: 99%
“…La expresión clínica de este proceso traduce la afectación de estructuras cartilaginosas, fundamentalmente otorrinolaringológicas, cardiovasculares y de algunos órganos de los sentidos. La afectación laringotraqueobronquial se da en el 49% de los casos, siendo ésta y la afectación cardiovascular indicadoras de mal pronóstico (10). El diagnóstico es clínico, siguiendo los criterios establecidos por McAdam (2) y modificados por Damiani (3), no siendo necesario el estudio anatomopatológico en todos los casos .…”
Section: Discussionunclassified
“…Esta inespecificidad de la sintomatología hace, que si el cuadro no es florido inicialmente, pueda confundirse con otro proceso, dando lugar a un retraso en el diagnóstico (16,17). Los síntomas relacionados con la afectación laringotraqueal son signos de mal pronósti-co, ya que puede dar lugar a insuficiencia respiratoria aguda debida al colapso de la vía aérea (2,10).…”
Section: Discussionunclassified