Aortic involvement in relapsing polychondritis. 2018

Maëlle Le Besnerais, and Laurent Arnaud, and Jonathan Boutémy, and Boris Bienvenu, and Hervé Lévesque, and Zahir Amoura, and Isabelle Marie
Department of Internal Medicine, Rouen University Hospital, 1, rue de Germont, 76000 Rouen, France; INSERM U 905, University of Rouen IFRMP, Institute for Biochemical Research, Rouen University Hospital, 76000 Rouen, France.

To assess prevalence of aortic involvement in relapsing polychondritis (RP) patients; to evaluate clinical features and long-term outcome of RP patients exhibiting aortitis, aortic ectasia and/or aneurysm. One hundred and seventy-two RP patients underwent aortic computed tomography (CT)-scan; they were seen in 3 medical centers. Eleven patients (6.4%) had aortic involvement, occurring within a median time of 2 years after RP diagnosis. CT-scan showed isolated aortitis (n=2); the 9 other patients exhibited: aortitis and aortic aneurysm (n=2) or ectasia (n=1), isolated aortic aneurysm (n=4) or ectasia (n=2); aortic localizations were as follows: thoracic (n=6), abdominal (n=2), thoracic and abdominal (n=4) aorta. Patients exhibited: resolution (n=3) improvement (n=3), stabilization (n=4) or deterioration (n=1) of aortic localization. Five patients experienced recurrence of aortic localization; one patient died of aortic abdominal aneurysm rupture. Predictive factors of death related to aortic complications were: aortitis on CT-scan, higher median levels of erythrocyte sedimentation rate. Predictive parameters of aortic relapses were: aortitis on CT-scan and involvement of the abdominal aorta. This study underlines that aortic involvement is severe in RP. Furthermore, we suggest that RP patients exhibiting poor prognostic factors, including panaortitis and higher values of ESR, may require more aggressive therapy.

UI MeSH Term Description Entries
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D011081 Polychondritis, Relapsing An acquired disease of unknown etiology, chronic course, and tendency to recur. It is characterized by inflammation and degeneration of cartilage and can result in deformities such as floppy ear and saddle nose. Loss of cartilage in the respiratory tract can lead to respiratory obstruction. Polychondritis, Chronic Atrophic,Atrophic Polychondritides, Chronic,Atrophic Polychondritis, Chronic,Chronic Atrophic Polychondritides,Chronic Atrophic Polychondritis,Polychondritides, Chronic Atrophic,Polychondritides, Relapsing,Relapsing Polychondritides,Relapsing Polychondritis
D011237 Predictive Value of Tests In screening and diagnostic tests, the probability that a person with a positive test is a true positive (i.e., has the disease), is referred to as the predictive value of a positive test; whereas, the predictive value of a negative test is the probability that the person with a negative test does not have the disease. Predictive value is related to the sensitivity and specificity of the test. Negative Predictive Value,Positive Predictive Value,Predictive Value Of Test,Predictive Values Of Tests,Negative Predictive Values,Positive Predictive Values,Predictive Value, Negative,Predictive Value, Positive
D011379 Prognosis A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations. Prognostic Factor,Prognostic Factors,Factor, Prognostic,Factors, Prognostic,Prognoses
D004108 Dilatation, Pathologic The condition of an anatomical structure's being dilated beyond normal dimensions. Ectasia,Dilatation, Pathological,Dilatations, Pathologic,Dilatations, Pathological,Pathologic Dilatation,Pathologic Dilatations,Pathological Dilatation,Pathological Dilatations
D005260 Female Females
D005602 France A country in western Europe bordered by the Atlantic Ocean, the English Channel, the Mediterranean Sea, and the countries of Belgium, Germany, Italy, Spain, Switzerland, the principalities of Andorra and Monaco, and by the duchy of Luxembourg. Its capital is Paris. Corsica,Saint Pierre and Miquelon,Miquelon and Saint Pierre,Miquelon and St. Pierre,St. Pierre and Miquelon
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000046 Academic Medical Centers Medical complexes consisting of medical school, hospitals, clinics, libraries, administrative facilities, etc. Medical Centers, Academic,Medical Centers, University,University Medical Centers,Academic Medical Center,Center, Academic Medical,Center, University Medical,Centers, Academic Medical,Centers, University Medical,Medical Center, Academic,Medical Center, University,University Medical Center

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