Relapsing Polychondritis: an Update on Pathogenesis, Clinical Features, Diagnostic Tools, and Therapeutic Perspectives

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Relapsing Polychondritis: an Update on Pathogenesis, Clinical Features, Diagnostic Tools, and Therapeutic Perspectives

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作者：Antonio Vitale ; Jurgen Sota ; Donato Rigante…
关键词：Relapsing polychondritis ; Cartilaginous tissue ; Biologics ; Autoimmune diseases ; Therapy
刊名：Current Rheumatology Reports
出版年：2016
出版时间：January 2016
年：2016
卷：18
期：1
全文大小：496 KB
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作者单位：Antonio Vitale (1)
Jurgen Sota (1)
Donato Rigante (2)
Giuseppe Lopalco (3)
Francesco Molinaro (4)
Mario Messina (4)
Florenzo Iannone (3)
Luca Cantarini (1)

1. Research Center of Systemic Autoimmune and Autoinflammatory Diseases, Rheumatology Unit, Policlinico Le Scotte, University of Siena, Viale Bracci 1, 53100, Siena, Italy
2. Institute of Pediatrics, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli, Rome, Italy
3. Rheumatology Unit, Interdisciplinary Department of Medicine, Policlinico of Bari, Bari, Italy
4. Division of Pediatric Surgery, Department of Medical Sciences, Surgery, and Neuroscience, University of Siena, Siena, Italy

刊物主题：Rheumatology;

出版者：Springer US

ISSN：1534-6307

文摘

Relapsing polychondritis is a rare multisystemic disease widely accepted as a complex autoimmune disorder affecting proteoglycan-rich structures and cartilaginous tissues, especially the auricular pinna, cartilage of the nose, tracheobronchial tree, eyes, and heart’s connective components. The clinical spectrum may vary from intermittent inflammatory episodes leading to unesthetic structural deformities to life-threatening cardiopulmonary manifestations, such as airway collapse and valvular regurgitation. The frequent association with other rheumatologic and hematologic disorders has been extensively reported over time, contributing to define its complexity at a diagnostic and also therapeutic level. Diagnosis of relapsing polychondritis is mainly based on clinical clues, while laboratory data have only a supportive contribution. Conversely, radiology is showing a relevant role in estimating the rate of systemic involvement as well as disease activity. The present review is aimed at providing an update on scientific data reported during the last 3 years about relapsing polychondritis in terms of pathogenesis, clinical features, diagnosis, and new treatment options.

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