Relapsing polychondritis: an update on pathogenesis, clinical features, diagnostic tools, and therapeutic perspectives

Donato Rigante, Antonio Vitale, Jurgen Sota, Giuseppe Lopalco, Francesco Molinaro, Mario Messina, Florenzo Iannone, Luca Cantarini

Risultato della ricerca: Contributo in rivistaArticolo in rivista

28 Citazioni (Scopus)

Abstract

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.
Lingua originaleEnglish
pagine (da-a)1-10
Numero di pagine10
RivistaCurrent Rheumatology Reports
DOI
Stato di pubblicazionePubblicato - 2016

Keywords

  • Relapsing polychondritis

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