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Rheumatology (Oxford). 2018 Sep 1;57(9):1525-1532. doi: 10.1093/rheumatology/kex406.

Relapsing polychondritis: a clinical review for rheumatologists.

Author information

1
School of Medical Education, King's College London, UK.
2
Louise Coote Lupus Unit, Guy's Hospital, London, UK.

Abstract

Relapsing polychondritis (RPC) is a rare autoimmune rheumatic disorder that is traditionally classified as a systemic vasculitis. It is characterized by inflammation of cartilage, and typical presenting features include chondritis of the nasal bridge, auricular chondritis, ocular inflammation and involvement of the bronchial tree. Its rarity often leads to considerable delay in establishing a diagnosis and poses a significant management challenge to clinicians, as no conventional guidelines exist. This review summarizes the clinical features of RPC and provides guidance for rheumatologists on making the diagnosis and assessing organ involvement. The current state of RPC management is reviewed, with a focus on the use of the anti-TNF-α agents in patients with pulmonary involvement, the leading cause of mortality and morbidity in RPC.

PMID:
29126262
DOI:
10.1093/rheumatology/kex406
[Indexed for MEDLINE]

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