Relapsing Polychondritis

, David G. I. Scott² and Chetan Mukhtyar²

(1)

Department of Rheumatology, Ipswich Hospital NHS Trust, Ipswich, UK

(2)

Department of Rheumatology, Norfolk and Norwich University Hospital, Norwich, UK

18.1 Introduction

Relapsing polychondritis (RP) is a rare condition characterized by inflammation and destruction of cartilage.

18.2 Definition and Classification

There are no validated diagnostic criteria, however a diagnosis of RP should be considered if at least three of the features given in Table 18.1 are present, or one of the clinical features with a biopsy confirmation or chondritis at two or more locations which responds to treatment with steroids and/or dapsone [1].

Table 18.1

Characteristic features of RP

Recurrent chondritis of both auricles

Non-erosive seronegative inflammatory polyarthritis

Nasal chondritis

Inflammation of ocular structures (conjunctivitis, keratitis, scleritis, episcleritis, uveitis)

Chondritis of the respiratory tract involving laryngeal and/or tracheal cartilage

Cochlear and/or vestibular damage causing sensorineural hearing loss, tinnitus and/or vertigo

18.3 Epidemiology

The incidence of RP has been estimated to be 3.5/million in Rochester County, USA. Males and females are affected equally, and the peak age of onset is 50 years.

Relapsing polychondritis is more common around the Mediterranean littoral, the Silk Route and Japan, but has been described in every ethnic group.

18.4 Etiology

The etiology is unknown, but like many autoimmune conditions, is generally believed to result from an environmental trigger interacting with a genetically predisposed host. Familial cases are rare. There is an association with HLA-DR4. No infectious triggers have been identified. There is frequently a co-existent autoimmune disease. Antibodies to type II collagen are found.

18.5 Clinical Features

18.5.1 Systemic

The initial symptoms may be nonspecific with fever, weight loss and fatigue.

18.5.2 Cartilage Inflammation

Auricular inflammation is present in almost all patients (85 %) (Fig. 18.1). This involves the cartilaginous part of the pinna and spares the noncartilaginous lobe. The pinna becomes red, painful, swollen, and these inflammatory episodes last a few days or weeks. Following recurrent inflammation, the pinna becomes floppy and loses its rigidity. There may be inflammation of the middle ear and audiovestibular structures.