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Relapsing polychondritis

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Abstract

Relapsing polychondritis (RP) is a rare multisystem autoimmune disease of unknown origin characterized by recurrent episodes of inflammation and progressive destruction of cartilaginous tissues. Elastic cartilage of the ears and nose, hyaline cartilage of peripheral joints, vertebral fibrocartilage and tracheobronchial cartilage, as well as proteoglycan-rich structures of the eye, heart, blood vessels or inner ear may all be affected. In most patients RP manifests in a fluctuating but progressive course which eventually results in a significant shortening of life expectancy.

The relatively uncommon occurrence, the unknown etiopathogenesis, the ambiguous clinical pattern, as well as the variety in its course and response to therapy may all contribute to the difficulties the physician must overcome when managing RP. Beside describing the main features of RP and seven clinical cases of our own, in the present review we focus on recent findings in the etiopathogenesis and novel treatment options.

Section snippets

History

The first case of relapsing polychondritis (RP) was reported in 1923 by Jaksch-Wartenhorst who called it ‘polychondropathy’.1 He described a 32-year-old male patient who presented with fever, asymmetric polyarthritis, pain, swelling and deformity of the ears and nose, as well as stenosis of the external auditory canals which resulted in diminished hearing. Biopsy of the nasal cartilage revealed loss of cartilage matrix and a hyperplastic mucous membrane. In 1960 Pearson et al reviewed 12 cases,

Epidemiology

The estimated annual incidence rate is 3.5/million in Rochester, Minnesota (personal observations by Michet and Luthra). The peak age for disease onset is the fifth decade, although cases have been reported in children as well as in the very elderly.3 RP is usually known to occur with equal frequency in both sexes; however, some authors found an increased prevalence among women. It seems to occur with equal frequency in all racial groups, but there are very few data available on non-Caucasian

Etiopathogenesis

The cause of polychondritis is unknown. At first it was suspected by Jaksch-Wartenhorst to be of degenerative origin, but since then several lines of evidence have accumulated to support the role of the immune system:

  • frequent association with other autoimmune diseases

  • CD4+ T lymphocytes, plasma cells, immunglobulins and complement are detectable in tissue lesions

  • presence of autoantibodies to type II, IX and XI collagens, and other cartilage proteins, including matrilin-1 and cartilage oligomeric

Clinical features

Disease onset is usually sudden and flagrant, with characteristic clinical signs. Table 3 summarizes the clinical features of patients with RP in four large series and a small case series reported in the literature.3., 6., 12., 25., 26. Non-specific initial symptoms such as fever, weight loss, fatigue or lethargy may also be present and delay the diagnosis significantly if no characteristic clinical signs are seen.

Auricular chondritis is present in almost all patients, involving the

Diagnosis and investigations

The diagnosis of RP is usually easy, based on characteristic clinical manifestations. Empirical diagnostic criteria (Table 4), originally proposed by McAdam et al25, were later modified by Michet et al3 and Damiani.34 Patients may sometimes be diagnosed after a significant delay from the onset of the disease if initial symptoms are non-specific. Moreover, many conditions can mimic the initial features, or an associated disorder may confuse the clinical pattern.

Laboratory findings in RP are

Differential diagnosis

Other types of chondritis and perichondritis should be ruled out. Infectious diseases—including acute streptococcal or fungal infection, leprosy and syphilis—may also lead to perichondritis. These conditions, as well as tuberculosis, Wegener's granulomatosis or lethal midline granuloma, may cause nasal damage, also a feature of RP. Auricular involvement must be differentiated from traumatic and chemical conditions, such as frostbite and insect bite. Unilateral auricular chondritis alone may be

Management

Treatment has primarily been symptomatic in RP. Non-steroidal anti-inflammatory drugs alone are adequate for patients with mild auricular or nasal chondritis and/or arthralgia in some patients. More serious manifestations, including acute polychondritis, laryngotracheal or ocular symptoms, or systemic vasculitis, require corticosteroids, prednisone or equivalent in doses of 0.5–1 mg/kg of body weight per day. Higher-dose oral steroids or even intravenous pulse therapy in doses of 1000 mg/day

Prognosis

RP generally shows an intermittent but progressive course. Sites of involvement, the severity of inflammation, inner organ manifestations and response to therapy are unpredictable. Eventually, most patients develop some degree of disability which may include hearing or visual impairment, phonation difficulties or cardiorespiratory problems. Musculoskeletal complications in RP, if not associated with RA, are rare. The life expectancy of patients with RP is reduced: earlier studies indicated

Summary

RP is a rare disorder occurring at nearly all ages. Its etiopathogenesis is still unknown, and diagnosis and monitoring are based on clinical symptoms. However, recent findings indicate that cartilage constituents, including CII and matrilin-1, in genetically susceptible individuals may trigger the autoimmune response leading to cartilage destruction. Cartilage degradation products such as urinary type II collagen neoepitope may be suitable for monitoring disease activity and response to

Acknowledgements

The authors thank Dr A. Judák (National Institute of Rheumatology and Physiotherapy, Budapest) for the photos of her patient with relapsing polychondritis.

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