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Relapsing polychondritis — Clinical Guidelines

Overview

Relapsing polychondritis (RP) is an uncommon autoimmune disorder characterized by recurrent inflammation primarily affecting cartilaginous structures, including the nose, ears, airways, and joints, but also impacting non-cartilage organs like blood vessels, skin, inner ear, and eyes 1 2.

Diagnosis

Key Clinical Features: Nasal bridge chondritis, auricular chondritis, ocular inflammation, bronchial tree involvement 2.

Recommended Tests:

- Imaging (e.g., CT, MRI) to assess organ involvement 1. - Laboratory tests for inflammatory markers (ESR, CRP) 1. - (18)F-FDG PET-CT for diagnosis, targeted biopsy, and monitoring disease activity 4.

Diagnostic Criteria: No universally accepted criteria; diagnosis often relies on clinical presentation and exclusion of other conditions 2.

Management

First-Line Treatments:

- Corticosteroids (dose varies; typically high initial doses for acute flares) 3 7.

Adjunctive Treatments:

- Immunosuppressive agents (e.g., methotrexate, azathioprine) for maintenance therapy 3. - Biologic agents (e.g., TNF inhibitors) in refractory cases 3.

Specific Interventions:

- Thoracic epidural morphine for intractable chest wall pain 7. - Surgical interventions for severe complications like valvular insufficiency 10.

Special Populations

Pregnancy: Limited data; management requires careful monitoring and individualized treatment plans 3.

Comorbidities: Patients with associated conditions like familial Mediterranean fever may require tailored management strategies 5.

Key Recommendations

Utilize (18)F-FDG PET-CT for accurate diagnosis and monitoring of disease activity in relapsing polychondritis (Evidence: Moderate) 4.

Initiate high-dose corticosteroids for acute flares and consider immunosuppressive therapy for maintenance to prevent relapses (Evidence: Expert opinion) 3.

Tailor pain management strategies, including thoracic epidural analgesia, for patients with severe chest wall pain (Evidence: Weak) 7.

Closely monitor and manage cardiac involvement, particularly valvular insufficiency, with potential surgical intervention if necessary (Evidence: Moderate) 10.

References

1 Mertz P, Sparks J, Kobrin D, Ogbonnaya SA, Sevim E, Michet C et al.. Relapsing polychondritis: Best Practice & Clinical Rheumatology. Best practice & research. Clinical rheumatology 2023. link 2 Kingdon J, Roscamp J, Sangle S, D'Cruz D. Relapsing polychondritis: a clinical review for rheumatologists. Rheumatology (Oxford, England) 2018. link 3 Smylie A, Malhotra N, Brassard A. Relapsing Polychondritis: A Review and Guide for the Dermatologist. American journal of clinical dermatology 2017. link 4 Lei W, Zeng H, Zeng DX, Zhang B, Zhu YH, Jiang JH et al.. (18)F-FDG PET-CT: a powerful tool for the diagnosis and treatment of relapsing polychondritis. The British journal of radiology 2016. link 5 Miller EB, Friedman JA, Lahav Y, Landau Z. Relapsing polychondritis and familial Mediterranean fever--an association. Clinical rheumatology 2011. link 6 Michet CJ. Vasculitis and relapsing polychondritis. Rheumatic diseases clinics of North America 1990. link 7 Waldman SD, Cronen MC. Thoracic epidural morphine in the palliation of chest wall pain secondary to relapsing polychondritis. Journal of pain and symptom management 1989. link90063-8) 8 Schlapbach P, Gerber NJ, Ramser P, van't Hooft FM. Relapsing polychondritis mimicking rheumatoid arthritis. Annals of the rheumatic diseases 1988. link 9 Hayward AW, al-Shaikh B. Relapsing polychondritis and the anaesthetist. Anaesthesia 1988. link 10 Balsa-Criado A, Garcia-Fernandez F, Roldan I. Cardiac involvement in relapsing polychondritis. International journal of cardiology 1987. link90214-2) 11 Weinberger A, Myers AR. Relapsing polychondritis associated with cutaneous vasculitis. Archives of dermatology 1979. link

Relapsing polychondritis