Makindo Medical Notes

Related Subjects: |Relapsing Polychondritis |Reactive Arthritis |Raynaud's Phenomenon |Polymyositis |Dermatomyositis |Polyarteritis nodosa |Osteoporosis |Rheumatoid Arthritis |Systemic Sclerosis (Scleroderma) |Rheumatology Autoantibodies |Overlap Syndrome |Sjögren’s syndrome

🎧 Relapsing Polychondritis is a rare, immune-mediated disorder characterised by recurrent inflammation and destruction of cartilaginous structures. 🧬 Often associated with antibodies to Type II collagen.

📘 About

• Autoimmune inflammation of cartilage and proteoglycan-rich tissues.
• Typically presents in middle age, but may occur in both children and elderly patients.
• ~30% of cases are associated with other autoimmune or haematological diseases:
  • Behçet's syndrome, IBD, systemic vasculitis, RA, SLE, Sjögren’s.
  • Primary biliary cirrhosis, myelodysplasia.

🩺 Clinical Presentation

• Systemic: Fever, malaise, lethargy.
• Auricular chondritis: Painful, red, swollen ear sparing the lobule (classic sign).
• Nasal cartilage: Destruction leads to saddle-nose deformity.
• Laryngotracheobronchial: Hoarseness, stridor, airway collapse (life-threatening).
• Cardiac: Aortic or mitral regurgitation due to valve involvement.
• Ocular: Episcleritis, scleritis, uveitis.
• Other: Hearing loss, arthritis, pulmonary complications.

🔬 Investigations

• FBC: Mild leukocytosis, normochromic normocytic anaemia.
• Inflammatory markers: ↑ ESR and CRP.
• Autoantibodies: Anti-Type II collagen; sometimes pANCA or cANCA.
• Imaging: CT/MRI may show airway collapse or cartilage damage.
• Biopsy: Confirms chondritis with lymphocytic infiltration and cartilage destruction.

🧾 Differential Diagnosis

• Granulomatosis with Polyangiitis (GPA)
• Sarcoidosis
• Infective chondritis (rare, post trauma or surgery)

💊 Management

• First-line: High-dose corticosteroids (e.g. prednisolone).
• Steroid-sparing agents: Methotrexate, azathioprine, cyclophosphamide.
• Dapsone: May reduce relapse frequency in some patients.
• Biologics: Anti-TNF or rituximab in refractory disease.
• Supportive: ENT input for airway disease; cardiology for valve involvement.

📚 Key Point

• Relapsing polychondritis can be life-threatening if airway or cardiovascular involvement occurs — early recognition and immunosuppression are crucial.

Cases — Relapsing Polychondritis (RP)

• Case 1 — Auricular chondritis 👂: A 46-year-old woman presents with recurrent painful swelling and redness of both pinnae, sparing the ear lobes. Episodes resolve but recur, leaving deformity of the external ear. No fever or systemic symptoms. Diagnosis: relapsing polychondritis affecting auricular cartilage. Treated with NSAIDs and corticosteroids during flares.
• Case 2 — Airway involvement 🫁: A 52-year-old man reports hoarseness, cough, and episodes of stridor. Exam: tender laryngeal cartilage. CT: tracheal wall thickening with sparing of the posterior membrane. Diagnosis: RP with airway involvement. Managed with systemic corticosteroids and close ENT/respiratory monitoring (risk of airway collapse).
• Case 3 — Multisystem disease 🌐: A 60-year-old woman presents with recurrent episcleritis, nasal bridge pain, and polyarthritis. Exam: saddle-nose deformity and inflamed sclera. Past history of recurrent ear chondritis. Diagnosis: systemic RP. Treated with high-dose steroids and methotrexate as a steroid-sparing agent.

Teaching Point 🩺: Relapsing polychondritis is a rare, immune-mediated disease causing recurrent inflammation of cartilaginous structures (ears, nose, trachea, ribs) and sometimes eyes and joints. Think of it with painful red ears sparing the lobes or unexplained airway disease. Mainstay treatment is corticosteroids ± immunosuppressants.