Relapsing Polychondritis
Relapsing polychondritis (RP) is a very rare systemic autoimmune disease that runs a chronic course with repeated flares. Its defining feature is destructive inflammation that targets cartilage and other proteoglycan-rich connective tissues throughout the body. The immune system mistakenly treats cartilage as foreign and produces autoantibodies against type II, IX and XI collagen and against matrilin-1. This autoimmune attack draws in large numbers of inflammatory cells, weakens the cartilage and destroys it irreversibly.
In clinical practice, ear cartilage inflammation (auricular chondritis) is the most common and recognizable feature, seen in more than 85% of patients. The classic picture is a sudden, intensely painful, swollen and bright red ear, involving the whole outer ear but sparing the earlobe, which contains no cartilage. After repeated flares, the ear cartilage is destroyed, shrinks and deforms into a "cauliflower ear." The nose is the next most common site (nasal chondritis), causing pain, nosebleeds and collapse of the nasal septum, which produces the characteristic saddle-nose deformity.
At Haute-Derma, we stress that relapsing polychondritis is about far more than the appearance of the ears or nose. It is a dangerous systemic disease that can attack the cartilage of the airways (trachea and bronchi), the joints, proteoglycan-rich structures of the eye (sclera, cornea), the heart valves and the aortic root, and it can be life-threatening if not actively controlled.
Our Approach to Treating Relapsing Polychondritis
Because RP can be so destructive to cartilage, Haute-Derma takes a comprehensive, multidisciplinary approach:
- Stopping flares early to save cartilage: our first priority is to shut down the autoimmune inflammation at the very start of a flare, preventing cartilage breakdown and tissue necrosis and preserving as much intact structure as possible.
- Close screening for respiratory and cardiovascular complications: we set up detailed lung function monitoring and regular cardiac imaging to catch tracheal cartilage damage or aortic aneurysm and dissection early, keeping patients as safe as possible.
- Personalized targeted biologic therapy: we use modern immunosuppressive regimens that act directly on key inflammatory cytokines (such as TNF-alpha and IL-6), giving long-term control while keeping the side effects of background medicines to a minimum.
Diagnosing Relapsing Polychondritis at Haute-Derma
No single blood test is fully specific for RP, so at Haute-Derma we follow the McAdam criteria (as modified by Damiani and Levine), which require clinical and laboratory findings to fit together:
Clinical Examination and the Six Key Features
The doctor carefully assesses whether the patient meets at least 3 of these 6 clinical criteria:
- Recurrent inflammation of the cartilage of both ears (earlobes spared).
- Seronegative, non-erosive polyarthritis (symmetrical pain in large and small joints).
- Nasal chondritis leading to septal collapse and a saddle nose.
- Eye inflammation (scleritis, uveitis, conjunctivitis).
- Respiratory tract chondritis (laryngeal, tracheal or bronchial cartilage).
- Inner ear (cochlear and vestibular) damage: sudden sensorineural hearing loss, tinnitus, vertigo.
Laboratory and Immunology Tests
- Systemic inflammatory markers: erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) rise markedly during acute flares.
- Autoantibody testing: blood testing for anti-type II collagen antibodies (positive in about 50% of patients during the acute phase). ANA, RF and ANCA are checked to rule out coexisting vasculitis or lupus.
Imaging and Specialized Function Tests
- Low-dose chest CT (or MRI): assesses thickening, calcification or collapse of the tracheobronchial cartilage. Scans are taken on both inspiration and expiration to detect dynamic airway collapse.
- Echocardiography and vascular Doppler: screen for aortic or mitral valve regurgitation and aortic root dilation.
- Pulmonary function tests (PFTs): flow–volume loops help detect central airway obstruction early.
Cartilage Biopsy (When Needed)
If the clinical picture is atypical, a small biopsy of ear cartilage is taken:
- Histology: shows loss of cartilage basophilia from proteoglycan breakdown, with a dense infiltrate of inflammatory cells (lymphocytes, plasma cells, neutrophils) around the perichondrium. In severe cases, fibrous tissue replaces the necrotic cartilage.
How Relapsing Polychondritis Is Treated at Haute-Derma
RP treatment at Haute-Derma is tailored to how severely the organs are affected, distinguishing milder disease limited to the ears, nose or joints from severe, life-threatening involvement of the airways or heart:
Systemic Therapy (Controlling the Immune System)
- First-line anti-inflammatory treatment: for mild to moderate flares of ear or joint inflammation, nonsteroidal anti-inflammatory drugs (NSAIDs) combined with low-dose oral corticosteroids quickly relieve pain.
- High-dose corticosteroids: essential in severe flares involving the eyes, airway cartilage or blood vessels. The doctor may give high-dose intravenous methylprednisolone (pulse therapy) for the first 3 days, then switch to oral maintenance and taper according to clinical response.
- Conventional immunosuppressants (DMARDs): to reduce reliance on corticosteroids and prevent relapses, drugs such as methotrexate, azathioprine, cyclosporine A or mycophenolate mofetil are added early. Cyclophosphamide is preferred for systemic vasculitis or sight-threatening eye disease.
Targeted Biologic Therapy
When standard immunosuppressants are not enough, Haute-Derma moves to biologic treatment:
- TNF-alpha inhibitors (such as infliximab, adalimumab, etanercept): highly effective at shutting down the inflammatory cascade, quickly reducing redness and swelling of ear and nose cartilage and protecting it from further destruction.
- Interleukin-6 blockers (tocilizumab) or anti-CD20 therapy (rituximab): used successfully in treatment-resistant RP to stabilize tracheal cartilage and prevent dangerous airway collapse.
Surgical and Supportive Care
- If tracheal cartilage collapses and causes acute airway narrowing, we coordinate with partner hospitals for airway stenting or emergency tracheostomy. Cosmetic reconstruction (saddle-nose repair, ear reconstruction) is only considered once the disease has been in full remission and stable on maintenance therapy for at least 1–2 years.
