Relapsing polychondritis (RP) is a rare autoimmune condition characterized by recurrent episodes of cartilaginous inflammation of the auricular, nasal, and tracheal cartilages.
Jaksh-Wartenhorst described the first case in 1923. A 32-year-old patient presented with fever, pain, and swelling of the ears and later developed stenosis of the external auditory canal and saddle nose deformity. A biopsy of the nasal cartilage showed the absence of cartilage. The term relapsing polychondritis was coined by Pearson and his coworkers in 1960. This activity reviews the cause, pathophysiology, and presentation of relapsing polychondritis and highlights the role of the interprofessional team in its management.
Objectives:
Relapsing polychondritis (RP) is a rare autoimmune condition characterized by recurrent episodes of cartilaginous inflammation of the auricular, nasal, and tracheal cartilages. Jaksh-Wartenhorst described the first case in 1923, when a 32-year-old patient presented with fever, pain, and swelling of the ears and later developed stenosis of the external auditory canal and saddle nose deformity. A biopsy of the nasal cartilage showed the absence of cartilage. The term relapsing polychondritis was coined by Pearson and his coworkers in 1960. They recognized it as an inflammatory condition of the cartilaginous and noncartilaginous structures.
The etiology of relapsing polychondritis is not known. It is suggested to occur in a genetically predisposed individual on exposure to a triggering factor. The triggering factor may be an infectious agent, chemical, toxic exposure, or direct trauma. Relapsing polychondritis cases have been reported after trauma to the pinna. A possible explanation includes cryptogenic antigenic release after trauma and the immune system’s recognition of the antigen. Genetic studies have shown an association between the HLA-DR4 antigen and relapsing polychondritis.
It is a rare disease, more common in Caucasians; the prevalence is 4.5 cases per million. The peak age of onset is between 40 and 50 years, though it can occur at any age. It occurs with equal frequency in both sexes and all racial groups. Over 30% of cases are associated with an existing autoimmune or hematologic comorbidity.
Relapsing polychondritis primarily involves cartilaginous structures, suggesting that immune mechanisms are acting against proteoglycans—the main constituents of cartilage. Circulating and tissue-specific antibodies against collagen types II, IX, and XI are seen in patients with relapsing polychondritis. In addition to humoral immunity, cellular immunity propagates inflammation of the cartilage.
CD4+ cells secrete cytokines such as interleukin-8, macrophage inflammatory protein 1β, and monocyte chemoattractant protein-1, which recruit monocytes and macrophages. Macrophages release proteolytic enzymes, metalloproteinase (MMP-3), cathepsin L and K, which lead to the destruction of cartilage. In the early stages of disease, there is polymorphic inflammatory cell infiltration; later, chondrocyte apoptosis, focal calcification, and cartilage fibrosis.
The clinical spectrum of relapsing polychondritis varies with disease duration and severity. Auricular involvement is present in 90% of cases, and inflammation is restricted to the cartilaginous portion of the ear, with relative sparing of the ear lobes. Patients most frequently present with ear discoloration and pain. Articular involvement is the second most common manifestation and is present in 50% to 75% of patients. Wrist, metacarpophalangeal, and proximal interphalangeal joints are commonly involved.
Ocular manifestations occur in 20% to 60% of patients with relapsing polychondritis and include episcleritis, scleritis, keratitis, and uveitis. Nasal chondritis occurs in about 25% of cases. Inflammation of the cartilaginous rings around the trachea and bronchi leads to collapse of these airways (tracheobronchomalacia). Such patients present with cough, hoarseness, and wheezing. Respiratory compromise is the most frequent cause of death in these patients.
Diagnosis of relapsing polychondritis is primarily based on a combination of clinical features, radiographic findings, and biopsy of a cartilaginous site. It is guided by a set of clinical criteria suggested by McAdams et al. Three out of six criteria are required to make the diagnosis. These include:
Any patient suspected of having relapsing polychondritis should have a dynamic exploratory CT scan to evaluate for airway involvement. CT scans findings include functional airway abnormalities such as air trapping and collapse. Additional findings include thickening of the airway wall and luminal narrowing. Pulmonary function testing is performed to evaluate airway trapping further and assess lung volumes. PET-CT is a new diagnostic modality that aids in early disease recognition and provides a site for targeted biopsy.
A biopsy of the auricular cartilage is performed to confirm the diagnosis of polychondritis. Positive serologic testing for rheumatoid factor and anti-nuclear antibody may be seen in patients with other associated rheumatologic diseases. Complement levels are usually normal in patients with relapsing polychondritis. Radiographic findings can include non-erosive juxta-articular osteopenia and uniform joint-space narrowing; these findings are suggestive of arthropathy in relapsing polychondritis. Anti-type II collagen antibody tests are not routinely available, and when they are performed, antibodies are not present in all cases. There are no lab markers to assess for ongoing cartilage damage.
Due to the rarity of the disease, there is no standardized treatment approach. The clinical presentation and disease severity guide treatment.
For patients presenting with auricular, nasal, or articular involvement without systemic involvement, treatment includes anti-inflammatory medications, colchicine, or dapsone. Low-dose glucocorticoid therapy is often required.
For patients with a more severe presentation, such as large airway involvement (e.g., laryngeal or tracheobronchial chondritis), abrupt-onset sensorineural hearing loss, or ocular involvement, glucocorticoid therapy with IV methylprednisolone 1 gram for 3 days followed by oral prednisone 1 mg/kg, along with immunosuppressive therapy, is indicated. The most commonly used immunosuppressive agents include cyclophosphamide, methotrexate, azathioprine, and cyclosporine. Cyclophosphamide is commonly used as the initial medication (1-2 mg/kg); after remission is achieved, it is switched to other, less toxic immunosuppressive agents, such as azathioprine or methotrexate.
Biologics have been used to treat relapsing polychondritis. The most commonly used biologic is the TNF-alpha inhibitor infliximab. Other biologics that have been tried with variable results include adalimumab, etanercept, abatacept, and tocilizumab. Data on rituximab have not shown success, and this agent is not recommended as the first-line biologic agent.
Surgical interventions such as stenting, airway dilation, tracheostomy, and laryngotracheal reconstruction are needed in cases of airway collapse.
The diagnosis of RP may be challenging. Nasal damage may occur as a result of drug abuse (cocaine), infections from fungi, tuberculosis, or syphilis, and granulomatous conditions such as ANCA-associated vasculitis and lymphomatoid granulomatosis. Eye involvement seen in relapsing polychondritis may also occur with rheumatoid arthritis, ANCA-associated vasculitis, polyarteritis nodosa, Behcet syndrome, or Cogan syndrome.
The outcome of patients with relapsing polychondritis has improved in recent years; survival has increased from 70% at five years to 91% at ten years. The more common clinical presentation is a relatively benign disease, with respiratory failure due to airway collapse being the most common cause of death.
RP is a very rare disorder that typically causes inflammation of cartilage-bearing tissues, such as the ear, nose, larynx, and airways. The inflammatory episodes are recurrent and unpredictable. A concomitant autoimmune disease also occurs in more than 30% of patients. The diagnosis is not always easy because there are no specific tests. Even when diagnosed, the treatment is not standardized. The drug treatment is tailored to each patient, and the cornerstone of therapy is the use of glucocorticoids. For patients with severe disease, other immunosuppressants, such as methotrexate and cyclophosphamide, are used.
Because these drugs have potent adverse effects, the roles of pharmacists and nurses are critical. Both these professionals play a role in educating the patient about the signs and symptoms of adverse effects and in ensuring compliance with the medications. Recently, newer biological agents have also been used to manage RP patients with varying results. However, before initiating treatment with these novel agents, the patient needs a thorough workup to ensure they are fit to receive the therapy. Patients experiencing worsening symptoms should be referred to a specialist.
Outcomes
Patients with RP have frequent relapses, and the quality of life is poor. The mortality rate of RP patients is twice that of the general population. Until randomized controlled studies are available, the treatment of RP will remain empirical and based on personal experience. For healthcare workers who have never treated such a patient, referral to a tertiary care center is recommended.
Source:
Chauhan K, Surmachevska N, Hanna A. Relapsing Polychondritis.
StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2026.
Available from: https://www.ncbi.nlm.nih.gov/books/NBK436007/
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