Relapsing polychondritis.

Area of Science:

• Rheumatology
• Immunology
• Rare Diseases

Background:

• Relapsing polychondritis (RP) is a rare autoimmune disorder characterized by recurrent inflammation and destruction of cartilage.
• While chondritis affects cartilage, arthritis is the most common initial presentation, often preceding clear signs of cartilage involvement.
• RP can be associated with other autoimmune diseases, occurring in one-third of patients.

Purpose of the Study:

• To summarize the key aspects of relapsing polychondritis, including its presentation, pathogenesis, diagnostic methods, and treatment strategies.
• To highlight the challenges in diagnosing RP, especially when initial symptoms are non-specific.
• To outline current therapeutic approaches for managing this complex condition.

Main Methods:

• Diagnosis is primarily clinical, supported by criteria such as Michet's criteria.
• Laboratory tests, imaging (CT, MRI, echocardiography), and pulmonary function tests aid in diagnosis and assessment.
• Serological markers like anti-collagen type II and anti-matrilin-1 antibodies lack diagnostic utility.

Main Results:

• Arthritis is the most frequent initial symptom, with over half of patients lacking evidence of chondritis at presentation.
• Autoimmune responses to unidentified cartilage antigens are implicated in RP pathogenesis.
• Effective management involves tailored treatment based on disease activity and severity.

Conclusions:

• Relapsing polychondritis requires a high index of suspicion, particularly in patients with unexplained polyarthritis or other systemic manifestations.
• Current diagnostic approaches rely on clinical evaluation and supportive investigations, as specific biomarkers are lacking.
• Glucocorticoids form the mainstay of RP treatment, with immunosuppressive agents used for severe or refractory cases.