Relapsing polychondritis (RP) is a rare systemic inflammatory disease that primarily affects cartilaginous structures in the body. Commonly involved organs include the nose, ears, airway, and joints. However, it can also affect organs not rich in cartilage, such as the vasculature, skin, inner ear, and eyes. Due to its rarity and relapsing nature, diagnosing, and treating RP can be challenging, leading to delays in appropriate care. Recently, the disease's phenotypic heterogeneity has been categorized into clinical subgroups based on affected organ systems, such as the auricular, nasal, pulmonary, and hematologic subgroups. These classifications help determine the unique natural history and outcomes associated with these subtypes.

Diagnosing RP relies on clinician knowledge and recognition of the pattern of organ involvement, which can be difficult due to the disease's rarity and systemic nature. As a result, patients with RP may initially seek help from various specialists, including rheumatologists, otolaryngologists, and pulmonologists. It's crucial for all specialists to familiarize themselves with the features of RP to ensure timely and accurate diagnoses.

There are currently no FDA-approved therapies for RP, and clinical trials to guide management are limited. Due to variations in disease severity and organ involvement, treatment approaches should be tailored to each patient, considering the specific organs affected and the extent of inflammation.

With this review we hope to provide general knowledge about the disease, organ assessment, and treatment recommendations.