Relapsing Polychondritis N045

Current RMA Instruments

Changes from previous Instruments

ICD Coding

• ICD-10-AM Codes: M94.1

Brief description

Relapsing polychondritis is a systemic autoimmune disease characterised by episodic and progressive inflammatory destruction of cartilage and connective tissue throughout the body. The condition most commonly affects the ears, nose, eyes, joints and respiratory tract, but other organs and structures may also be involved. The disease course is variable, with recurrent flares and periods of remission. 

Confirming the diagnosis

Diagnosing relapsing polychondritis involves a clinical presentation suggesting episodic, progressive inflammation affecting cartilaginous and connective-tissue structures throughout the body. This requires clinical assessment of symptoms- typically recurrent ear or nasal chondritis, ocular inflammation, joint symptoms, or respiratory tract involvement- and examination findings consistent with active chondritis or related inflammatory changes. 

To confirm the diagnosis, the clinical features should be supported by appropriate investigations. These may include inflammatory markers, imaging that demonstrates involvement of cartilaginous structures, or biopsy of affected cartilage. Biopsy of auricular cartilage during an active episode may show characteristic histopathology that can support confirmation; however, biopsy from other sites or during inactive phases may be non-diagnostic. 

If the clinical picture is not consistent with the recognised manifestations of relapsing polychondritis, the diagnosis cannot be confirmed. Confirmation of diagnosis and management requires the involvement of a rheumatologist.

Additional diagnoses covered by SOP

• Auricular chondritis
• Costochondritis
• Laryngotracheal chondritis
• Nasal chondritis

Conditions that are excluded from SOP

• Infective chondritis #
• Seronegative arthritis * - other SOPs apply but which one depends on the specific diagnosis

* another SOP applies

^# non-SOP condition

Clinical onset

Clinical onset refers to the earliest time at which symptoms or signs consistent with relapsing polychondritis were first present. Early manifestations- particularly intermittent ear inflammation- may go unrecognised for long periods, and diagnosis is often delayed. Once the diagnosis is established, onset may be backdated to the time earlier characteristic symptoms (e.g. recurrent auricular chondritis, nasal tenderness, ocular inflammation, or joint symptoms) first appeared, provided alternative causes have been excluded. 

Clinical worsening

The only SOP factor for clinical worsening is inability to obtain appropriate clinical management. The course of relapsing polychondritis is unpredictable and may involve episodic flares with progressive tissue damage over time. While no therapy has been conclusively shown to alter the long-term natural history of the disease, appropriate treatment- including immunosuppressive therapy- can suppress clinical activity and reduce the severity of inflammatory episodes. Assessing whether worsening is attributable to inadequate management requires evaluation by a rheumatologist.