Bilateral, sequential orbital inflammation secondary to relapsing polychondritis

Area of Science:

• Rheumatology
• Ophthalmology

Background:

• Relapsing polychondritis (RPC) is a rare multisystem autoimmune disease characterized by progressive destruction of cartilaginous structures.
• Orbital inflammation is an uncommon but significant manifestation of RPC, potentially leading to vision impairment.

Observation:

• A Caucasian male in his 60s presented with recurrent bilateral sequential orbital inflammatory disease.
• Initial symptoms included subclavian vein periphlebitis/thrombosis and arthropathy, followed by orbital inflammation unresponsive to antibiotics but responsive to oral steroids.
• Subsequent episodes involved auricular inflammation, rash, and recurrent orbital inflammation, again responding to corticosteroids.

Findings:

• The patient met the McAdam diagnostic criteria for relapsing polychondritis.
• The clinical presentation, including inflammatory arthropathy, auricular inflammation, and orbital inflammation, strongly suggested RPC.
• Rapid resolution of inflammatory signs with oral prednisolone supported the diagnosis and guided treatment.

Implications:

• This case underscores the importance of considering RPC in patients with unexplained orbital inflammation, particularly when accompanied by other systemic inflammatory signs.
• Prompt diagnosis and initiation of immunosuppressive therapy, such as corticosteroids and methotrexate, are crucial for managing RPC and preventing irreversible damage.
• The successful management highlights the efficacy of steroid-sparing agents like methotrexate for long-term disease control in RPC.