Clinical Scorecard: Bilateral Acute Angle Closure as an Uncommon Presentation of Relapsing Polychondritis: A Diagnostic Dilemma

At a Glance

Key Highlights

• RP can present atypically with bilateral angle-closure glaucoma due to supraciliary effusion before classical cartilage inflammation.
• Diagnosis is clinical and challenging without specific laboratory tests; systemic inflammatory signs and cartilage involvement guide diagnosis.
• Management includes systemic corticosteroids and immunosuppressants like azathioprine with close ophthalmic and rheumatologic follow-up.

Guideline-Based Recommendations

Diagnosis

• Consider RP in patients with bilateral angle-closure glaucoma and systemic inflammatory signs even without classical perichondritis.
• Use Modified Michet criteria incorporating auricular chondritis, nasal cartilage inflammation, and laryngotracheal involvement for diagnosis.
• Exclude infectious and other autoimmune causes with comprehensive laboratory and imaging investigations.

Management

• Initiate systemic corticosteroids at 1 mg/kg with gradual tapering based on clinical response.
• Add immunosuppressive agents such as azathioprine (2 mg/kg) for maintenance and relapse prevention.
• Use topical antiglaucoma medications and cycloplegics (e.g., atropine 1%) to manage elevated intraocular pressure.

Monitoring & Follow-up

• Regular ophthalmic evaluation for intraocular pressure, anterior chamber inflammation, and angle status.
• Monitor systemic symptoms including fever, cartilage inflammation, hearing loss, and airway involvement.
• Adjust immunosuppressive therapy based on relapse occurrence and steroid side effects.

Risks

• Relapse of angle-closure glaucoma and anterior uveitis during steroid tapering.
• Potential airway compromise due to laryngotracheal cartilage inflammation.
• Side effects of long-term corticosteroid and immunosuppressive therapy.

Patient & Prescribing Data

Middle-aged adult male with systemic inflammatory symptoms and bilateral angle-closure glaucoma

Initial high-dose corticosteroids effectively reduce ocular and systemic inflammation; azathioprine aids in maintaining remission and preventing relapses.

Clinical Best Practices

• Maintain high suspicion for RP in atypical bilateral angle-closure glaucoma cases with systemic inflammation.
• Perform multidisciplinary evaluation including rheumatology, otolaryngology, and neurology for comprehensive assessment.
• Exclude infectious and other autoimmune etiologies before initiating immunosuppressive therapy.
• Use imaging modalities like ultrasound biomicroscopy and MRI to assess ocular and systemic involvement.
• Taper corticosteroids cautiously to avoid relapse and monitor closely for recurrence of ocular or systemic symptoms.

References

• Relapsing Polychondritis overview
• Ocular involvement in Relapsing Polychondritis
• Case report of RP presenting as acute angle-closure glaucoma
• Modified Michet criteria for RP diagnosis