Relapsing polychondritis presenting as bilateral acute angle closure: an unusual diagnostic challenge

To report a rare case of relapsing polychondritis (RP) presenting initially as bilateral angle-closure glaucoma due to supraciliary effusion, highlighting the diagnostic challenges when classical features like perichondritis appear late.

A 54-year-old male presented with bilateral eye pain, redness, and elevated intraocular pressure. Clinically there was presence of bilateral angle closure glaucoma and peripheral choroidal detachment in left eye. Ultrasound biomicroscopy revealed bilateral supraciliary effusion. Systemic symptoms including intermittent fever, sensorineural hearing loss, nasal tenderness, and hoarseness of voice were noted. Infectious and autoimmune evaluations were inconclusive. Indocyanine green angiography excluded Vogt-Koyanagi-Harada syndrome. The patient was managed with corticosteroids and antiglaucoma medications. Later development of auricular chondritis confirmed the diagnosis of RP using Modified Michet's criteria.

The patient experienced recurrent bilateral angle closure and anterior uveitis despite immunosuppression. Classical signs of RP such as perichondritis and laryngotracheal involvement manifested late, allowing retrospective correlation of earlier ocular and systemic signs. The patient showed clinical improvement with high-dose corticosteroids and azathioprine. Multidisciplinary collaboration was essential in establishing the diagnosis and guiding management.

This case expands the clinical spectrum of RP by presenting with bilateral angle-closure glaucoma prior to typical cartilage involvement. It emphasizes the importance of considering RP in patients with unexplained ocular inflammation and cartilage-related systemic symptoms. Early recognition, even in the absence of overt perichondritis, is key to timely diagnosis and effective management. A multidisciplinary approach remains crucial in addressing the systemic and relapsing nature of RP.