To report a case of relapsing polychondritis with the involvement of multiple parts of the eye as keratitis, pan uveitis, disk swelling and retinal vasculitis simultaneously and bilaterally

A 26-year-old female was consulted to our emergency department because of diminishing vision associated with ocular pain.Complete ocular examination , optical coherence tomography ,OCT ,fluorescein angiography, FAG, were performed at baseline and during the treatment.Uveitis work-up was performed

Her initial best corrected visual acuity was 20/500 (+1.4 Log MAR) bilaterally. Ocular movements were normal except for nystagmus that was pendular. The slit lamp exam revealed conjunctival congestion, diffuse nodular patchy keratitis and anterior chamber (AC) inflammation of 3+ cells and 1+ flare. In fundus examination, apart from vitreous opacity, bilateral disc swelling and retinal hemorrhage and whitening and peripheral vasculitis were observed . OCT that has poor quality due to vitritis shows IS/OS junction and COST (cones outer segment tips) line destruction especially in the left eye and disc edema. All of the uveitis work-ups were inconclusive except for pinnal cartilage biopsy that revealed non-localized inflammation and the presence of fibrous tissue instead of cartilage that were pathognomonic of relapsing polychondritis. The patient was treated with high dose oral steroid , cyclophosphamide pulse therapy and azathioprine. After one year follow-up ,systemic and ocular inflammation were in remission.

Considering the fact that relapsing polychondritis is an enigmatic disease with debilitating and even life-threatening consequences, the high suspicious of the disease is of paramount importance in any retinal vasculitis and uveitis. A systemic review has great importance in order to avoid a delayed diagnosis and the subsequent morbidity and mortality.