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Bilateral episcleritis, erythema nodosum, and atrial fibrillation

Poster Details

First Author: F.Vingopoulos GREECE

Co Author(s):    S. Palioura                    

Abstract Details


To report a case of Polyarteritis Nodosa (PAN) presenting as bilateral episcleritis and interstitial keratitis (IK) along with systemic symptoms and to review the literature on PAN with anterior segment findings. 


Tertiary referral center (Athens Vision Eye Institute) in Athens, Greece


A 35-year old man presented with a two-month history of bilateral episcleritis, skin lesions consistent with erythema nodosum, joint effusions and episodes of prolonged diarrhea and atrial fibrillation. Ophthalmic examination was significant for diffuse injection and nummular corneal stromal infiltrates. The patient underwent an extensive infectious and inflammatory work-up that was negative except for an elevated erythrocyte sedimentation rate and C-reactive protein. Blind biopsies performed during colonoscopy were consistent with polyarteritis nodosa.


Disease control was achieved with systemic prednisone and azathioprine. Upon self-tapering of both medications the patient developed hearing loss and IK recurred. The diagnosis of Cogan’s syndrome/PAN was made and the patient has been on systemic immunosuppression without recurrence. Since PAN generally involves the posterior segment, we performed a literature review on PAN with anterior segment involvement. Only 11 cases were identified, seven of those 11 patients had presented initially only with ocular signs and symptoms and 4 of these 7 patients died due to delayed diagnosis of their systemic disease months after their initial presentation to an ophthalmologist.


Early diagnosis of PAN is crucial, as the five-year mortality rate is close to 90%; upon initiation of systemic immunosuppression the mortality rate drops to 20%. Though PAN manifestations in the anterior segment are rare, we should have a high index of suspicion in cases of bilateral episcleritis and IK.

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