Posters

Management of spontaneous bilateral ulcer perforation in patient with Sjogren's Syndrome secondary to primary biliary cirrhosis

Poster Details

First Author: A.Lo Cascio SPAIN

Co Author(s):    N. Saenz Madrazo   A. Baeza Autillo                 

Abstract Details

Purpose:

Describe an aggressive case of corneal melting with the consequent risk of spontaneous ocular perforation as an ocular manifestation of a systemic disease such as primary biliary cirrhosis (PBC) associated with Sjögren's syndrome (SS). PBC is a chronic cholestatic autoimmune disease that presents keratoconjunctivitis sicca in a high percentage of patients. Severe cases of corneal melting have been described especially when the diagnosis is delayed

Setting:

The follow-up was performed in the cornea department of the Gregorio Marañon hospital in Madrid, Spain

Methods:

A descriptive case report study was carried out where the patient was followed for 1 year at the cornea department. Clinical data and the response to treatment were evaluated in conjunction with other services such as internal medicine, immunology and rheumatology

Results:

An 80-year-old male, with a history of PBC and chronic liver disease, is currently undergoing treatment with ursodeoxycholic acid, who comes to the emergency department with a corneal abscess on the right eye (RE). At the slit lamp, corneal edema and 4x4mm abscess were observed, associating central descematocele and hypothalamia. The contralateral eye was normal. Systemic antibiotics and strengthened antibiotic ophthalmic solutions are started. Due to the progressive worsening, surgical treatment was indicated, with a penetrating keratoplasty in the RE. The postoperative period is favorable and the crops are sterile. A month later, the appearance of the graft got worse, presenting important melting and the evolution towards to an ulcer in the central area, in addition to another ulcer on the left eye (LE) with significant thinning of the corneal stroma. Positive results were obtained for SS-A/SS-B and SCL70 antibodies, in addition to those of PBC (AMA type M2, ANA) and confirmatory results of SS on salivary scintigraphy. The use of RGTA OTR4120 (Cacicol®) was necessary in addition to anticolagenolytic agents for adequate control of the corneal lysis. Also, it received an amniotic membrane graft on both eye in 2 surgical times. However, the left eye presented a spontaneous perforated ulcer that resulted in another surgical intervention and initiation of immunomodulatory treatment for adequate long-term control

Conclusions:

Patients with PBC who also associate SS are predisposed to the appearance of corneal ulcers that are difficult to control. The use of anticolagenolytic agents plus amniotic membran are a useful option in disorders of ocular surface. A multidisciplinary management is necessary to pause corneal lysis and to stimulate its epithelialization

Financial Disclosure:

None