# Management of Spontaneous Corneal Perforation in Undiagnosed Primary Sjögren’s Syndrome

## TL;DR

A case study shows how a rare eye condition revealed an autoimmune disease, emphasizing the need for early diagnosis and teamwork in treatment.

## Contribution

This case highlights spontaneous corneal perforation as a rare manifestation of pSS and the effectiveness of a multidisciplinary treatment approach.

## Key findings

- Spontaneous corneal perforation was diagnosed as linked to primary Sjögren’s syndrome.
- Treatment with corticosteroids and immunosuppressants led to a favorable outcome.
- Early diagnosis and multidisciplinary care are crucial for managing pSS-related complications.

## Abstract

Spontaneous corneal perforation is a true diagnostic and therapeutic emergency that can reveal autoimmune diseases such as primary Gougerot-Sjögren syndrome (pSS). We report the case of a 59-year-old female patient with no notable medical history who presented with a painful unilateral red eye and decreased visual acuity. Examination revealed a unilateral paracentral corneal perforation, which was subsequently confirmed to be associated with pSS based on the positivity of anti-SSA/SSB antibodies and a minor salivary gland biopsy showing lymphocytic infiltration, without any signs suggestive of an associated systemic disease. The patient underwent conjunctival covering and received systemic treatment with corticosteroids and immunosuppressants, leading to a favorable clinical outcome. This case highlights the importance of early diagnosis and a multidisciplinary approach to prevent severe complications associated with pSS.