Ocular involvement in mucous membrane pemphigoid is characterised by chronic cicatrising conjunctivitis and shortening of the conjunctival fornices. Advanced disease eventuates in scarring of the ocular surface leading to impaired vision and blindness. Documentation using established grading methods have a critical role in providing accurate and objective assessments of disease severity and progression.
To conduct a review of the literature to describe and evaluate the grading methods for mucous membrane pemphigoid with ocular involvement.
The literature search was performed using three online databases: MEDLINE, EMBASE and Scopus. The follow search terms were used: [‘Pemphigoid, Benign Mucous Membrane’] in different combinations with [‘clinical assessment tool’, 'conjunctiva', 'cornea', ‘disease severity’, ‘disease scoring’, ‘eye disease’, ‘fornix’, ‘grading’, ‘ocular’, ‘progression’, ‘staging system’]. Articles of all languages were included.
Majority of the methods focused on grading disease severity and progression using permanent damage parameters. Traditional methods included estimation of the percentage horizontal obliteration of the lower fornix, subjective evaluation of subepithelial fibrosis, and the presence of specific clinical signs. Newer methods aimed to overcome identified difficulties by offering novel measuring techniques, accounting for the upper fornix, and introducing custom metric tools. The validation of these methods have been undertaken in various populations. However, recent literature on the ocular surface has defined and demonstrated the importance of having both activity and damage indices, providing a platform for the development of future grading methods.
Several existing and proposed methods for grading ocular involvement in mucous membrane pemphigoid have been described in the literature, each with their own limitations. Further development of grading tools incorporating both activity and damage would facilitate more comprehensive assessments of severity and progression in this devastating group of diseases.