EuCornea Medal Lecture charts recent developments in cicatrising conjunctivitis

Cicatrising conjunctivitis remains one of the most challenging causes of ocular surface disease today, and early diagnosis and treatment are necessary to prevent vision loss or blindness, Prof John Dart, UK, told delegates attending his EuCornea Medal Lecture at the opening ceremony of the 6th EuCornea Congress in Barcelona.

In a broad overview of the incidence, clinical features and treatment options for the disease, Prof Dart said that cicatrising conjunctivitis remains a major therapeutic challenge. Although early diagnosis and treatment are important, most individual clinicians see few cases and therefore may fail to recognise the clinical features in the early stages of disease which may result in delayed or suboptimal treatment.

“It is a rare disease and while it is difficult to identify and treat we can achieve a lot with good surface disease management and with inflammation control. Immunosuppressive therapy improves outcomes in most cases, but scarring progresses in half of patients. Major problems still persist in the areas of diagnosis and therapies for inflammation and scarring,” he said.

The motivation to learn more about cicatrising conjunctivitis stems from the potentially sight-threatening consequences of a missed or late diagnosis, said Prof Dart.

“It is a devastating disease described as an ‘evil curse’ in a recent editorial. Morbidity is due to the chronic discomfort and blindness. Diagnosis may be difficult: in places where trachoma is no longer present, there are over 30 other causes, of which mucous membrane pemphigoid (MMP) is the commonest. The management of the disease is complex and outcomes are generally poor,” he added.

Apart from MMP, Prof Dart said that other causes of either slowly progressive or progressive cicatrising conjunctivitis include atopic keratoconjunctivitis (AKC), Sjögren’s syndrome (SS), ocular rosacea, Stevens-Johnson Syndrome (SJS) and its more severe form, toxic epidermal necrolysis (TEN), drug-induced conjunctival cicatrisation (DICC), graft versus host disease (GVHD), linear IgA disease, epidermolysis bullosa acquisita (EBA), and mucocutaneous paraneoplastic disorders, some of which can be clinically indistinguishable from  ocular MMP.

MMP, which used to be known as cicatricial pemphigoid (CP), is the commonest of the immunobullous disorders with eye involvement, noted Prof Dart. Making the diagnosis of ocular MMP is relatively easy when conjunctival scarring occurs with MMP at other sites, but is difficult when conjunctival scarring is the only presenting sign.

“For such cases we need to differentiate from other causes of conjunctival cicatrisation such as atopic keratoconjunctivitis , drug-induced scarring, neoplasia and rosacea,” he said.

Controlling inflammation is a key part of current therapeutic approaches to cicatrising conjunctivitis, said Prof Dart. “Topical therapy is ineffective so systemic treatment is the standard of care. Systemic treatment controls inflammation in about 90 per cent of cases, but drug side-effects and failures mean that treatment needs to be modified in about in 30 per cent of patients. It is also worth noting that scarring progresses in 50 per cent of patients even with inflammation control,” he said.

The evidence for current therapy in the scientific literature comes from two random controlled trials and 27 cohort studies and case series, said Prof Dart.

•             A full report of Prof Dart’s  lecture will appear in a future  edition of EuroTimes