Incidence, presenting features, and diagnosis of cicatrising conjunctivitis in the United Kingdom

Abstract

PurposeCicatrising conjunctival disorders are uncommon, and are difficult to diagnose and manage. This study was designed to assess the annual incidence and underlying diagnosis of patients with cicatrising conjunctivitis (CC) within the United Kingdom.MethodsClinical data of newly diagnosed cases of CC were reported via the British Ophthalmological Surveillance Unit at diagnosis and at 12 months follow-up.ResultsA total of 50 (61%) ocular mucous membrane pemphigoid (OcMMP), 16 (20%) Stevens–Johnson syndrome/toxic epidermal necrolysis (SJS-TEN) and 16 (20%) other causes of CC, equating to an incidence of 0.8, 0.2, and 0.2 per million, respectively, were reported. Although diagnosis of SJS-TEN was usually within a median of 7 days of symptom-onset, that for OcMMP and other CC was a median 225 days for both. At diagnosis, 64/163 (39%) eyes had moderate/severe conjunctival inflammation, and 102/164 (62%) had symblepharon formation. Although 43/82 (52%) patients were commenced on immunosuppression or had this therapy modified, at follow-up there was an increase in the number of symblepharon, despite control of inflammation (P<0.001). Mortality only occurred in the SJS-TEN group (4/16 (25%)).ConclusionCC has a substantial morbidity and for non-SJS-TEN causes, diagnosis is frequently delayed. The proportion of patients given immunosuppressive therapy to prevent disease progression may be less than optimal. These data highlight the need for developing patient access to specialist-designated centres with expertise in CC.

The 2016 Bowman Lecture Conjunctival curses: scarring conjunctivitis 30 years on

“…4,12 K Paraneoplastic pemphigoid and pemphigus (PNP) may rarely present in the conjunctiva at the same time as a neoplasm, and before other signs of malignancy. 13…”

“…Ocular surface squamous carcinoma (OSSN) 4 and sebaceous cell carcinoma 121 Rare causes of conjunctival scarring and inflammation indistinguishable clinically from MMP, except that cases are usually unilateral…”

“…Blinding scarring diseases Progressive inflammatory and scarring diseases for which systemic immunomodulation is often needed for control of scarring and inflammation Mucous membrane pemphigoid (MMP) 4 Currently 60% of all CC cases in the UK are caused by MMP which now includes the conditions mucosal dominated epidermolysis bullosa acquisita, linear IgA disease and anti-laminin 332 (formerly anti-epiligrin or anti-laminin 5) pemphigoid 18 Drug-induced progressive conjunctival cicatrisation 17 A rare complication of topical medication, usually for glaucoma. A small subset of patients with these diseases develop autoantibody-positive or negative progressive conjunctival indistinguishable from MMP Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) with progressive scarring 33 Only a small subset of patients with these diseases develop autoantibody-positive or negative progressive conjunctival scarring similar to that in MMP which may continue from the acute episode or develop acutely years later…”

“…But, causes in single clinics are skewed by case selection and referral bias. For this reason, the epidemiology of CC is best described by the study from Radford (2012) 4 in the UK, which lists the causes of cicatrizing conjunctivitis from the only national incidence study to have been reported. This study may have underestimated the incidence of CC by 15%, but the figures for MMP are in line with those reported for that disease from French and German estimates of 1.13 per million in France and 0.87 per million in Germany.…”

“…This study may have underestimated the incidence of CC by 15%, but the figures for MMP are in line with those reported for that disease from French and German estimates of 1.13 per million in France and 0.87 per million in Germany. 4 Table 2 lists 30 causes of CC with references and notes about the associated diseases and the rarity of the conditions. Although not complete, it includes those reported causes for which there is reasonable evidence of causation.…”