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Cherry F. Radford, Saaeha Rauz, Valerie P. Saw, Geraint P. Williams, John K. Dart; The Incidence of Cicatrising Conjunctival Disorders in the UK. Invest. Ophthalmol. Vis. Sci. 2011;52(14):1136.
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Cicatrising conjunctivitis (CC) includes a group of rare, potentially sight-threatening disorders for which early diagnosis and appropriate treatment are essential. A study was designed to determine the minimum incidence of CC disorders in the United Kingdom.
Newly diagnosed cases of CC were reported via the British Ophthalmological Surveillance Unit over a twelve month period (2009). Reporting ophthalmologists completed a clinical data sheet for each patient. Patients with uninflamed eyes, a history of trachoma, acute membranous conjunctivitis or trauma were excluded.
There were 124 reports of newly diagnosed CC, but for 13 (11%) data were unavailable and 12 (10%) were duplicate or erroneous reports. 50/82 (61%) were diagnosed as either proven (n=13) or presumed (n=37) ocular mucous membrane pemphigoid (OcMMP), 16/82 (20%) had Stevens-Johnson Syndrome or Toxic Epidermal Necrolysis (SJS/TEN) and 16/82 (20%) had other disorders causing CC, including drug-induced CC (n=3, 4%), ocular rosacea (n=3, 4%), graft-versus-host disease (n=2, 2%), lichen planus (n=2, 2%), adenovirus (n=1, 1%), atopic disease (n=1, 1%), conjunctival intraepithelial neoplasia (n=1, 1%), linear IgA disease (n=1, 1%) and Sjögren’s syndrome (n=1, 1%). The incidence per million was 0.8 for proven/presumed OcMMP, 0.2 for SJS/TEN and 0.2 for other causes of CC. The overall incidence was 1.3, but with geographical variation (0 - 3.6 per million). At diagnosis the median duration of symptoms was 7 days (range 1-120) for SJS / TEN but significantly (p<0.0001) longer for OcMMP (225, 7-3650) and other causes of CC (225, 16-7300). 18/150 (12%) eyes had a visual acuity of less than 6/18 without any other attributable cause. 64/163 (39%) eyes had moderate or severe inflammation necessitating initiation or "step-up" to more potent immunosuppression in 43 (52%) patients. 101/164 (62%) eyes had symblepharon formation and 26/164 (16%) had keratinisation of the ocular surface, but lower fornix depth measurements were often not recorded (48/164, 29% eyes).
Widespread diagnostic delay highlights the need for increasing awareness of CC and for establishing a network of specialist referral centres dedicated to the diagnosis, monitoring and management of this rare group of disorders.
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