Progression of disease in ocular cicatricial pemphigoid.

Elder, Mark; Bernauer, Wolfgang; Leonard, J.N.; Dart, John K.G.

doi:10.1136/bjo.80.4.292

Cited by 81 publications

(57 citation statements)

References 15 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…92 Use of immunosuppressive therapy has been shown to slow progression of disease in one case series 92 and control of inflammation has been shown to prevent progression in one RCT. 23 With current immunosuppressive regimens, progression of cicatrisation has still been observed in 10-53% of ocular MMP patients [92][93][94] and is more rapid in patients o60 years of age. 95 A subset of MMP patients with ocular involvement have ongoing conjunctival fibrosis without overt clinical signs of inflammation.…”

Section: Use Of Systemic Immunomodulation To Control Immunemediated Imentioning

confidence: 99%

“…42 Clinical signs are illustrated in Figure 8, ocular MMP typically presents with a red eye and persistent conjunctivitis that has not responded to topical therapy, or with cicatricial entropion and trichiasis, that may have failed surgical repair. About 30% of patients present with acute conjunctivitis and limbitis leading to rapidly progressive scarring and surface failure if uncontrolled.…”

Section: Clinical Signs Of Disease Progressionmentioning

confidence: 99%

“…About 30% of patients present with acute conjunctivitis and limbitis leading to rapidly progressive scarring and surface failure if uncontrolled. 42 Similarly, persistent epithelial defect has a poor prognosis occurring in about 20% of patients. The remaining patients present with subacute or low grade chronic inflammation and slowly progressive scarring.…”

Section: Clinical Signs Of Disease Progressionmentioning

confidence: 99%

“…15 The rate of progression of scarring can usually be established by taking a careful history of the onset of symptoms of inflammation and scarring. The scarring may have been noticed by a healthcare professional or as 42 …”

Section: Oculodermal Diseasesmentioning

confidence: 99%

“…Criteria for initiating immunosuppressive therapy About 25% of OcMMP patients do not require immunosuppression 93 as they have few symptoms, limited scarring, mild or no inflammation and slow progression, or are in remission. In end-stage 'burned out' disease, immunosuppression is also unnecessary as eyes are usually comfortable, albeit blind, and treatment only slows scarring without significantly reversing it.…”

Section: Use Of Systemic Immunomodulation To Control Immunemediated Imentioning

confidence: 99%

See 4 more Smart Citations

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

Dart

2017

Eye

Self Cite

View full text Add to dashboard Cite

This review is in two sections. The first section summarises 35 conditions, both common and infrequent, causing cicatrising conjunctivitis. Guidelines for making a diagnosis are given together with the use of diagnostic tests, including direct and indirect immunofluorescence, and their interpretation. The second section evaluates our knowledge of ocular mucous membrane pemphigoid, which is the commonest cause of cicatrizing conjunctivitis in most developed countries. The clinical characteristics, demographics, and clinical signs of the disease are described. This is followed by a review and re-evaluation of the pathogenesis of conjunctival inflammation in mucous membrane pemphigoid (MMP), resulting in a revised hypothesis of the autoimmune mechanisms causing inflammation in ocular MMP. The relationship between inflammation and scarring in MMP conjunctiva is described. Recent research, describing the role of aldehyde dehydrogenase (ALDH) and retinoic acid (RA) in both the initiation and perpetuation of profibrotic activity in MMP conjunctival fibroblasts is summarised and the potential for antifibrotic therapy, using ALDH inhibition, is discussed. The importance of the management of the ocular surface in MMP is briefly summarised. This is followed with the rationale for the use of systemic immunomodulatory therapy, currently the standard of care for patients with active ocular MMP. The evidence for the use of these drugs is summarised and guidelines given for their use. Finally, the areas for research and innovation in the next decade are reviewed including the need for better diagnostics, markers of disease activity, and the potential for biological and topical therapies for both inflammation and scarring.

show abstract