Cicatricial Pemphigoid (CP) is a distinct disease entity of unknown but presumed autoimmune cause. Its effect on the eyes is characterized by chronic cicatrizing conjunctivitis, progressive conjunctival subepithelial fibrosis, and resultant fornix foreshortening, symblepharon formation, trichiasis, distichiasis, meibomian duct obstruction, and eventual lacrimal duct compromise with reduced tear supply to the ocular surface. It is of substantial ophthalmologic interest because of its bilaterality, and because it eventually results in legal blindness due to corneal scarring in a high proportion of people with the disease. No topical therapy is effective in stopping the progression of scarring of the conjunctiva. High doses of systemic prednisone can control the process, but the long-term consequences of moderate-to-high doses of systemic steroid required for control of CP are unacceptable. Systemic immunosuppressive therapy, alone or in combination with systemic dapsone, is highly effective in controlling the disease, and may cure it in many instances.These clinical characteristics coupled with the ocular and the systemic immunopathologic correlates in patients with CP show it to be a systemic disease, and most evidence strongly suggests it is caused or propagated by a defect in immunoregulation.CP has been one of the main interests of Dr. Foster's research group. Struck by the catastrophic consequences of this disease, the total lack of efficacy of conventional therapy, and the circumstantial evidence suggesting that CP is an autoimmune disorder, they made a major commitment to the care and study of patients with CP. The studies have been both clinical and basic, and the care has involved personal responsibility not only for the ocular therapy but also for the systemic management and monitoring of the patients.The purpose of this book is to describe the results of their experience with 130 patients who have CP.

Cicatricial Pemphigoid

Medical Books

Foster, C. Stephen

Medicine

OIUF

This book arises from the efforts of many doctors and patients alike, all who agreed to travel to Cambridge, Massachusetts (including some from long distances and foreign lands) to attend a unique one day meeting dedicated to the subject of Birdshot Retinochoroidopathy (BSRC). The meeting, sponsored by the Ocular Immunology and the Uveitis Foundation (OIUF) and the Massachusetts Eye Research and Surgery Institution (MERSI) and by generous, philanthropic donors, had as its audience ophthalmologists and patients with BSRC.Through the publication of this title, the hope is twofold: 1)To bring to the attention of the ophthalmic community the essentials of this rare, blinding disorder, and 2)To share a consensus on the matters of diagnosis, current best treatment, and recommended longitudinal monitoring methods.Additional goals include the stimulation of change in practice patterns which will reduce the prevalence of visual disability secondary to BSRC and to encourage further research on the causes of the disease and its cure.

Birdshot Retinochoroidopathy: Volume 1

The purpose of this book is to provide a concise but precise consensus on the impact of uveitis in children and on the diagnostic and therapeutic approaches. Experts in Ocular Immunology and Rheumatology were convened in an effort to provide guidelines in the diagnosis and treatment of uveitis in children. The result of those efforts are captured in this monograph.

Childhood Uveitis

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Books by OIUF

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Cicatricial Pemphigoid

Birdshot Retinochoroidopathy: Volume 1

Childhood Uveitis