OCULAR CICATRICIAL PEMPHIGOID
Ocular cicatricial pemphigoid (OCP) is a rare form of mucous membrane pemphigoid (MMP) that affects the conjunctiva, which is the thin, transparent membrane that covers the white part of the eye, also known as the sclera, and lines the eyelids. This progressive autoimmune disease affects the mucous membranes throughout the body, resulting in ulceration and scarring. OCP usually appears in the sixth to eighth decade of life, and rarely in individuals younger than 30. The incidence has been reported to range from between 1 in 12,000 to 1 in 60,000 and women are affected more than men by a ratio of 2 to 1. Approximately 61% of patients with cicatricial pemphigoid have eye involvement. In the early stages, these patients develop recurrent conjunctivitis, with associated tearing, irritation, burning, and occasional mucus drainage. Early treatment is critical since untreated, this disease progresses in up to 75% of patients. Without treatment, OCP can lead to keratinization of the ocular surface and dramatic scarring of the conjunctiva.
WHAT CAUSES OCP?
The exact underlying cause for OCP is unknown, but like other immune conditions, OCP results when the body overproduces antibodies that attack itself. It is possible for an environmental insult or a drug to trigger an immune attack on mucosal tissue in genetically predisposed individuals. However, identifying a precise cause remains challenging.
HOW IS OCP DIAGNOSED?
Diagnosing OCP can be challenging since the clinical course is variable. Sometimes, it starts out slowly and goes into remission, while other cases have rapid onset. In addition, other conditions, such as trauma and chemical burns and infection, can mimic OCP and make diagnosis even more difficult. A thorough history is helpful since it often reveals that other systems, such as the gums, mouth or throat, have been affected. For example, the presence of mouth ulcers, which are often present in early disease, can help identify OCP. This knowledge, combined with inflammation or scarring of the conjunctiva, can help guide diagnosis, which can then be confirmed by conjunctival biopsy.
TREATMENT FOR OCP
Eye drops are typically not enough to treat OCP. While topical therapy can be used as an adjunct for surface disease, it should not be used in place of systemic therapy. Because OCP is a systemic immune disease, it must be managed with systemic immunosuppressant therapy. Without this, scarring will progress and can lead to permanent vision loss. While receiving systemic therapy, patients with OCP need to see an eye doctor regularly to control conjunctival inflammation, prevent conjunctival and corneal scarring, and protect the ocular surface. Artificial tears, steroids, antibiotics, punctual plugs and cyclosporine may be prescribed. Following systemic treatment, which often lasts several years, remission is possible.
CONTACT LENSES FOR OCP
Ocular surface protection is an important goal when treating OCP. However, drops are not usually sufficient to lubricate and protect the ocular surface for these patients. In many cases, a scleral lens should be considered, as these lenses can aid in the visual rehabilitation and ocular surface protection in patients with OCP. Scleral lenses are large-diameter contact lenses that vault over the entire corneal surface and rest on the white part of the eye, known as the sclera. Liquid fills the space between the eye and the back surface of the scleral lens. This liquid bandage protects the cornea from the ongoing mechanical shearing effect of the eyelids that occurs when you blink. It also continuously bathes the sensitive corneal tissue in OCP patients. Such protection can be beneficial in these cases and has been shown to help in both patient comfort and in maintaining the ocular surface. In addition, for people with OCP who also need vision correction, the fluid layer helps smooth out defects, providing a more uniform refractive surface.
If you have OCP, contact our team at ACLI and schedule a consult to determine the most appropriate management strategy for you.
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