Ocular cicatricial pemphigoid (OCP) is a rare autoimmune disorder defined by persistent inflammation and subsequent scarring of the conjunctiva. The conjunctiva is the clear membrane that covers the white part of the eye (the sclera) and lines the inside of the eyelids. This condition is a specific manifestation of mucous membrane pemphigoid (MMP), which can affect various mucous membranes. OCP’s incidence is higher in women, with an onset around 60 years of age.
The disease follows a chronic and progressive course, where the body’s immune system damages the eye’s surface tissues. This process leads to scarring that can have significant consequences for vision if not properly managed. Because its symptoms can mimic other common eye conditions, an accurate diagnosis can be a challenge.
Symptoms and Pathophysiology
The underlying cause of OCP is a misdirected immune response known as a Type II hypersensitivity reaction. The immune system mistakenly produces autoantibodies that attack proteins within the basement membrane zone of the conjunctiva. This zone anchors the top layer of conjunctival cells to the underlying tissue. This autoantibody attack triggers chronic inflammation, which is the direct cause of tissue damage.
This persistent inflammation leads to the destruction of specialized goblet cells, which are responsible for producing the mucous layer of the tear film, contributing to severe dry eye. As the inflammation continues, the body attempts to heal the damaged tissue by forming progressive scar tissue (cicatrization). This scarring is responsible for the disease’s most severe complications.
The initial symptoms of OCP are often nonspecific, resembling chronic conjunctivitis or dry eye syndrome. Patients experience persistent redness, a gritty or burning sensation, excessive tearing, and sensitivity to light. There is usually minimal discharge, which helps differentiate it from infectious causes of conjunctivitis.
As the disease advances, the signs become more distinct and related to progressive scarring. Subconjunctival fibrosis, or scarring beneath the surface, leads to a shortening of the inferior fornix—the pocket between the lower eyelid and the eyeball. A characteristic sign known as symblepharon may appear, which is an adhesion of scar tissue that tethers the eyelid to the eyeball, restricting movement. The scarring can also cause the eyelid margin to turn inward (entropion), forcing the eyelashes against the cornea (trichiasis).
The chronic abrasion from inturned eyelashes and severe dryness can lead to corneal abrasions, ulcers, and the growth of new blood vessels onto the cornea (neovascularization). Ultimately, the cornea may become cloudy or opaque, a process called keratinization, which results from limbal stem cell failure. This corneal opacification is a primary cause of irreversible vision loss in untreated OCP.
The Diagnostic Process
Diagnosing OCP begins with a clinical examination by an ophthalmologist using a slit-lamp microscope. The doctor inspects the ocular surface for chronic inflammation and evidence of scarring. Specific indicators include subepithelial fibrosis, a shortening of the fornices, and the presence of symblepharon. The presence of these findings in a progressive pattern raises strong suspicion for OCP.
While a clinical examination is suggestive, a definitive diagnosis relies on a conjunctival biopsy. A small tissue sample is taken from an inflamed but not yet heavily scarred area and analyzed using direct immunofluorescence (DIF). This test detects autoantibodies (IgG, IgA, or C3) bound to the basement membrane zone. A positive DIF result, showing linear deposits of these antibodies, confirms the diagnosis.
A negative biopsy result does not completely rule out OCP, as a negative finding can occur despite the presence of the disease. In such cases, the diagnosis may be made based on strong clinical evidence and the progressive nature of the scarring. Once confirmed, physicians use a staging system to categorize the extent of scarring, which provides a baseline to monitor disease progression and guide therapy.
Therapeutic Interventions
The goal of treatment for OCP is to control the autoimmune-driven inflammation and halt the progressive cycle of scarring. For active disease, the main approach involves systemic immunosuppression to stop the immune system’s attack on the conjunctiva. Treatment is often initiated with systemic corticosteroids, which act quickly to reduce severe inflammation.
Because long-term use of high-dose steroids carries significant side effects, they are used for short-term control. Patients are then transitioned to steroid-sparing immunosuppressive agents for long-term management, such as methotrexate or mycophenolate mofetil. The selection of an agent depends on disease severity, patient health, and tolerance, with the aim of achieving durable remission.
Topical treatments play a supportive role. Anti-inflammatory eye drops, including topical corticosteroids, can help alleviate surface inflammation and symptoms like redness and irritation. They are not powerful enough on their own to stop the underlying disease process and are used as an adjunct to systemic therapy.
Surgical intervention is reserved for managing complications from scarring and is not a cure. Procedures may correct eyelid malposition (entropion repair) or release scar tissue (symblepharon lysis). Surgery must be delayed until the underlying inflammation is controlled with systemic medication, as operating on an actively inflamed eye can trigger a severe disease exacerbation.
Long-Term Management and Prognosis
Long-term supportive care is aimed at protecting the ocular surface and managing persistent symptoms. A primary part of daily management is aggressive lubrication to combat the severe dry eye that results from the disease. This involves the frequent application of preservative-free artificial tears, lubricating gels, and nighttime ointments.
For more severe ocular surface disease, specialized scleral lenses may be prescribed. These large-diameter, rigid lenses vault over the cornea and rest on the sclera. The space between the lens and cornea is filled with a saline solution, creating a liquid reservoir that bathes the cornea, protecting it from scarred eyelids and improving vision.
Given the chronic and relapsing-remitting nature of OCP, lifelong monitoring by an ophthalmologist is required. These follow-up appointments are used to assess for any signs of renewed inflammation or disease progression, even in stable patients. This allows for prompt adjustments in therapy to prevent further scarring.
The prognosis for OCP has improved significantly with modern systemic immunosuppressive therapies. While there is no cure, early diagnosis and aggressive treatment can often halt the progression of scarring and preserve vision. The outlook is variable, depending on disease severity at diagnosis and the individual’s response to treatment. Some patients may achieve long-term remission, while others require continuous therapy.