Ocular Cicatricial Pemphigoid

What is Ocular Cicatricial Pemphigoid?

Ocular cicatricial pemphigoid (OCP) is a chronic autoimmune disease that causes relapsing conjunctivitis and, over time, scarring of the conjunctiva. If it is untreated or does not respond to treatment, it can lead to corneal clouding and permanent vision loss.^[1] It is a form of mucous membrane pemphigoid limited to the eyes, and it makes up about 60% to 70% of cases of that disease.^[1]

Common symptoms

The disease usually starts with a red, irritated, chronically inflamed eye that comes and goes. As scarring builds, the eye surface can become very dry, and the scarring can cause eyelid deformities and corneal damage.^{[1, 2]}

• Redness, burning and pain
• Dryness, or excess tearing and discharge
• Sensitivity to light and blurred vision
• Inward-turning lashes or eyelids, and scar bands that tether the eyelid to the eye (symblepharon)^[2]

The disease can also involve the mouth, nose, throat, esophagus, skin and genitals, causing erosions, blisters and narrowing.^{[1, 3, 4]}

Causes and risk factors

The cause is not fully understood. Autoantibodies attack structural proteins that anchor the surface layers of the body's moist linings.^[3] A genetic predisposition and exposure to environmental triggers are suspected.^[2] The disease mainly affects women around 60 years of age, and it is not contagious.^[2]

How it is diagnosed and treated conventionally

Diagnosis rests on an ophthalmologist's examination and a biopsy of the conjunctiva with direct immunofluorescence testing. A negative biopsy does not rule the disease out, and up to 40% of patients have one.^{[2, 5]}

If scarring is active or progressing, systemic immune suppression is needed.^[5] Dapsone is first choice for mild to moderate disease in people without G6PD deficiency, while more severe cases need drugs such as azathioprine, mycophenolate, methotrexate or cyclosporine, and recalcitrant cases may receive cyclophosphamide, rituximab or immune globulin. With long-term systemic therapy, about 90% of cases can be controlled.^[2] European guidelines note the complete lack of high-quality randomized trials.^[6] Dry eye needs constant lubrication, and surgery should be planned only in a quiet phase, since even minor trauma to the conjunctiva can make the disease worse.^[2]

Why Ocular Cicatricial Pemphigoid calls for a whole-person approach

OCP is a disease of the immune system that happens to show itself on the eye, and its treatment involves long-term medicines with their own burdens. Good care therefore looks beyond the eye surface.^{[2, 5]}

Supportive care cannot change the autoimmune process. It can address some of the day-to-day effects.

An autoimmune process

OCP is driven by autoantibodies, and the same process can involve the mouth and other linings.^[3] This is why a team that may include ophthalmology, dermatology and other specialists often manages it.

The dry, painful eye surface

Scarring can severely reduce the eye's tear film. Dry eye syndrome requires constant lubricating medication and, at times, topical anti-inflammatory drugs.^[2]

Other mucous membranes

When the mouth or throat is involved, eating, speaking and swallowing can become difficult.^{[1, 3]} Any new swallowing or breathing problems need medical review.

Long-term medication

Treatment often means years of immune-suppressing drugs, which is why every herb or supplement needs to be reviewed against your medicine list.^{[2, 7]}

Acupuncture as supportive care in Ocular Cicatricial Pemphigoid

What the research shows

No studies of acupuncture in OCP or any pemphigoid disease were found in PubMed. We therefore cannot say that acupuncture helps this condition.

The closest evidence concerns ordinary dry eye, which has different causes. A meta-analysis of 21 trials found acupuncture improved tear break-up time, tear production and symptom scores compared with artificial tears alone.^[8] A second meta-analysis of 16 trials found that adding acupuncture to artificial tears improved tear measures and inflammatory markers, though not the patient-reported Ocular Surface Disease Index.^[9] Another review of 394 patients reached a similar conclusion and reported no severe adverse effects.^[10] A sham-controlled trial of 90 people with dry eye after cataract surgery found modest gains in tear film stability that were not seen in every measure.^[11] These findings come from dry eye without scarring autoimmune disease, and the studies vary in quality. They cannot be assumed to apply to OCP.

How acupuncture may work

One meta-analysis of dry eye trials reported lower levels of the inflammatory molecules IL-6 and TNF-alpha with acupuncture, which suggests that some effect on inflammation is possible.^[9] This has not been tested in OCP.

What treatment involves

Treatment uses fine, sterile, single-use needles, usually left in place for about 20 to 40 minutes. In OCP, needling on or near the eyes and eyelids is best avoided, because the surface of the eye is fragile and minor trauma can make this disease worse.^[2] A short course of visits is followed by reassessment.

Serious problems from acupuncture are rare. In prospective studies, about 9 in 100 patients had a minor reaction such as brief bleeding, soreness or redness at a needle site, and serious events occurred in about 1 in 10,000 patients.^[12] Tell the practitioner first if you have a bleeding disorder, take blood thinners or immune-suppressing medicines, are pregnant, or have a pacemaker (relevant to electroacupuncture).

Ayurvedic medicine as supportive care in Ocular Cicatricial Pemphigoid

The Ayurvedic view

Ayurveda has no classical diagnosis equal to OCP. Chronic red, burning, dry eyes are traditionally linked to aggravated Pitta, sometimes with Vata dryness. This is a traditional framework, not a biomedical diagnosis.

Therapies that may be used

Once you are under specialist care, supportive Ayurvedic care may include:

• Diet and daily routine aimed at steady digestion and sleep, avoiding very spicy or sour foods in a Pitta pattern
• Stress-reducing practices such as breathing exercises and relaxation
• Individually prescribed herbs, chosen with attention to your medicines

Nothing should be applied to or inside the eyes. Panchakarma, including eye procedures such as Netra Tarpana, is not appropriate here: it is not suitable during pregnancy, acute illness or frailty, and the fragile, scarred surface of the eye should not be treated with unregulated substances.

What the research shows

There are no clinical trials of Ayurvedic treatment for OCP or any pemphigoid disease. A review of herbal supplements in autoimmune skin disease concluded there is little to no evidence that they improve these conditions.^[13] Among patients at one inflammatory eye disease clinic, 42% used complementary medicine, and the authors advised physicians to ask about it because of possible effects on the disease and interactions with immunosuppressive treatment.^[14]

Herbal medicine as supportive care in Ocular Cicatricial Pemphigoid

Herbs and formulas that have been studied

No trials of herbal medicine in OCP were found. Herbs are mentioned in traditional practice for dry eyes and inflammation, but no herb has been shown to treat or control this disease.^[13]

What the research shows

Because there are no studies in OCP, we do not claim a benefit. The only relevant evidence is a caution. A review of herbal supplements in autoimmune skin disease found little to no evidence of benefit.^[13]

Safety and herb-drug interactions

Case reports link immunostimulatory herbs such as echinacea, alfalfa, spirulina and chlorella with the onset or flares of immune-driven conditions, including autoimmune blistering disorders.^[13] For that reason, herbs promoted to stimulate the immune system are best avoided.

Herbs can also change levels of immune-suppressing drugs. St John's wort, for example, has lowered blood levels of ciclosporin and tacrolimus, and some herbs affect blood clotting.^[7] Herbal and dietary supplements account for about one fifth of drug-induced liver injury cases in the United States, which matters if your medicines are also processed by the liver.^[15]

About one fifth of Ayurvedic medicines bought online contained detectable lead, mercury or arsenic.^[16] Herbs should come from tested sources. Tell us if you are pregnant or breastfeeding, or have liver or kidney disease.

Translating traditional medicine into modern biological language

This is interpretation, not equivalence. Chinese medicine may describe red, dry, gritty eyes as heat or dryness with deficiency of fluids or yin. Researchers instead describe an autoimmune attack on the conjunctiva, loss of tear film and scarring.^{[2, 3]} In dry eye research, acupuncture has been linked to lower inflammatory markers.^[9]

Ayurveda's aggravated Pitta describes heat and inflammation in general terms. No study has shown that it corresponds to a specific immune process in OCP.

How this care fits with your medical care

OCP needs an ophthalmologist's care and usually systemic treatment. For a condition like this, we ask that you are under a physician's care for it and that your treating team knows you are receiving complementary care. Our role is limited to supportive care for comfort, stress and general well-being.

Do not stop or change immune-suppressing drugs, steroids, eye drops or any prescribed treatment without your prescriber. We do not diagnose or manage the disease itself. Please bring your diagnosis, eye examination and biopsy reports, and a full list of medicines and supplements.

When to seek urgent medical care

Sudden changes in the eye or in other mucous membranes need prompt medical review.

• Sudden loss or worsening of vision
• Severe eye pain, a very red eye, or a white or cloudy spot on the cornea
• New or worsening light sensitivity or discharge
• Trouble swallowing, hoarseness or noisy breathing, which may signal throat or airway involvement^[3]
• Fever or signs of infection while taking immune-suppressing drugs

If any of these occur, call 911 or go to the nearest emergency department.