Autoimmune disease that causes blindness

Contents

Vision Problems and Autoimmune Disorders

Autoimmune disorders can affect every inch of your body, from your nerves and joints to your eyes.

But with more than 80 different autoimmune disorders in existence, it is difficult to catalog all the possible impacts on your vision.

You need to be informed about the risks that your specific autoimmune disorder poses to your vision and to seek assistance if you start to experience changes, such as cloudiness, blurriness, pain, dryness, or light sensitivity.

The impact of autoimmune disorders on your eyes can be lessened with appropriate medication.

“There are several areas of the eye that are involved in autoimmune disease,” says ophthalmologist Alan H. Friedman, MD, clinical professor of ophthalmology and pathology at the Mount Sinai School of Medicine in New York City. “There are probably common building blocks, like proteins and sugars, that are involved, so that when one area of your body is affected, your eye can be affected also.”

On such problem is autoimmune retinopathy, which is “usually manifested as a vascular problem,” says Dr. Friedman. This means that the immune system attacks and inflames the blood vessels in the back of the eye, on the retina, which can affect vision.

Autoimmune Disorders: See Your Eye Doctor Regularly

One of the most important things you can do to protect your eyesight is to see your ophthalmologist regularly. The American Academy of Ophthalmology, a professional organization for eye doctors, advises all adults to get an eye screening at age 40 if they have not seen an eye doctor previously. If you have an autoimmune disease:

  • You should not wait until age 40 for a vision health screening. See an ophthalmologist now if you have not been to one in the past year.
  • Make sure you ask your eye doctor about how often you need to be screened.
  • Get screened more frequently (even as much as every six months) if you have symptoms of vision health problems.
  • You should also get frequent eye exams if you are taking certain autoimmune disorder medications for treatment, such as Plaquenil (hydroxchloroquine), which is known to affect eyesight.

Autoimmune Disorders Linked to Vision Health

There are many autoimmune disorders that affect the eye, including:

  • Behcet disease. This rare autoimmune disorder is a leading cause of blindness in some developing countries. Eye symptoms may be accompanied by mouth and genital sores.
  • Lupus. Eye inflammation is one of the many possible effects of lupus. Symptoms include blurred vision, headaches, sore eyes, dry eyes, and sensitivity to light.
  • Multiple sclerosis (MS). The connection between vision health and this autoimmune disorder is so strong that eye doctors may be the first ones to suspect an autoimmune disorder, says John Rose, MD, director of the Center for Autoimmune Disease Research at Johns Hopkins University in Baltimore. Initial symptoms of MS often include optic neuritis, which is a gradual or sudden loss of vision due to inflammation of the optic nerve – the large nerve entering the back of the eye.
  • Psoriasis. Inflammation of the conjunctiva (the clear membrane covering the undersides of your lids and the whites of your eye) can cause redness and pain in your eye.
  • Reiter’s syndrome. In addition to causing joint inflammation, reactive arthritis can cause inflammation of the front part of your eye.
  • Rheumatoid arthritis (RA). RA can cause dryness, inflammation of the white of the eye, thinning of the cornea, and other painful ocular conditions.
  • Sjogren’s Syndrome. This chronic inflammatory condition can lead to dryness of the eye due to an autoimmune attack on the tear-producing glands.
  • Thyroid diseases. Autoimmune disorders that result in either high or low thyroid function increase the risk of glaucoma (a condition of high pressure inside the eye). Research to understand this relationship is ongoing, but it is thought that with hyperthyroid conditions such as Graves’ disease, tissues build up around the eye and increase pressure in that way. With low-thyroid disorders, the eye may not be able to circulate its fluids effectively, causing pressure to build up from the inside.
  • Type 1 diabetes. This autoimmune disorder is one of the leading causes of blindness in the United States, because chronic poor blood sugar control can cause damage to the small blood vessels in the back of the eyes.
  • Ulcerative colitis and Crohn’s disease. About 5 percent of patients will have “nonspecific inflammation of the eye.”
  • Uveitis. This is an autoimmune disorder that directly affects the pigmented cells of the iris in the eye, and sometimes the middle layers of the eye as well. It causes inflammation, which can lead to blurred vision, “floaters,” and redness of the eye. Uveitis can occur alone, or may be a symptom of another autoimmune disorder.

Vision Health and Autoimmune Disorder Treatments

Some medications for autoimmune disorders may have adverse effects on your vision health over the long term, including:

  • Corticosteroids. Long-term use can cause cataracts and increase the risk of glaucoma.
  • Antimalarials. Plaquenil can occasionally cause decreased vision.

If you have an autoimmune disease, you are probably alert to changes throughout your body — but don’t forget to take care of your eyes with regular screenings and attention to vision changes.

Glossary of Eye Conditions

Suggested resources:

Hyperopia

This common vision problem, also known as farsightedness, occurs when light rays entering the eye focus behind the retina, not directly on it. People with hyperopia are usually able to see distant objects well, but close objects appear blurry. Hyperopia may cause eyestrain or headaches, especially with reading. Eyeglasses or contact lenses can correct hyperopia. For people who do not want to wear glasses or contact lenses, laser vision correction is sometimes possible.

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Keratoconus

Rare condition, often inherited, in which the cornea becomes progressively thinner and gradually bulges outward, causing blurred or distorted vision. Keratoconus usually affects both eyes. At first, people with this condition can correct their sight with eyeglasses. However, as symptoms worsen over time, specially designed contact lenses are needed to improve vision. Most people with keratoconus will not experience severe visual impairment. However, as many as one in five will eventually require a corneal transplant (surgical replacement of the old cornea with a new one).

Suggested resource: www.nkcf.org

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Late-Onset Retinal Degeneration (L-ORD) A genetic retinal disorder. Onset typically occurs in the fifth to sixth decade of a person’s life. Symptoms include night blindness, progressing to severe central and peripheral degeneration with choroidal neovascularization and chorioretinal atrophy. Because it is late onset, it is often mistaken for regular macular degeneration. Note, however, that this disorder, in its severest state, affects both central and peripheral vision and thus leads to total blindness, unlike some of the more common macular disorders. Laurence-Moon-Bardet-Biedl Syndrome

Rare, inherited disorder affecting many parts of the body. People with this condition have retinitis pigmentosa accompanied by mental retardation, paralysis of the legs, and various other symptoms.

Suggested resource: http://rarediseases.about.com/od/rarediseasesl/a/lmbbs.htm

Leber’s congenital amaurosis

Inherited condition, probably caused by degeneration of the retina, in which an infant is born blind or develops severe vision loss soon after birth. Children with Leber’s congenital amaurosis typically also have nystagmus, and some also have mental retardation and hearing disorders. At present, there is no treatment for this condition.

Suggested resources:
Foundation for Retinal Research

Legal blindness A level of visual impairment that has been defined by law to determine eligibility for benefits. It refers to central visual acuity of 20/200 or less in the better eye with the best possible correction, or a visual field of 20 degrees or less. Low vision

Vision loss that may be severe enough to impede a person’s ability to carry on everyday activities, but still allows some functionally useful sight. Low vision may be caused by macular degeneration, cataracts, glaucoma, or other eye conditions or diseases. Low vision may range from moderate impairment to near-total blindness. It cannot be fully corrected by eyeglasses, contact lenses, or surgery. However, a person with low vision may benefit from any of a variety of available optical devices, such as electronic magnifying glasses or eyeglass-mounted telescopes. In addition, special software developed for computer users with low vision can display type in large size or read text aloud.

Suggested resource: https://www.aao.org/eye-health/diseases/low-vision
Getting Started Kit for People New to Vision Loss

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Macular degeneration

Disease that causes dysfunction of the macula, the area in the middle of the retina that makes possible the sharp central vision needed for such everyday activities as reading, driving, and recognizing faces and colors. The condition is commonly known as age-related macular degeneration (AMD) and is the leading cause of visual impairment among older people. However, there are also other types of macular degeneration, such as Stargardt’s Disease and Best’s Disease. Macular degeneration causes blurred, distorted, or dim vision or a blind spot in the center of the visual field. Peripheral vision is generally not affected. This condition is painless and may progress so gradually that the affected person at first notices little change. There is no cure for macular degeneration, but drug therapy, laser surgery, or other medical treatment may in some cases be able to slow the disease’s progression or prevent further vision loss. People with macular degeneration can also benefit from the use of various devices for low vision, such as magnifiers, high-intensity lamps, and pocket-sized telescopes.

Suggested resources:
Age-related Macular Degeneration (AMD)

Macular hole

A macular hole is a full thickness hole in the central part of the retina called the macula. It may be caused by injury or inflammatory swelling of the retina, but most commonly occurs as an age-related event without any predisposing conditions. Macular holes are thought to be caused by tractional forces associated with the vitreous gel separating from the retina in the macula and around the central macula called the fovea. Surgery is the treatment of choice for full-thickness macular holes.

Suggested resources:
Macular Hole

Marfan Syndrome

Disorder of the connective tissue, affecting the heart and blood vessels, skeletal system, eyes, and other parts of the body. The condition is present at birth. Symptoms vary from person to person, ranging from mild to severe. People with Marfan syndrome are often nearsighted (see myopia), and about half have dislocation of one or both lenses of the eye. There is no cure for Marfan syndrome. Treatment depends on which body systems are affected. Early eye examinations can detect vision problems related to the disorder, which can usually be corrected with eyeglasses, contact lenses or eye surgery.

Suggested resource: www.marfan.org

Microphthalmia

Rare disorder, usually inherited, in which one or both eyes are abnormally small. The degree of visual impairment varies, from reduced vision to blindness. Extreme microphthalmia resembles some forms of anophthalmia. There is no treatment or cure for microphthalmia. In certain cases, artificial eyes can be used to promote proper growth of the eye sockets and to help with cosmetic appearance.

Suggested resource: http://www.nei.nih.gov/health/anoph/

Myopia

This condition, commonly known as nearsightedness, occurs when light rays entering the eye focus in front of the retina, not directly on it. People with myopia are usually able to see close objects well, but objects in the distance—such as highway signs or writing on a chalkboard—appear blurred. People with this condition may squint to see distant objects and experience eyestrain or, sometimes, headaches. Eyeglasses or contact lenses can correct myopia. Surgery is another alternative.

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Neurological visual impairment (NVI)

See cortical visual impairment.

Suggested resource: www.sfsu.edu/~cadbs/Eng022.html

Neuromyelitis optica (NMO) Neuromyelitis optica (NMO), also known as Devic’s disease, is an autoimmune disorder in which immune system cells and antibodies mistakenly attack and destroy myelin cells in the optic nerves (neuritis) and the spinal cord (myelitis). NMO leads to loss of myelin, which is a fatty substance that surrounds nerve fibers and helps nerve signals move from cell to cell. The syndrome can cause blindness in one or both eyes and can be followed by varying degrees of paralysis in the arms and legs. Most individuals with the syndrome experience clusters of attacks months or years apart, followed by partial recovery during periods of remission. The onset of NMO varies from childhood to adulthood, with two peaks, one in childhood and the other in adults in their 40s. The syndrome is sometimes confused with multiple sclerosis (MS) because both can cause attacks of optic neuritis and myelitis. Non-24-Hour Sleep-Wake Disorder (Non-24)

Non-24-Hour Sleep-Wake Disorder (Non-24) is a serious, chronic, and rare circadian rhythm disorder that affects a majority of totally blind individuals who lack light perception and cannot reset their master body clocks to the 24-hour day. Non-24 is most commonly found in blind individuals who cannot perceive light, the primary environmental cue for synchronizing their circadian rhythm to the 24-hour day. In the United States, this disorder affects approximately 80,000 totally blind individuals who lack the light sensitivity necessary to reset their internal “body clocks.” In general, individuals with Non-24 suffer from a variety of clinical symptoms as they cycle into and out of phase, resulting in disrupted nighttime sleep patterns and/or excessive daytime sleepiness.

Suggested resources:
FDA Approval for Drug That Regulates Sleep Patterns
Discovering That I Had Non-24

Nystagmus

Condition that involves involuntary, rapid, repetitive movements of one or both eyes from side to side, up and down, or in a circular motion. Nystagmus may be present at birth or, less commonly, may result from disease or injury. In some cases, the condition can reduce or interfere with vision. For example, children with nystagmus may frequently lose their place when reading. Placing a cutout reading window over words or using a card to “underline” text can be helpful.

Suggested resource: www.nystagmus.org

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Optic nerve atrophy Degeneration of the optic nerve, which carries vision information from the eye to the brain. People who have optic nerve atrophy may have dimmed or blurred vision as well as a reduced field of vision. They may also have difficulty seeing contrast and fine detail. Vision loss through optic nerve atrophy is permanent. However, if the underlying cause can be identified and successfully treated, further vision loss may be prevented. Bright lighting, high contrast, and bold colors can help children with optic nerve atrophy see more clearly. Optic nerve hypoplasia

Condition, present at birth, in which the optic nerve is underdeveloped, so that adequate visual information is not carried from the eye to the brain. The effects of optic nerve hypoplasia have a broad range, from little or no visual impairment to near-total blindness. The condition may affect one or both eyes. There is no treatment or cure for optic nerve hypoplasia. However, depending on the degree of visual impairment, a person with this condition may benefit from the use of devices for low vision.

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Presbyopia

The eye’s gradually decreasing ability to focus on nearby objects. Presbyopia is a normal part of aging and affects virtually everyone, usually becoming noticeable after age 40. People with presbyopia typically hold reading materials at arm’s length in order to bring the words into focus. They may experience headaches or eyestrain while reading, viewing a computer screen, or doing close work. Presbyopia can be corrected with reading glasses, bifocal or variable focus lenses, or contact lenses. Using bright, direct light when reading is also helpful.

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Retinal detachment

Separation of the retina from the underlying supportive tissues. Retinal detachment may result from injury, disease, or other causes. A person with retinal detachment usually does not experience pain, but may see floaters (see floaters and spots) or bright flashes of light, may have blurred vision, or may see a shadow or curtain over part of the field of vision. Retinal detachment requires prompt medical attention to prevent permanent vision loss. There are several methods of treatment for retinal detachment, including laser surgery.

Suggested resource: www.nei.nih.gov/health/retinaldetach/index.asp

Retinitis pigmentosa

Degeneration of the retina, resulting in decreased night vision, a gradual loss of peripheral vision, and in some cases, loss of central vision. The degeneration progresses over time and can lead to blindness. Retinitis pigmentosa is a rare, inherited disease for which there is as yet no treatment or cure. Some ophthalmologists believe that treatment with high doses of Vitamin A can slow the progression of retinitis pigmentosa, and that taking Vitamin E makes it worse. Early diagnosis enables a person with the disease to plan and prepare for its progression. In addition, depending on the degree of vision loss, electronic magnifiers, night-vision scopes, and other such special devices for impaired vision can provide some benefit for people with the disease.

Suggested resource:

Retinoblastoma

Malignant tumor (cancer) of the retina, generally affecting children under the age of 6. Usually hereditary, retinoblastoma may affect one or both eyes. Retinoblastoma has a cure rate of over 90 percent if treated early. Without prompt treatment, the cancer can spread to the eye socket, the brain, and elsewhere, and can cause death. Depending on the size and location of the tumor, treatment options include laser surgery, cryotherapy (a freezing treatment), radiation, and chemotherapy. In some cases, the affected eye may need to be removed.

Suggested resource: www.aoa.org/x8066.xml

Retinopathy of prematurity (ROP)

Condition associated with premature birth, in which the growth of normal blood vessels in the retina stops, and abnormal blood vessels develop. As a result, the infant has an increased risk of detachment of the retina (see retinal detachment). Retinopathy of prematurity can lead to reduced vision or blindness. Laser therapy can help this condition if diagnosis and treatment occur early. Children who experience minor effects may benefit from the use of devices for low vision as they get older. Retinopathy of prematurity was formerly called retrolental fibroplasia.

Suggested resources:

Retrolental fibroplasia See retinopathy of prematurity. Rod-cone dystrophy

See Cone-rod dystrophy.

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Scotoma

Gap or blind spot in the field of vision that may result from damage to the retina. How much a scotoma impairs sight depends mainly on whether it affects central or peripheral vision. Common causes of scotoma include macular degeneration, glaucoma, and inflammation of the optic nerve. People who experience significant vision loss because of scotomas may benefit from the use of magnifiers, bright lighting, and large-print reading materials.

Suggested resource: http://www.tsbvi.edu/curriculum-a-publications/1093-scotoma

Septo-Optic Dysplasia (SOD)

See De Morsier’s Syndrome.

Suggested resources:

Stargardt’s Disease

Inherited disease that causes gradual degeneration of the macula, the area in the middle of the retina that makes possible the central vision needed for reading, driving, recognizing colors, and other activities of daily life. Effects of Stargardt’s Disease, which start at an early age, vary from minor to total loss of detail vision. Over a period of years, people with the disease typically lose sharpness of vision, experience decreased color vision, and may have blind spots. However, peripheral and night vision usually remain unaffected, and complete loss of sight is rare. There is no cure or treatment for Stargardt’s Disease, but such devices as magnifying screens and binocular lenses can help people cope with vision limitations.

Suggested resource:

Strabismus

Condition in which the eyes are not both directed toward the same point simultaneously. Strabismus occurs when eye muscles are not working together properly. It is most commonly an inherited condition, but may also be caused by disease or injury. If diagnosed early, strabismus can usually be corrected. The condition may be treated with corrective eyeglasses, eye-muscle exercises, surgery, or a combination of these approaches. Young children with this condition may need to wear an eye patch over their stronger eye to force their weaker eye to function correctly. Children whose strabismus is not corrected may develop amblyopia.

Sturge-Weber Syndrome

Disorder, present at birth, characterized by a facial birthmark and any of various neurological, visual, and developmental symptoms. People with Sturge-Weber syndrome may, for example, experience seizures, glaucoma, partial paralysis, and learning disabilities. There is no cure for Sturge-Weber syndrome, but many of the symptoms can be treated. For instance, medications may be prescribed to control seizures, and surgery or eye drops may be used to treat glaucoma.

Suggested resource: http://sturgeweber.kennedykrieger.org/

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Thyroid Eye Disease

Thyroid eye disease (TED) is an inflammatory condition closely associated with Graves’ disease. In thyroid eye disease (also called Graves’ Orbitopathy, Graves’ Eye Disease, or Graves’ ophthalmopathy), the immune system sets off an abnormal reaction to the muscles and fatty tissue around the eyes. The symptoms that occur in thyroid eye disease include bulging eyes, swollen eyes, redness, misaligned eyes, tenderness or eye pain, and problems with vision such as light sensitivity, blurriness, or double vision. Although many patients with thyroid eye disease will have abnormal blood tests for thyroid hormone levels, some people experience eye symptoms even though their hormone levels are normal.

Suggested resources: www.preventblindness.org/thyroid-eye-disease

Trachoma

Contagious eye infection, caused by bacteria, that affects the eyelid and cornea. Trachoma can lead to scarring and blindness if not treated. The infection is spread by contact with discharge from the eyes or nose of infected persons and also transmitted by certain flies. Trachoma is rare in the United States, but it affects millions of people around the world, many of them children. Antibiotics are generally an effective treatment for trachoma, especially if used early in the infection. In certain cases, eyelid surgery may be needed.

Suggested resource: www.trachoma.org

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Usher Syndrome

Inherited condition that causes partial or total hearing loss accompanied by gradual vision loss resulting from retinitis pigmentosa. Some people with Usher Syndrome also have problems with balance. There is no cure for the condition. However, early diagnosis makes it possible to help people with Usher Syndrome by providing hearing aids, training in sign language and lip reading, devices for impaired vision, and counseling for preparing for the future.

Suggested resource:

Uveitis

Inflammation inside the eye, affecting the structures that provide most of the blood supply to the retina. Uveitis may affect one or both eyes. The condition may be associated with an underlying disease or have other causes, but in many cases it affects people who are otherwise healthy. People with uveitis typically experience redness of the eye, blurred vision, and light sensitivity. They may also feel pain and see floaters (see floaters and spots). If not properly treated, uveitis can lead to scarring and vision loss. Treatment depends on which eye structures are affected and whether there is an underlying disease. Eye drops and other medications are commonly prescribed to reduce inflammation.

Suggested resource: www.uveitis.org

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Vitelliform Macular Dystrophy

See Best’s Disease.

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How lupus affects the eyes

Systemic lupus is a chronic autoimmune disease that can affect any part of the body, including the eyes. Lupus most often affects the heart, joints, skin, lungs, blood vessels, kidneys and central nervous system (CNS). The clinical course is unpredictable and is characterized by periods of remissions and flares, which may be acute or chronic.

The effects lupus may have in and around the eyes include: changes in the skin around the eyelids, dry eyes, inflammation of the white outer layer of the eyeball, blood vessel changes in the retina, and damage to nerves controlling eye movement and affecting vision.

Involvement of the skin around the eyelids

  • This is most often related to the discoid lupus erythematosus form of cutaneous lupus.
  • The skin lesion is well-defined, slightly raised, scaly, and misformed (atrophic).
  • There are typically no symptoms, but occasional burning and itching may occur.
  • Scarring may result in deformities along the edge of the eyelids.

Dry eyes

  • Approximately 20 percent of people with lupus also have secondary Sjogren’s syndrome, a condition in which the tear glands do not produce sufficient tears to lubricate and nourish the eyes; the other moisture-producing glands are similarly affected. (Primary Sjogren’s syndrome is a systemic disease that, like lupus, can affect many parts of the body.)
  • Typical symptoms are irritated, gritty, scratchy, or burning eyes, a feeling of something in the eyes, excess watering, and blurred vision.
  • Advanced cases of dry eyes may result in damage to the front surface of the eye and impaired vision.
  • The dry eye that is seen in lupus cannot be distinguished from other dry eye conditions.

Scleritis

  • This painful red eye condition is caused by inflammation in the white scleral (outer) layer of the eye.
  • Scleritis occurs in approximately one percent of people with lupus and may be the first sign of the disease.

Retinal vascular lesions (blood vessel changes in the retina)

  • This is the most common form of eye involvement in lupus.
  • The occurrence in lupus can vary depending on the population studied. The lowest incidence reported is three percent, seen in outpatient clinics, and the highest is 28 percent in those hospitalized for lupus-related complications.
  • The presence of these lesions seems to correlate with active disease.
  • Retinal blood vessel changes are due to lack of adequate blood supply to this delicate tissue, and may cause decreased vision ranging from mild to severe. For individuals with severe retinal vascular disease, the prognosis for vision is poor.
  • Retinal vein occlusions (blockages) and retinal artery occlusions have been reported, but these complications are rare and seem to be more related with CNS lupus.
  • The choroidal layer of the eye — the nourishing tissue underneath the retina — can also be affected by lupus, but this is very uncommon. This involvement can appear as excess fluid between the retinal layers. There is an association between lupus choroidal disease and blood vessel disease in the rest of the body, which may be related to kidney disease and blood vessel disease complications seen in lupus.

Neuro-ophthalmic involvement (nerve damage)

  • Cranial nerve palsies can result in double vision, poor eye movement and alignment, poor pupil reflexes, and droopy eyelids.
  • Lupus optic neuropathy occurs in one-two percent of people with lupus. Slow progressive vision loss also can result in more rapid loss of vision from lupus optic neuropathy.
  • Damage to the visual nerve fibers in the brain may cause hallucination and loss of peripheral vision and/or central vision.

Side-effects of certain lupus medications

In addition, some of the medications used in the treatment of lupus may have ocular side effects. In particular, hydroxychloroquine (Plaquenil®) can cause retinal toxicity over time, particularly at high dosages.

Annual comprehensive eye examinations are recommended by the American Optometric Association for people with lupus, especially for anyone taking Plaquenil. As a precaution, people treated with Plaquenil should get a baseline eye exam before (or soon after) starting the drug and visit an eye doctor (ophthalmologist) annually. Long term plaquenil users on high doses will need to monitor eye health regularly to prevent retinal toxicity from long-term use.

Sjogren’s syndrome: What is it?

Conditions

By Marilyn Haddrill; with contributions and review by Robert L. Epstein, MD

Sjogren’s syndrome (pronounced SHOW-grins; also spelled Sjögren’s) is an autoimmune disease that attacks and destroys glands responsible for keeping the eyes, mouth and other parts of the body moist and lubricated. For this reason, dry eyes are a common symptom of Sjogren’s syndrome.

Men and women of any age or race can develop Sjogren’s syndrome, but it is most prevalent among Caucasian women, with onset usually beginning between ages 40 and 60. The Sjogren’s Syndrome Foundation (SSF) estimates that as many as 4 million Americans have the disease, and about 90 percent are women. As many as 3 million may be unaware they have the condition.

Because dry eyes are such a distinctive feature of Sjogren’s syndrome, many cases of the disease go unreported. It’s estimated that 1 in 10 dry eye patients also have Sjogren’s syndrome; and it can take up to four years or longer from onset of the disease to get an accurate diagnosis, according to researchers.

Causes Of Sjogren’s Syndrome

Sjogren’s syndrome is one of the more common autoimmune disorders. In these diseases, a person’s white blood cells attack his or her own tissues and organs, damaging them.

Why abnormal immune responses develop and destroy a body’s own tissues is not clearly understood. Ordinarily, our immune system recognizes our own body parts as “friendly,” and becomes activated only to fight and destroy “foreign” substances or harmful organisms, such as viruses.

Abnormal immune responses may be inherited, or they may be related to prior viral or bacterial infections.

Sjogren’s syndrome can occur alone (primary Sjogren’s syndrome) or it can occur along with other autoimmune diseases, such as rheumatoid arthritis, lupus, celiac disease or scleroderma (secondary Sjogren’s syndrome).

How To Know If You Have Sjogren’s Syndrome

Sjogren’s syndrome symptoms. Image: Sjogren’s Syndrome Foundation.

The classic symptoms of Sjogren’s syndrome are: dry eyes, dry mouth, fatigue and joint pain. Other symptoms may include:

  • Contact lens discomfort
  • Dry sinuses and frequent sinus infections or nosebleeds
  • Sore mouth, tongue or throat
  • Difficulty chewing or swallowing
  • Dry or peeling lips
  • Blepharitis
  • Dry and sore skin
  • Muscle pain without accompanying swelling
  • Vaginal dryness

Other eye symptoms that can occur with Sjogren’s syndrome include blurred vision, a gritty or burning sensation and light sensitivity.

Your eye doctor may perform a number of tests to confirm a diagnosis of Sjogren’s syndrome. In one common test (called a Schirmer’s test), the tip of a small strip of test paper is inserted under your lower eyelid to measure the amount of tears you produce over a certain period of time (usually five minutes).

Other tests may include use of dyes that color your tears; your doctor will then examine your eyes with a microscope to see how quickly your tears evaporate and whether any dryness-related damage has occurred to your cornea or conjunctiva.

The amount and quality of saliva produced in your mouth may also be measured. Your doctor may order other related tests, including blood tests. (Certain antibodies in the blood are commonly found in people with Sjogren’s syndrome.)

A diagnostic test called Sjö (Bausch + Lomb) offers an even more sensitive and specific means of detecting Sjogren’s syndrome. In addition to checking for the traditional antibodies commonly found in the bloodstream of patients with the disease, the Sjö checks for three additional biomarkers that are specific to Sjogren’s syndrome for greater diagnostic accuracy, according to Bausch + Lomb.

How Dry Eye From Sjogren’s Syndrome Is Treated

Dry eye resulting from Sjogren’s syndrome may require ongoing treatment with artificial tears, ointments or other remedies.

You may need lubricating eye drops or ointments to treat dry eye caused by Sjogren’s syndrome.

Because many people with Sjogren’s syndrome have a problem with their tears evaporating too quickly, oil- or lipid-based eye drops sometimes are especially helpful. These drops help slow down tear evaporation so the eyes can stay moist longer between blinks.

Your eye doctor also will check for a problem called meibomian (“my-BOH-me-un”) gland dysfunction (MGD), which is a leading cause of evaporative dry eye. In this condition, the glands in the eyelids that secrete oil (meibum) into the tear film become inflamed and obstructed. A decrease in the amount or quality of the meibum also may be involved. This oil is essential to keep tears from evaporating too quickly. Successfully treating MGD can help reduce Sjogren’s-related dry eye discomfort.

For pain or inflammation, you may also need to take nonsteroidal anti-inflammatory drugs (NSAIDs) such as aspirin or ibuprofen. If you have a severe case of Sjogren’s syndrome, your doctor also may recommend immunosuppressant medications.

Side Effects Of Sjogren’s Syndrome

Because chronic dry eye is one of the major symptoms of Sjogren’s syndrome, you must make sure your eyes are lubricated to avoid damage that can lead to scarring and infection of the eye.

Sjogren’s syndrome destroys glands responsible for lubricating eyes and other parts of the body.

Sjogren’s syndrome also can cause dryness of the mouth that can lead to tooth decay or even loss of teeth. Use of mouth lubricants (artificial saliva) may be needed to keep the mouth adequately moist and to assist in swallowing.

Also, people with Sjogren’s syndrome are more likely to develop lymphoma (cancer of the lymphatic system, which contains white blood cells that play a major role in fighting disease). Lymph nodes may become enlarged or swollen.

Sjogren’s syndrome also can lead to vasculitis (inflammation of blood vessels) that can cause problems throughout the body.

Pregnant women diagnosed with Sjogren’s syndrome should notify their doctors. Certain proteins produced by the immune system that are capable of attacking other potentially beneficial proteins in the body can be passed along to infants.

How To Prevent Dry Eye And Other Sjogren’s Syndrome Symptoms

Using artificial tears and drinking plenty of water can alleviate symptoms of Sjogren’s syndrome.

There are no known ways to prevent Sjogren’s syndrome, but these steps may help ease symptoms:

  • Drink more fluids, especially water.
  • Chew sugarless gum or use hard candies to moisten your mouth.
  • Regularly use artificial tears and ointments to keep your eyes moistened. (Your doctor can recommend the best brands for your needs.)
  • Use saline spray for your nose.
  • Install a humidifier to help reduce dry eyes, nose, mouth and skin.
  • Tell your eye doctor about any drugs you are taking, because some, such as antihistamines for allergies, can cause dryness.
  • Use vaginal lubricants if needed.
  • Don’t smoke, and avoid alcohol.

Sjogren’s Syndrome Resources

If you have Sjogren’s syndrome or know someone who does, additional information — including access to support groups and patient seminars — is available through the Sjogren’s Syndrome Foundation.

Page updated April 2017

Schedule an exam.

Find an eye doctor near you. Notes and References

Sjogren’s Syndrome Foundation website. Accessed May 2015.

The epidemiology of Sjogren’s syndrome. Clinical Epidemiology. July 2014.

Prevalence and predictors of Sjogren’s syndrome in a prospective cohort of patients with aqueous-deficient dry eye. British Journal of Ophthalmology. December 2012.

Clinical manifestations and early diagnosis of Sjogren’s syndrome. Archives of Internal Medicine. July 2004.

Ocular Manifestations of Systemic Lupus Erythematosus: A Review of the Literature

Abstract

About one-third of patients suffering from systemic lupus erythematosus have ocular manifestations. The most common manifestation is keratoconjunctivitis sicca. The most vision threatening are retinal vasculitis and optic neuritis/neuropathy. Prompt diagnosis and treatment of eye disease is paramount as they are often associated with high levels of systemic inflammation and end-organ damage. Initial management with high-dose oral or IV corticosteroids is often necessary. Multiple “steroid-sparing” treatment options exist with the most recently studied being biologic agents.

1. Introduction

Systemic lupus erythematosus (SLE) is a chronic, autoimmune, connective tissue disorder affecting multiple organ systems often with a relapsing and remitting clinical course. Prevalence, clinical manifestations, and morbidity vary significantly between the developing and industrialized worlds. While SLE is more common in people of African and Asian descent, thrombotic complications are more common in Caucasian patients . The highest prevalence has been reported in Italy, Spain, Martinique, and the UK Afro-Caribbean population . The median age of onset is between the late teens and early 40s with a 9 times higher incidence in women compared to men. Ocular manifestations—occurring in up to one third of patients—can be associated with significant morbidity and also a marker for overall systemic disease activity.

2. Genetic Considerations

Concordance rates for SLE among monozygotic and dizygotic twins are 25% and 2%, respectively, suggesting a significant genetic contribution . Major histocompatibility complex genes, such as HLA-A1, B8, and DR3 , as well as alleles that cause deficiency in complement components—C1q, C2, and C4 —have all been linked to lupus.

3. Mechanism of Disease

SLE is a complex disease process demonstrating dysregulation of the immune system at multiple levels. Autoantibodies against double-stranded DNA were first isolated from kidney specimens in patients with lupus nephritis in 1967 . Other autoantibodies that have been implicated in disease include anti-Ro, La, Sm, nucleosome, NMDA receptor, phospholipid, and α-actinin. Two major theories exist on how these autoantibodies cause tissue damage. The first model suggests that anti-double-stranded DNA antibodies bind to circulating nucleosomes to form immune complexes that then get deposited in end-organ capillary beds such as the renal glomerulus and activate immune/inflammatory responses . The second hypothesizes that these autoantibodies cross-react with normal renal proteins causing tissue destruction . The source of autoantigens that trigger the formation of the aforementioned antibodies is thought to arise from apoptotic cells. Normally, early complement factors, such as C1q, bind cellular debris from apoptotic cells, which facilitate phagocytosis by macrophages. Deficiency of such complement factors is an independent risk factor for the development of SLE .

Mass production of autoantibodies relies on multiple factor, which have each independently been targeted as potential immunotherapy in the treatment of lupus. Important steps include T-cell activation via antigen binding to the T-cell receptor and proper costimulation; T-cell activation of B cells; production of cytokines such as TNF-α, INF-γ, IL-10, and B-lymphocyte stimulator.

Medications, hormonal influences, and other factors such as sunlight have all been implicated in disease exacerbation. Drug-induced lupus, most commonly due to procainamide, hydralazine, and quinidine, usually presents with disease involving the skin and joints with renal and CNS manifestations being much more rare . Hormonal replacement therapy has been associated with an increased risk of mild-to-moderate flares .

4. Diagnostic Criteria

According to the 1982 revised criteria for systemic lupus erythematosus, a diagnosis of SLE can be made by the serial or simultaneous presentation of at least 4 of the following 11 criteria: malar rash, discoid rash, photosensitivity, oral ulcers, nonerosive arthritis, serositis, renal dysfunction, neurological derangements (i.e., seizures or psychosis), hematologic disorder (i.e., anemia, leukopenia, thrombocytopenia), immunologic disorder (i.e., anti-DNA antibody, anti-Sm antibody, and false positive VDRL testing), and presence of antinuclear antibodies.

5. Ocular Manifestations

SLE can affect the periorbita, ocular adnexa, eye, and optic nerve. The most common association is keratoconjunctivitis sicca, while the most visually devastating sequelae occur secondary to optic nerve involvement and retinal vaso-occlusion.

5.1. Orbit

Orbital involvement is a rare manifestation of SLE. Vasculitis, myositis, and panniculitis have all been described. Signs and symptoms include proptosis, enophthalmos, orbital pain, blurred vision, chemosis, and restriction of extraocular movements.

Orbital vasculitis leads to nonperfusion of the globe and extraocular muscles. This has been shown to cause irreversible vision loss secondary to ischemic injury to the optic nerve as well as elevated intraocular pressure from neovascular glaucoma .

Orbital myositis is often initially misdiagnosed as bacterial orbital cellulitis, as it usually presents with significant pain, proptosis, periorbital swelling, and globe restriction. CT and orbital ultrasound are both valuable in demonstrating enlargement of one or multiple extraocular muscles. Creatinine kinase, aldolase, and myoglobin levels are markedly elevated. Inflammation and symptoms typically respond to steroids .

Subcutaneous inflammation secondary to SLE was first described by Kaposi in 1883, and the term “lupus erythematosus panniculitis” was coined in 1940 . It is most commonly encountered in the setting of discoid lupus erythematosus. Clinical findings include tender deep subcutaneous nodules usually involving the proximal extremities, trunk, face, and scalp . Orbital involvement is very rare and has only been reported in a handful of paper. Histopathology shows perivascular lymphocytic infiltration . Response to steroids can be quite dramatic in most cases ; however, few cases have shown a more virulent course with significant enophthalmos secondary to fat atrophy and even melting of orbital structures .

5.2. Periorbita

Periorbital edema is an uncommon manifestation of systemic and discoid lupus erythematosus with an overall incidence of 4.8% . It is most common in patients of African decent . Violaceous swelling with overlying eczematous changes without any skin necrosis is seen. Some cases can resemble chronic blepharitis . Etiologies include nephrosis, increased vascular permeability, dermal mucin deposits, and angioedema secondary to C1 deficiency. Treatment options include observation , topical/intradermal/systemic corticosteroids , and antimalarials .

5.3. Eyelids

Typical lesions of discoid lupus erythematosus are slightly raised, scaly, and atrophic. Most commonly, they occur on the head, face, neck, and other sun-exposed areas. Rarely does it affect the eyelids. Histopathologic study shows a hyperkeratotic epithelium with liquefactive degeneration of the basal layer and a dense perivascular/periappendageal lymphocytic infiltration . Diagnosis in most cases is delayed because lesions are often mistaken for blepharitis and eczema. Patients most commonly present with chronic erythema, blepharoconjunctivitis with inflammation of the meibomian glands. Long-term complications include madarosis, lid scarring, and cicatricial ectropion/entropion .

5.4. Ocular Surface

The most common ocular manifestation of SLE is keratoconjunctivitis sicca with the majority of patients endorsing at least one dry eye symptom . Dryness can occur from multiple etiologies. Most patients with SLE develop a secondary Sjogren’s syndrome. In their review of 283 SLE patients, Manoussakis et al. identified 9.2% who had developed Sjogren’s syndrome (SS). The SLE-SS group had a higher frequency of Raynaud’s phenomenon, anti-Ro antibody, anti-La antibody, and rheumatoid factor and a lower frequency of renal involvement, lymphadenopathy, and thrombocytopenia. These patients tend to undergo a more benign course with a significantly reduced mortality and need for immunosuppression . The hallmark of disease is a decreased production of the aqueous layer of the tear film.

An abundance of proinflammatory markers such as IL-17 can be found in the tear film of SLE patients. These are some of the same markers that are found in cicatrizing inflammatory conditions such as Steven Johnsons syndrome. Clinical findings can include symblepharon formation, forniceal foreshortening, and exposure keratopathy. Histopathological findings include loss of goblet cells, keratinization of the conjunctival epithelium, monocellular infiltration, and granuloma formation in the substantia propria . Immunopathology shows immune complex deposition within the epithelial basement membrane with an increased number of CD4+ and CD8+ T cells, B cells, and macrophages .

5.5. Episclera/Sclera

Episcleritis is generally a benign inflammation of the episclera. Typically occurring in young women, symptoms include a dull ache, red eye, and tearing. Decreased visual acuity and severe pain are uncommon. Systemic associations are extremely rare in adults, and a systemic workup is not necessary. Incidence in adult patients with SLE has been reported at 2.4% . However, in children, episcleritis is much more rare but systemic associates are much more common. Read et al. found 6 of 9 patients in their series on pediatric episcleritis to have systemic connective tissue disease. Treatment options include observation or topical/systemic nonsteroidal anti-inflammatory drugs.

Scleritis is a more painful and potentially a vision-threatening condition that warrants prompt treatment. Anterior scleritis can be nodular or diffuse and presents with a red, painful eye that is tender to touch. The injected deep episcleral vessels give a violaceous due to the sclera, which is best appreciated in natural light (Figure 1). Posterior scleritis on the other hand may not be associated with a red eye because it affects sclera posterior to the equator of the globe. Presenting symptoms are pain, blurred vision, limited eye movements, and proptosis. Blurred vision is most commonly caused by exudative retinal detachment, macular distortion due to a large scleral mass, and cystoid macular edema.


Figure 1
Slit-lamp photo demonstrating diffuse anterior scleritis in a patient with SLE.

5.6. Cornea

Corneal epitheliopathy, scarring, ulceration, and filamentary keratitis can all occur secondary to keratoconjunctivitis sicca. More rare corneal complications include peripheral ulcerative keratitis , which can be a marker of active systemic vasculitis, interstitial keratitis, and keratoendothelitis . Spectral microscopy has been used to show dysfunctional appearing corneal endothelial cells in both patients with corneal edema and asymptomatic patients .

Corneal biomechanical properties differ in SLE. Yazici et al. used Reichert ocular response analyzer measurements to show that corneal hysteresis and corneal resistance factor were both lower in SLE patients which can lead to an underestimated IOP and development of keratoconus .

5.7. Retina

Lupus retinopathy is one of the most common vision-threatening complications of systemic lupus erythematosus with an incidence of up to 29% in patients with active systemic disease. A strong correlation exists between presence of retinopathy and CNS disease . The most common pattern of retinopathy is microangiopathy similar to diabetic and hypertensive retinopathy. The earliest findings are small intraretinal hemorrhages and cotton wool spots . Pathogenesis is attributed to deposition of immune complexes in the vessel wall and microemboli. Histopathology shows immunoglobulin and complement deposits, perivascular monocellular infiltrate, and rarely fibrinoid necrosis . Studies using fluorescein angiography describe hyperpermeability of arterioles and venules as well as capillary nonperfusion . Although it is poor prognostic factor for survival, visual outcomes in this group are usually very good .

Retinal vasculitis, a subset of retinal vasculopathy featuring inflammation of the retinal arterioles or venules, tends to have poorer visual outcomes and present in an acute onset fashion. A large percentage of these patients have concomitant antiphospholipid antibodies including anticardiolipin and lupus anticoagulant. In one study, 77% of patients with SLE and retinal involvement had positive antiphospholipid antibody titers, whereas only 29% of SLE patients without retinal disease had positive titers . Histopathologic specimens show fibrinoid change with thrombus formation without a true arteritis . CNS vascular disease demonstrates similar pathology, thus providing a link between CNS vasculitis and severe lupus vasculopathy . In 1984, Hall et al. first reported the link between severe lupus retinal vasculopathy and presence of antiphospholipid antibodies. Since that time, multiple cases have been demonstrating severe vision loss secondary to central retinal artery/vein occlusions, vitreous hemorrhage, retinal ischemia, and neovascularization . While the milder form of retinal vasculopathy is mediated by immune-complex deposition and inflammation, the more severe vaso-occlusive disease stems from fibrinoid degeneration/necrosis without significant inflammation.

Immunosuppression has been successful in improving the appearance of the retinopathy; however, visual recovery has only been reported in few cases. The permanent loss of visual acuity is likely due to retinal ischemia. Addition of anticoagulation to immunosuppression helps to stabilize retinal disease and prevent further vascular events . Other therapies that have been reported for severe disease include plasmapheresis and plasma exchange . Panretinal photocoagulation, intravitreal antivascular endothelial growth factor agents, and vitrectomy may also be considered for the treatment of complications of ocular ischemia (Figure 2).


Figure 2
Fundus photograph demonstrating severe retinal vasculitis. Significant ischemia is present which is highlighted by the attenuated and sclerotic vasculature. Panretinal photocoagulation was required to treat ischemic and neovascular complications.

5.8. Choroid

Lupus choroidopathy with exudative retinal detachments is a rare ocular manifestation with fewer than 40 patients reported in the literature (Figure 3). It is generally seen in patients who have highly active disease including CNS vasculitis and nephropathy as well as uncontrolled blood pressure. Clinical diagnostic ophthalmic imaging is paramount for the diagnosis of choroidal and retinal pathologies. Specifically indocyanine green is extremely valuable for evaluating choroidal vascular and tissue inflammation, while fluorescein angiography is helpful in identifying optic nerve inflammation, retinal vascular disease, retinal ischemia, and macular edema. Baglio et al. used indocyanine green angiography (ICG) to demonstrate that subtle changes in the choroidal circulation can be seen in patients with SLE-associated nephropathy, while similar findings are not seen in SLE patients without renal involvement. The pathogenesis is thought to be multifactorial; uncontrolled hypertension , immune complex deposition in the choriocapillaris , and antiretinal pigment epithelium antibodies have all been implicated as contributing factors.


(a)
(b)
(c)
(a)
(b)
(c) Figure 3
(a) Macular serous retinal detachment in a patient with lupus choroidopathy. (b) Multiple areas of hyperfluorescence seen on fluorescein angiography caused by increased vascular permeability of the choroidal circulation. (c) Large accumulation of subretinal fluid is seen on optical coherence tomography.

Recently, ICG imaging has been used to visualize the choroidal circulation in lupus choroidopathy. Studies have shown focal, transient early-phase hypofluorescence followed by late-phase diffuse hyperfluorescence, distortion of the large choroidal vessels, and also focal clusters of choroidal hyperfluorescence in the intermediate phase. Transient early hypofluorescence and late hyperfluorescence are likely secondary to choroidal vascular perfusion delay with subsequent leakage due to an increase in vascular permeability, which are also observed in other vascular and inflammatory diseases. Unique findings include focal areas of hyperfluorescence in the intermediate frames, which may represent ICG staining of immune complexes .

Although it is a marker of high disease activity, lupus choroidopathy has been shown to be responsive to corticosteroids and other forms of immunosuppression. Given its associations with CNS and renal disease, the presence of choroidopathy is likely an indication for aggressive, long-term immunosuppression.

5.9. Optic Nerve/Central Nervous System

Optic nerve disease is a rare manifestation of SLE and consists of optic neuritis and ischemic optic neuropathy . Presenting visual acuity in SLE-associated optic neuritis is poor with most patients seeing worse than 20/200 . In the Optic Neuritis Treatment Trial (ONTT), only 35.9% had a similar vision . Visual recovery is variable in most patients and can range anywhere from full recovery to count fingers vision. In a study by Lin et al. only 50% of patients recovered to better than 20/25, while 37.5% maintained a visual acuity worse than 20/200. In ONTT, 87% of patients recovered to better than 20/25 at 5 years of followup . The increased severity of disease in SLE-associated optic neuritis compared to idiopathic optic neuritis stems from differences in pathogenesis. SLE-optic neuritis is not due to a primary inflammatory demyelinating process but rather an ischemic process that can cause subsequent demyelination and axonal necrosis. The degree of axonal loss correlates to visual outcome . Luckily, the optic neuritis responds dramatically to corticosteroid treatment . Early diagnosis and prompt treatment with high-dose corticosteroids is associated with better visual outcomes .

The neuromyelitis optica spectrum disorders (NMOSDs) are characterized by a combination of optic neuritis and transverse myelitis. Few cases have been reported in the literature of the presentation of NMOSD in SLE . A recent paper by Jarius et al. demonstrates a high association of aquaporin-4 antibodies in patients with connective tissue disease and symptoms suggestive of NMOSD. The antibodies cause tissue destruction by complement activation. Aquaporin-4 antibody positivity has important clinical implications as it is associated with a relapsing course of myelitis and optic neuritis and can lead to blindness and immobility quickly if not treated

Optic neuropathy in SLE is caused by an ischemic process that affects the small vessels supplying both the optic nerve head and retrobulbar nerve. It usually presents as an acute loss of vision with an altitudinal visual field defect with or without optic disc edema. The disease is most commonly bilateral except in patients with circulating antiphospholipid antibodies. In this subset, a focal thrombotic event in the ciliary vasculature is thought to occur as opposed to a generalized vasculitis . Standard treatment for lupus optic neuropathy includes intravenous high-dose corticosteroids followed by an extended oral taper . Other studies have shown success with other immunosuppressive agents such as cyclophosphamide, cyclosporine, methotrexate, and azathioprine .

Eye movement abnormalities are common in SLE and have been reported in up to 29% of patients . Ischemic microvascular disease of the brainstem is usually the etiology. Sixth nerve palsies are the most common cause of disconjugate gaze abnormalities , while internuclear ophthalmoplegia is the most common cause of conjugate gaze abnormalities .

Retrochiasmal involvement can cause visual hallucinations, visual field defects, nystagmus, and cortical blindness. Few cases of idiopathic intracranial hypertension have been reported. 60% of cases reported in the literature are associated with antiphospholipid antibodies .

6. Therapeutic Considerations

Treatment options for SLE range from nonsteroidal anti-inflammatory drugs, corticosteroids, antimalarials, immunomodulatory, and biologic agents. Significant ocular involvement—orbital inflammation, scleritis, retinal vasculitis, choroiditis, and optic neuritis—warrants systemic therapy. The goal of treatment is to suppress immune activity, specifically decreasing the level of autoantibodies.

Corticosteroids are the mainstay and most effective short-term therapy for SLE . They inhibit both the innate and adaptive immune response by preventing proliferation and inducing apoptosis of T cells, B cells, and macrophages as well as reducing levels of cytokines and prostaglandins . Generally, by the time patients present with ocular manifestation, they have a high level of systemic inflammation. Previous papers have shown that a high correlation exists between CNS vasculitis and retinal vasculitis as well as nephritis and choroiditis . Nguyen et al. recently described a series of four of 28 patients with choroidopathy who died from lupus-related complications . Early and aggressive treatment is needed for this group to prevent increased morbidity and mortality. Thus, it is of extreme importance that patients presenting with severe ocular manifestations be treated with high-dose oral or even IV steroids early on in the disease course. Periocular steroid injections may have a role in unilateral/asymmetric disease; however, they should be used cautiously and avoided in patients with scleritis.

Steroid-sparing immunosuppressive agents are used in a large amount of patients secondary to treatment failure or harmful side effects of corticosteroids. Antimalarials such as chloroquine and more commonly hydroxychloroquine are often used. These medications are highly efficacious in curtailing future flares with fewer side effects compared to other immunomodulatory drugs such as alkylating agents. However, ocular effects of these drugs are well known. Irreversible vision loss secondary to a drug-induced maculopathy has been well documented in the literature. Factors associated with high risk of developing maculopathy include greater than 5–7 years of therapy, greater than a cumulative dose of 1000 g of hydroxychloroquine, impairment of liver or kidney function, obesity, age greater than 65, and preexisting retinopathy. The American Academy of Ophthalmology recommends a baseline-dilated eye exam on all patients starting hydroxychloroquine followed by annual exams starting at 5 years after initiating therapy. A Humphrey 10–2 automated visual field test along with multi-focal electroretinogram, spectral domain optical coherence tomography, or fundus autofluorescence should be performed at each of these visits. Discontinuation of the drug should be recommended at the earliest sign of toxicity . Unfortunately, cases of progression of retinopathy despite cessation of therapy have been reported .

Methotrexate, azathioprine, mycophenolate mofetil, cyclosporine A, cyclophosphamide, and chlorambucil have all been employed with varying degrees of success.

In the past few years, newer drugs, categorized as biological agents, have emerged targeting specific molecules involved in B- and T-cell activation. One of the first to be utilized in SLE was rituximab, a chimeric murine/human anti-CD20 antibody. Multiple studies have shown clinical improvement in refractory patients . Rituximab has also shown efficacy in treating noninfectious forms of ocular inflammation including that secondary to SLE .

7. Conclusions

In summary, a myriad of ocular manifestations of systemic lupus erythematosus have been described, and in some patients, these findings may be a presenting sign of systemic disease. Moreover, their presence can be a sign or a marker of disease activity. In the cases of choroidopathy and retinopathy, ophthalmic findings can be a poor prognostic systemic risk factor with the potential for both ophthalmic and systemic morbidity. For this reason, treatment typically involves a considered assessment of both the systemic and ophthalmic findings in determining the proper therapy and duration of treatment. Close communication between the consultant ophthalmologist and treating rheumatologist is critical in the effective management of these complex clinical situations.

Conflict of Interests

This manuscript has not been previously submitted to any other journal. The authors have no financial conflict of interests.

Funding

This research was funded in part by an unrestricted grant from Research to Prevent Blindness (Emory Eye Center, Emory University School of Medicine).

Autoimmune Disease And Vision

An autoimmune disease is a disorder that causes the immune system to mistake healthy cells for dangerous pathogens and attack them.

Many autoimmune diseases impact the health and function of the eyes. It’s important to be aware of these affects if you or someone you love has an autoimmune disease, so let’s take a look at some of the more common ones.

Multiple Sclerosis

Multiple sclerosis is so closely linked to vision health that eye doctors are sometimes the first to spot the disorder. In many cases, optic neuritis (a gradual or sudden loss of vision due to the inflammation of the optic nerve) is one of the first symptoms to appear.

Psoriasis

We tend to think of psoriasis as a skin condition where skin cells build up to form scaly, dry, itchy patches. However, it can also cause inflammation of the conjunctiva, the clear membrane covering the inside of the eyelids and the whites of the eyes, causing redness and discomfort.

Thyroid Disease

Disorders that cause high or low thyroid function lead to an increased risk of glaucoma, a serious eye condition in which increased pressure in the eye damages the optic nerve, causing vision loss. High thyroid function can cause tissues to build up around the eyes, increasing the pressure. At the other end of the spectrum, low thyroid function can make it harder for the eyes to circulate fluids, another way that fluid pressure can increase.

Lupus

Lupus is a chronic autoimmune disease that can damage the skin, joints, organs, and even the eyes. Symptoms range from mild to life-threatening. Lupus affects the eyes by causing inflammation. Symptoms include blurred vision, headaches, dry eye, soreness, and light sensitivity.

Type 1 Diabetes

All forms of diabetes have dangerous implications for vision health, but type 1, even though it is far less common than type 2, is one of the leading causes of blindness in the US. Poor blood sugar control is very hard on blood vessels over time, including the blood vessels in the back of the eye. When the weakened vessels break, they compromise the retina’s blood supply and leak blood into the eye. This is called diabetic retinopathy, but diabetes also increases the risk of developing cataracts and glaucoma.

Your Optometrist Is Your Best Resource

Other autoimmune conditions that impact eye health include Sjorgen’s syndrome, ulcerative colitis, Crohn’s disease, uveitis, and Behcet disease. If you or someone you love is living with an autoimmune condition, make sure your local Vision Source® member optometrist knows about it so that they can work with you to keep your eyes healthy and your vision strong.

Vision Source® is honored to be your lifelong partners in vision health!

Find a Vision Source® practice near you using our search tool.

Top image used under CC0 Public Domain license. Image cropped and modified from original.
The content on this blog is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of qualified health providers with questions you may have regarding medical conditions.

Author Vision Source — Published December 17, 2018

Posted In Eye Health Awareness

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