Autoimmune Diseases, Treatment, and Contact Lens Wear

Content written by: June Smith-Jeffries – FCLSA, NCLE, COT Content originally published in the Summer 2015 edition of The Eighth Line

Table of Contents • Introduction • Autoimmune Diabetes

o Type 1 Diabetes Mellitus o Latent Autoimmune Diabetes of Adulthood (LADA) • Sjögren’s Syndrome • Fibromyalgia • Rheumatoid Arthritis (RA) • Systemic Lupus Erythematosus (SLE) • Ankylosing Spondylitis (AS) • Systemic Sclerosis (Scleroderma) • Raynaud’s Phenomenon • Sarcoidosis • Multiple Sclerosis (MS) • Myasthenia Gravis • Ulcerative Colitis • Corticosteroids and Dilated Eye Exams • Post Test

Introduction Autoimmune diseases occur when a person’s immune system does not distinguish between healthy tissue and antigens. An antigen is any substance foreign to the body that evokes an immune response. As a result, the body sets off a reaction that destroys normal tissues.

Normally the white blood cells in the body’s immune system help to protect against harmful substances such as bacteria, viruses, toxins, cancer cells and blood and tissue from outside the body. These substances contain antigens. The immune system produces antibodies against these antigens that enable it to destroy these damaging substances.

1 | Page An autoimmune disease may affect one or more organ and various types of tissue. Areas often affected by autoimmune diseases include: connective tissues, joints, muscles, blood vessels, the skin and endocrine glands. There is significant overlap in endocrine and autoimmune diseases because many autoimmune diseases originate in an endocrine gland, for instance, type 2 diabetes, thyroid diseases and Addison’s disease.

Autoimmune diseases have been found in virtually every organ system in the body. Most autoimmune diseases continue for the lifetime of the patient because there are no cures. Patients who have autoimmune diseases often require continual care. Some conditions may go into temporary remission, requiring only intermittent care. Many the medications prescribed for treating these diseases are expensive and have unfortunate side effects.

The exact cause of autoimmune disease is unknown. One theory is that some microorganisms (bacteria or viruses) or drugs may trigger changes that confuse the immune system. Autoimmune diseases tend to affect far more women than men. While there are several theories, the most commonly agreed upon is that estrogen production puts women at a greater risk.

There are at least eighty known autoimmune diseases, all of which of course cannot be discussed within the confines of this article. If you have a patient who has any type of autoimmune disease it is advisable to educate yourself about the condition so that you will know how the disease affects the patient and its possible ocular complications.

2 | Page The Two Types of Autoimmune Diabetes

Type 1 Diabetes Mellitus Insulin dependent diabetes mellitus is an inflammatory autoimmune disease of the pancreas, which results in a lack of insulin production. Insulin is produced in the pancreas by beta cells of the islets of Langerhans. The main source of energy for all cells and especially for brain cells is glucose. Insulin is necessary for glucose to get into cells and be used for energy production. After eating, the glucose level in blood rises, which leads to insulin being released from the pancreas. In a person with type 1 diabetes, beta cells of Langerhans are damaged by autoimmune inflammation, leading to an insufficiency of insulin. When the glucose level in blood rises, cells do not have enough energy for proper metabolism. Type 1 diabetes is typically diagnosed early in childhood or adolescence. Patients with type 1 diabetes become insulin dependent very rapidly.

Latent Autoimmune Diabetes of Adulthood (LADA) Latent autoimmune diabetes of adulthood (LADA) is a relatively newly discovered form of diabetes. Previously, patients who had LADA were often diagnosed as having type II diabetes. LADA, also known as diabetes type 1.5, is a slowly progressive form of type 1 diabetes mellitus.

To confirm a diagnosis of LADA a patient must be at least 30 years of age or older, positive for at least one of the autoantibodies found in Type I diabetes, have a lean build or a low body mass index (BMI). Having a low BMI is a major difference from type II diabetes, in which patients have a high body mass index usually as a result of obesity. Patients with LADA eventually must take insulin injections, but never within the first six months of the diagnosis.

Ocular Signs and Symptoms Autoimmune Diabetes and Contact Lens Wear Patients with autoimmune type diabetes are at significant risk of developing corneal lesions. There is also a possibly of the development of corneal numbness. For these reasons, these patients should not be fit with contact lenses. Keratoconjunctivitis sicca is a major problem for patients with autoimmune type diabetes, just as it is in endocrine related type 2 diabetes, as is the very real possibility of diabetic retinopathy.

Sjögren’s (pronounced show-grins) Sjogren’s syndrome often accompanies other immune system disorders such as rheumatoid arthritis and lupus. In Sjogren’s syndrome, the mucous membranes and moisture-secreting glands of eyes and mouth are usually affected first. The condition may affect other parts of the body, including the lungs and kidneys. The cause of Sjögren’s syndrome is unknown. Although it can develop at younger ages and in men, Sjögren’s syndrome occurs most often in women between the ages of 40 to 50.

3 | Page Ocular Signs and Symptoms Severe keratoconjunctivitis sicca is a major complication of this disorder. In most cases, contact lenses should not be fit. Under unusual circumstances in which a patient cannot achieve functional visual acuity with eye glasses, such as in keratoconus, a scleral lens may give the best result. Systane®; an eye drop, should be advised and in such a case, the patient will require frequent follow up with an optometrist or an ophthalmologist, in conjunction with the fitter seeing the patient more often than normal.

Fibromyalgia Fibromyalgia, also known as fibro myositis and fibrositis, is a common chronic disease which is characterized by widespread, constant pain throughout the body, sleep disturbances and exhaustion. The majority of patients with fibromyalgia are women. Typically, symptoms appear between the ages of 20 and 50. However, fibromyalgia can also affect older women, men, teenagers and children.

Ocular Signs and Symptoms Fibromyalgia patients suffer from mucous membrane dryness of the nose and mouth and the eyes. It has been reported that tear production may be decreased in approximately 90 percent of patients with the disorder. Medications prescribed for fibromyalgia usually include a combination of sleep medications, muscle relaxants and antidepressants, all of which will likely worsen the drying effect. As with anyone who has keratoconjunctivitis sicca, careful evaluation of tear production and the quality of the tear film must be undertaken before considering contact lens fitting. Many patients who have fibromyalgia cannot tolerate any type of soft lens. Success is usually greater with RGP lenses.

Rheumatoid arthritis (RA) RA is a highly inflammatory disease. It causes pain, swelling, deformity and destruction of the joints leading to loss of function. The most commonly affected joints are the small joints of the fingers, thumbs, wrists, feet and ankles. However, any joint may be affected. This condition also affects organs, such as the heart and kidneys. Severity of the disease varies; however some patients with RA are wheel-chair bound.

Ocular Signs and Symptoms Keratoconjunctivitis sicca is the most common ocular manifestation of RA and has a reported prevalence of 15 to 25 percent. Additional ocular conditions include scleritis, episcleritis and keratitis. Other, less common ocular manifestations of RA include choroiditis, retinal vasculitis, episcleral nodules, exudative or serous retinal detachments and macular edema.

Disease modifying drugs are used to ease painful symptoms and to help slow the progression of the disease. Methotrexate is usually the first drug of choice. It has many unpleasant side effects; however, the only ocular

4 | Page complication is possible blurred vision. Patients with RA suffer from joint stiffness and swelling of the fingers along with keratoconjunctivitis sicca. Therefore, careful consideration should be made before fitting an RA patient with contact lenses. If a patient who has RA is fit, follow up visits should be more frequent. Systane® eye drops should also be advised.

Systemic Lupus Erythematosus (SLE) Women are eight times more likely than men to develop this disease. SLE is a chronic and complex disease that can affect multiple organs and regions of the body causing a wide range of symptoms. Most people with SLE develop joint and muscle pain. The small joints of the hands and feet tend to be the ones affected most. Joint stiffness is common and is usually worse first thing in the morning. Mild joint swelling may occur but severe arthritis with joint damage is unusual. A form of lupus dermatitis that is isolated to the skin without internal disease is called discoid lupus. Plaquenil (chloroquine) is one of the main drugs used to treat SLE. This drug has a propensity to cause retinopathy, consequently, patient taking Plaquenil must have semi-annual dilated retinal examinations.

Ocular Signs and Symptoms SLE manifests in episodes of episcleritis, keratitis, and keratoconjunctivitis sicca. Due to joint stiffness and swelling of the fingers in conjunction with dryness of the eyes, careful consideration should be made before fitting a patient who has SLE with contact lenses. If an SLE patient is fit, follow up visits should be more frequent. Systane® drops should be advised.

Ankylosing Spondylitis (AS) Ankylosing spondylitis (pronounced ank-kih-low-sing spon-dill-eye-tiss), or AS, is a form of arthritis that primarily affects the spine, although other joints can become involved. It causes inflammation of the spinal vertebrae that can lead to severe, chronic pain and discomfort. In the most advanced cases the inflammation can lead to new bone formation on the spine, causing the spine to fuse in an immobile position, sometimes creating a forward-stooped posture. Along with the stooped posture, patients are often unable to bend the neck so that looking upward is almost impossible. AS can also cause inflammation, pain and stiffness in other areas of the body such as the shoulders, hips, ribs, heels and small joints of the hands and feet. Medications for this condition are generally for pain relief and include OTC anti- inflammatory drugs and prescribed corticosteroids.

Ocular Signs and Symptoms People with AS may develop iritis or uveitis. Because of the inability to move the neck freely and possible inflammation in the hands, contact lenses are not advised for these patients.

5 | Page Systemic Sclerosis (Scleroderma) The word scleroderma literally means hard skin. In systemic sclerosis, the immune system damages the connective tissue cells which are found in many parts of the body. Connective tissues support, binds or separates other tissues and organs within the body. Scleroderma is also classed as a connective tissue disease. It is not known what triggers the immune system to cause systemic sclerosis. It may be triggered by certain viruses, chemicals or drugs. There is also a likely an inherited tendency to develop this condition. Certain cells called fibroblasts are affected by the immune system in some way which caused it to make too much collagen. The collagen is deposited in skin, blood vessels and organs. This causes scarring and fibrosis.

Raynaud’s phenomenon Raynaud’s phenomenon, in which there are temporary changes to the skin in response to cold or stress, often accompanies scleroderma. Affected areas are usually in the fingers, toes and nose. The skin turns pale, blue and then red and returns to normal when warm or no longer stressed. Ulcerations can form on fingertips. Swelling of fingers and toes is a common early sign. Fingers may look sausage-like; hand movement may be painful or difficult. People with scleroderma also suffer from joint pain and swelling and difficulty in moving joints because of the tightness of the skin. Although scleroderma has no ocular signs or symptoms, it is inadvisable to fit patients with contact lenses that have this condition due to swelling of the fingers and difficulty in movement of the joints in the hands. Various medications are used to treat scleroderma depending on the organs and joints involved, as not two cases of this condition are alike.

Sarcoidosis Sarcoidosis is a disease of unknown etiology in which inflammation occurs in the lymph glands, the chest, lungs, liver, eyes, skin or other tissues. There is no cure for this condition. As with scleroderma, medications are prescribed depending on which organs or areas of the body are involved. Most of these drugs are some type of corticosteroid.

Ocular Signs and Symptoms Ocular manifestations include conjunctival nodules, enlarged lacrimal glands, cranial nerve paralysis, uveitis and optic neuropathy. In most cases it is permissible to fit patients who have sarcoidosis with contact lenses; however large conjunctival nodules or lacrimal gland or cranial never involvement may make fitting more difficult.

Multiple sclerosis (MS) MS is a disease where patches of inflammation occur in parts of the brain and/or spinal cord. This can cause damage to parts of the brain and lead to multiple symptoms. Some MS patients find that their symptoms worsen gradually with time, although it is more common for symptoms come and go at different times in what is referred to as a flare.

6 | Page Medications for MS are disease modifying agents which help reduce activity and disease progression. One such drug is Novantrone which has no ocular side effects.

Ocular Signs and Symptoms The first symptom of MS for approximately one in four people is inflammation and swelling of the optic nerve. Optic neuritis can be extremely painful and may cause vision loss. In MS patients optic neuritis usually only affects one eye. Other ocular symptoms include noticeably blurred vision and diplopia. Unless a patient is having a flare with ocular involvement, contact lenses may be fit. When a flare occurs patients should discontinue wear their lenses until the flare is in remission.

Myasthenia Gravis Myasthenia gravis is a chronic autoimmune/neuromuscular disease characterized by varying degrees of weakness of the voluntary muscles of the body. The condition is caused by faulty nerve stimulation the muscles to contract. Myasthenia gravis is caused by a defect in the transmission of nerve impulses to muscles. It occurs when normal communication between the nerve and muscle is interrupted. There are effective treatments for this condition which include medications that help with muscle strength and muscle contractions. Corticosteroids are also used, if necessary. In some cases surgery to remove the thymus gland may be recommended.

Ocular Signs and Symptoms The muscles around the eyes are most commonly first affected as these are constantly in use and tire quickly. This causes ptosis and diplopia. In some patients, the muscles around the eyes are the only ones affected. If symptoms only affect the muscles around the eyes for longer than two years then the condition is unlikely to progress to other muscles. This is known as ocular myasthenia and affects 1 in 6 people with the disease. Due to the development of ptosis and diplopia, patients with this condition should not be fit with contact lenses. Ulcerative Colitis In this disease, the immune system mistakes harmless bacteria inside the colon for a threat and attacks the tissues of the colon causing it to become inflamed. The condition, like many autoimmune diseases tend to be long-term. Mild to moderate flares can usually be treated at home. However, more severe flare-ups need to be treated in hospital to reduce the risk of serious complications.

Ocular Signs and Symptoms Approximately five percent of patient with ulcerative colitis and Chron’s disease have ocular inflammation, such as episcleritis, uveitis, keratoconjunctivitis sicca and keratopathy. Often inflammation of the eyes is a precursor of these diseases. If any of these ocular conditions should occur, contact lens patients should be taken temporarily out of their lenses and referred to an optometrist or an ophthalmologist.

7 | Page Corticosteroids and Dilated Eye Exams During the course of many autoimmune diseases, corticosteroid drugs are be prescribed in order to lessen symptoms or to slow the disease process. Some conditions require steroids to be taken on regular basis. In such cases, patient should be advised to have dilated eye exams every six months, because of the possible ocular complications of steroid medications.

8 | Page Post Test: Autoimmune Diseases, Treatment and Contact Lens Wear Complete the following quiz based on the above information and submit the quiz via email, fax, or mail to the ACAO to receive 1EC credit. Note: More than one answer may apply

Name: ______

License #: ______

Date: ______

1. Ocular complications for patients who have autoimmune type diabetes include which of the following?

a. Keratoconjunctivitis sicca

b. Episcleritis

c. Corneal numbness

d. Retinal detachment

2. In which of the following conditions are the mucous membranes and moisture‐secreting glands of eyes and mouth are

usually affected first?

a. Rheumatoid arthritis

b. Lupus

c. Fibromyalgia

d. Sjogren's

3. Widespread, constant pain throughout the body, sleep disturbances and exhaustion characterize which of the following conditions?

a. Rheumatoid arthritis

b. Fibromyalgia

c. Lupus

d. Sjogren's

9| P a ge 4. What is the reported prevalence of keratoconjunctivitis sicca in RA patients?

a. 5 to 10 %

b. 10 to 20 %

c. 15 to 25 %

d. 25 to 30 %

5. What condition is Plaquenil commonly used to treat?

a. SLE

b. Discoid lupus

c. RA

d. AS

6. What condition is often accompanied by Raynaud’s phenomenon?

a. Sarcoidosis

b. Scleroderma

c. Ankylosing spondylitis

d. Systemic lupus erythematosus

7. Conjunctival nodules and enlarged lacrimal glands may manifest in which of the following conditions?

a. SLE

b. Scleroderma

c. Sarcoidosis

d. MS

10 | P a ge 8. What is the first symptom for approximately one in four people who develop MS?

a. Iritis

b. Optic neuritis

c. Optic atrophy

d. Keratoconjunctivitis

9. How often should autoimmune disease patient who are prescribed regular use of corticosteroids be advised to have dilated eye examinations?

a. Every three months

b. Every four months

c. Bi‐annually

d. Annually

10. Ocular inflammatory diseases are often a precursor to which of the following?

a. Myasthenia Gravis

b. Sjogren's

c. Ulcerative colitis

d. Chron’s

11 | P a ge