Birdshot Chorioretinopathy

What is birdshot chorioretinopathy?

Eyeball Anatomy

See Anatomy of the Eye

Birdshot chorioretinopathy (BSC) is a type of uveitis (pronounced, “you-vee-EYE-tis”), a term used to describe inflammation inside the eye.  BSC mainly causes inflammation of the choroid and retina, but may affect other parts of the eye as well.  The choroid is the part of the uvea that lies under the retina, which is the “film” in the back of the eye that “takes the picture” of objects you look at.  BSC is fairly rare form of inflammation affecting both eyes of men and women, usually starting in middle age.

What causes birdshot chorioretinopathy?

Birdshot chorioretinopathy (BSC) is strongly related to genetics.  Most people with BSC have inherited a cell protein called HLA-A29.  However, most individuals with HLA-A29 do not develop BSC; it appears to be triggered by an external event, such as an infection that “awakens” the immune response, which then abnormally attacks the eyes.  BSC is most common in people of European ancestry.       

What are the symptoms of birdshot chorioretinopathy?

Birdshot chorioretinopathy (BSC) usually presents with the slow-onset of floaters and blurred vision in both eyes.  The floaters appear as tiny floating dots, which move or “float” in the vision and are seen especially well in bright environments.  Shimmering lights may also be reported. Some patients note difficulty seeing at night. Symptoms may be very bothersome despite normal vision as measured on the eye chart.  Over many years without treatment, the vision deteriorates further with loss of contrast, color vision, peripheral vision, and central vision.  The symptoms vary from person to person and some have more rapid and severe deterioration than others.

How is birdshot chorioretinopathy diagnosed?

The diagnosis of birdshot chorioretinopathy (BSC) may be delayed due to the slow onset of symptoms and the subtle findings on the eye exam.  A retinal specialist or uveitis specialist may be needed to perform sophisticated testing and make the diagnosis.  Inflammation may be detected in many different parts of the eye, but the most typical findings include numerous pale spots inside the back of the eye.  Blood testing for HLA-A29 is positive in the vast majority of patients with BSC.  However, not all patients with uveitis who are positive for HLA-A29 have birdshot chorioretinopathy.  Therefore, it is necessary to exclude other diseases that may simulate BSC including lymphoma, sarcoidosis, tuberculosis, syphilis, and cancer medications such as pembrolizumab and others.    

How is birdshot chorioretinopathy managed?

Birdshot chorioretinopathy (BSC) usually requires management by an experienced retinal or uveitis specialist.  In most cases, systemic treatment (pills or injections into the skin) are needed to control the inflammation.  In a small subset of patients, localized treatment to the eye is sufficient.  This is more often the case in older patients at onset of symptoms.  When pills are used, the eye doctor frequently coordinates medical care with the expert assistance of a rheumatologist (a medical specialist with expertise in auto-immune diseases, like rheumatoid arthritis).  In BSC the rheumatologist monitors the patient for medication side-effects that may develop outside the eyes. In many cases, the uveitis may be long-lasting. In these cases, years of therapy are needed to preserve vision.  

Your doctor will choose from a variety of medications. Steroids (pills, eye drops, and injections) may be used at the start of treatment to gain rapid control of inflammation.  However, long-term steroid treatment in high doses is usually avoided to prevent side-effects of steroid therapy.  For long-term control methotrexate (MTX) pills or skin injections may be given weekly.  MTX has a long record of safety and is affordable.  If MTX fails or causes side-effects (liver or bone marrow), CellCept is another suitable medication, though it may cause diarrhea.  Cyclosporin has been used effectively, but is fraught with a high incidence of problems with hypertension (high blood pressure) and kidney toxicity.  Humira is a new biologic treatment given as an injection into the skin every two weeks.  It has been approved by the FDA for treatment of uveitis, such as BSC.  All medications used to treat BSC may have adverse effects and must be monitored for effectiveness and safety in a given patient. 

Birdshot chorioretinopathy is a serious eye problem and may result in loss of vision or blindness.  However, by seeing your eye doctor and taking the medications exactly as recommended, damage to your vision can be minimized.

By Scott E. Pautler, MD

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

Copyright  © 2020-2023 Designs Unlimited of Florida.  All Rights Reserved.

Steroid Eye Drops

What are Steroid Eye Drops?

Steroid eye drops are prescription medications used to reduce pain, swelling and inflammation. This class of medication is separated from non-steroids by mechanism of action, effectiveness, and side effects. There are a number of steroid eye drop brands available. Examples include prednisolone (Pred Forte, AK-Pred), fluorometholone (FML, FML Forte, Flarex), dexamethasone (Ocu-dex), loteprednol (Lotemax, Alrex), difluprednate (Durezol), rimexolone (Vexol).

How do steroid eye drops work?

Steroid eye drops work by inhibiting a wide variety of biochemicals in the body that promote inflammation. Steroid eye drops are usually more effective that non-steorid eye drops (NSAID eye drops) when used alone, but these two classes of anti-inflammatory drops often work best when used together.

There are advantages and disadvantages to the various brands of steroid eye drops. To varying degrees, all steroid drops reduce inflammation and they all have side effects. The most important side effects include cataract formation and elevation of Intraocular pressure (glaucoma). Prednisolone acetate has been the gold standard for treating ocular inflammation. Dexamethasone generally is not as effective and it has comparable side effects. Difluprednate (Durezol®) is as effective as prednisolone and can be used less often, but it is much more expensive. Loteprednol (Lotemax®) and rimexolone (Vexol®) are less likely to cause glaucoma, but they are expensive. Low concentrations of fluorometholone (FML®) is not likely to cause cataract or glaucoma, but it is not as strong as prednisolone and are mainly used for treating inflammation outside the eye, as in cases of blepharitis (inflammation of the eyelids) and keratitis (inflammation of the cornea).

How does the doctor choose which steroid drop to use?

In some cases the doctor has had good experience with a specific agent for a given situation. In other cases the choice may be guided by convenience and cost. For convenience the costlier difluprednate may be used twice a day compared to 4 times a day for prednisolone. Patients with a tendency for glaucoma, may require more expensive medication such as rimexolone or loteprednol.

If you have strong preferences, be sure to communicate with your doctor to be given the best steroid eye drops for your situation. Always use your eye drops exactly as prescribed and keep all appointments as scheduled in order to monitor for effectiveness and safety.

Ophthalmic Steroid Prices6/25/18 
    
GenericTradeCostSource
PrednisolonePred Forte $            27GoodRx
FluorometholoneFML Forte $            35GoodRx
DexamethasoneDecadron $            60CVS
RimexoloneVexol $            93GoodRx
DifluprednateDurezol $          180GoodRx
LoteprednolLotemax $          230GoodRx

By Scott E. Pautler, MD


What is the Amsler grid?

The Amsler grid is a test used to detect and monitor macular disease (see Anatomy of the Eye). The macula is the area of the retina in the back of the eye that is responsible for seeing details in the central vision.  The retina is a thin layer of delicate nerve tissue that lines the inside wall of the eye like the film in a camera.  In the eye, light is focused onto the retina, which “takes the picture” and sends the image to the brain.

How is the Amsler grid used?

The grid is observed one eye art a time with reading glasses if needed for proper focusing at normal reading distance. The patient is asked to fixate on the center of the grid while using “side vision” to see if there are any missing areas. The lines on the grid should appear straight and uniform. If any abnormalities are noted, an ophthalmologist (retinal specialist) may be consulted to determine the cause of the problem.

Below is a link to download or print an Amsler grid chart for use at home.

Amsler grid RVAF

Some patients prefer a more sensitive (and more expensive) test to monitor the vision called the Foresee Home Monitoring Program.

By Scott E. Pautler, MD

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

Copyright © 2018-2022 Designs Unlimited of Florida.  All Rights Reserved.

Pain in and Around the Eye

globe anatomy
anatomy of the eye (click on image to enlarge)

Pain is a symptom that often warns of serious problems. The nature of the pain may help to determine the origin of the pain and, sometimes, the cause. The following are types of pain and their causes:

Severe, sharp, jabbing pain in the eye: If persistent and associated with a sensation like sand in the eye, this pain often suggests a problem in the cornea. The cornea is the clear front window at the front of the eye. It is replete with very sensitive nerve endings that may be irritated by any disruption of the “skin” on the surface like a corneal abrasion. Treatment is important to relieve pain and prevent infection or scarring of the cornea as this may result in a loss of vision.

A very brief isolated episode of a jab in the eye: May be due to a benign blockage of an oil gland in the eyelid margin (blepharitis). It may also be a symptom of a type of migraine called an “ice pick” headache.

A burning, tingling pain over the forehead: May indicate the onset of shingles (Herpes Zoster). The pain often precedes the onset of a painful rash composed of small vesicles (blisters) on the forehead. It is important to start antiviral medication and evaluate the eye to determine if there is direct eye involvement.

Deep, boring pain: May be seen with sinus pathology alone and may be present in varying severity. However, this type of pain may also be due to problems in the brain such as aneurysm or brain tumor. This pain may also be caused by high pressure in the eye (acute glaucoma) or serious inflammation inside the eye (uveitis). Rarely, poor blood flow to the eye from the carotid artery may cause a deep pain around the eye; atherosclerosis or giant cell arteritis may be the cause.

Pain may be caused by dry eye syndrome. It is usually associated with dryness, burning, and foreign body syndrome.

This monograph does not cover all types or causes of eye pain. Any significant, persistent pain in the eye warrants evaluation. In many cases the more severe the pain, the more urgent the need for evaluation.

By Scott E. Pautler, MD

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

Copyright  © 2018-2022 Designs Unlimited of Florida. All rights reserved.

NSAID Eye Drops

What are NSAID Eye Drops?

NSAID eye drops are prescription medications used to treat macular edema or reduce pain and inflammation. NSAID stands for Non-Steroidal Anti-Inflammatory Drug. This class of medication is separated from steroids by mechanism of action, effectiveness, and side effects. There are a number of NSAID eye drop brands available. Examples include Ketorolac, Acular, Acuvail, Voltaren, Nevanac, Ilevro, Xibrom, Bromday, Prolenza, Ocufen, and Bromsite.

How do NSAIDS work?

NSAIDS work by inhibiting the COX enzyme that produces specific prostaglandins, which promote inflammation. Prostaglandins are a major class of inflammatory mediators in the body. There are other mediators of inflammation that sometimes need to be controlled, so steroid eye drops are often used in addition to NSAIDS.

There is conflicting evidence as to whether one NSAID drug is better than another. Some believe that Nevanac and Ilevro are better for pain control. Others believe that Voltaren is better to control signs of inflammation inside the eye.

How does the doctor choose which NSAID to use?

In some cases the doctor has had good experience with a specific agent for a given situation. In other cases the choice may be guided by convenience and cost. For convenience some drops may be used once or twice a day (e.g. Ilevro, Xibrom, Bromday, Prolenza, and Bromsite). Although their prices vary, they tend to be expensive in comparison to generic drugs.

As an alternative, other NSAIDS are used more frequently, but cost less. Acular and Voltaren are available in generic formulations that cost under $20. These drops are frequently used four times a day. They are less expensive even though more eye drops are used per day. They may cause eye irritation in some patients.

If you have strong preferences, be sure to communicate with your doctor to be given the best NSAID eye drops for your situation. Be sure to use your eye drops exactly as prescribed and keep all appointments as scheduled in order to determine the effect of the medication and to look for side effects.

By Scott E. Pautler, MD

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

Copyright © 2017-2022 Designs Unlimited of Florida. All Rights Reserved.

Treatment of Uveitis

globe anatomy
anatomy of the eye (click on image to enlarge)

How is Uveitis treated?

The key to treating uveitis is to identify the underlying cause. However, the specific cause may not always be found. Therefore, it is helpful to place a given case of uveitis into various classifications in order to treat most effectively. In some cases there is an infection that requires treatment with antibiotics. In other cases there is an underlying inflammation in the body outside the eye that is not associated with infection. In such cases the treatment of the systemic condition is required to settle the eye. Finally, there are inflammatory conditions not associated with infection that only affect the eyes. In these cases treatment may be directed to the eye alone. Such treatment often starts with eye drops.

What infections cause uveitis?

A large variety of organisms may infect the eye: bacteria, viruses, fungi, worms, insect larvae, protozoa, and other parasites. Some infect the eye alone. Others infectious agents affect other parts of the body as well. Infectious agents may enter the eye from a cut or opening into the eye from an eye injury. This is called endophthalmitis. Urgent antibiotic treatment is required as the risk of permanent loss of vision is high.

In other types of infection, the organism enters the eye through the blood stream. An infectious agent may enter the body through a cut in the skin, through the gastro-intestinal tract, the uro-genital tract or through the lungs. Once it is in the body the organism may enter the blood vessels and travel to the eye. For example, toxoplasmosis is a parasite found in contaminated food that enters the gastro-intestinal tract. It then spreads to the eye through the blood stream and infects the retina.

Because a large variety of infectious agents may enter the eye, the patient must inform the doctor of possible exposure to infection and carefully complete a uveitis questionnaire. Sometimes, a medical specialist in infectious disease is consulted.

What systemic inflammatory conditions can affect the eye?

Many autoimmune conditions cause inflammation without infection. The immune system abnormally identifies the body as being “foreign.” The resultant inflammation may affect various organs of the body. For example, rheumatoid arthritis in an autoimmune condition that affect the joints and sometimes causes inflammation of the sclera (the white outer coat of the eye).

In order to identify an autoimmune disease, the doctor will ask many questions about inflammation outside the eye (uveitis questionnaire) and order appropriate tests.

What are inflammatory conditions that affect the eye alone?

Sometimes, the immune system attacks the eye without affecting other organs in the body. The underlying trigger or cause of inflammation cannot usually be found. These conditions are placed into categories that help plan treatment strategies. For example, anterior uveitis (inflammation of the front of the eye) is initially treated with anti-inflammatory eye drops. On the other hand, Birdshot Chorioretinitis (BSCR) is an inflammation of the back part of the eye that usually requires long-term systemic treatment (pills or injections in the skin). There are many different ocular inflammatory conditions, which are identified by tests ordered by the doctor.

What medications are used for uveitis?

The type of treatment depends on the cause and category of inflammation. Antibiotics are used if an infection is suspected. The doctor prescribes antibiotics by pill or IV (intravenous) if the infection affects organs outside the eye. The doctor prescribes eye drops, pills, and/or injections if the infection affects only the eye.

The eye doctor may consult a rheumatologist to help monitor treatment with a systemic anti-inflammatory medication (pills and/or injections) if an inflammation affects organs outside the eye. Sometimes, an inflammation only affecting the eye requires the use of systemic medication, too. The ophthalmologist may also use eye drops and painless eye injections to control the inflammation.

Anti-inflammatory eye drops include steroid eye drops and non-steroid eye drops. They may be used separately or together depending on the type of inflammation. Steroid eye drops may cause the intra-ocular pressure to rise and must be monitored. Non-steroid eye drops may irritate the cornea (the front window of the eye). Dilating drops are often used to minimize pain from inflammation and help prevent harmful scar tissue from damaging the iris (the brown or blue part on the front of the eye).

Steroid injections may be given next to the eye (subtenon’s injection) or into the eye (intravitreal injection). Anesthetics help prevent pain with injection. Steroid implant injections (Ozurdex and Iluvien) offer longer duration of effect. Steroid injections may be especially useful in the treatment of macular edema (swelling of the retina) in patients with uveitis.

Steroid pills are often used at the beginning of treatment to control severe inflammation. Prednisone is the most common medication used to treat uveitis. It is usually used at high starting doses and then is slowly tapered down to an acceptable dose for long-term use or is completely discontinued. Prednisone has unacceptable side effects if used in high doses for many months to years.

Non-steroidal anti-inflammatory drugs (NSAIDS) given as pills may provide steroid-free treatment for some cases of uveitis. Some are available over-the-counter. Others are available by prescription. They may adversely affect the stomach and kidney.

Immune system suppressants help to quell uveits. Methotrexate, azathioprine, mycophenolate mofetil, cyclosporin are often used safely and effectively. Routine blood tests help detect side effect before permanent damage occurs. Although there was concern of an increased risk of skin cancer and lymphoma due to immune suppression, the SITE extension study showed that cancer risk is not increased. Very strong medications are used in very severe inflammation that threatens life or blindness (cyclophosphamide and chlorambucil). Pregnancy is avoided while on immune suppressants.

Biologic medications are new and very effective in the treatment of uveitis. Humira is given at home as an injection under the skin every two weeks. Long-term effects are being studied.

By Scott E. Pautler, MD

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

Copyright © 2017-2022 Designs Unlimited of Florida. All rights reserved.

Ozurdex

Ozurdex

 

What is Ozurdex and why is it used in the eye?

Ozurdex is a very small tube-like implant that contains steroid medication that is slowly released into the eye for up to 3-6 months. The Ozurdex implant is injected into the eye in the office setting in order to decrease inflammation, swelling, or leaky blood vessels inside the eye. It has been shown to improve vision in eyes with retinal vein occlusion, diabetic macular edema, and inflammation in the eye. The Ozurdex implant completely dissolves in the eye. Repeated injections may be necessary for continued improvement in vision.

How is an Ozurdex injection performed?

Anesthetics are used to make the procedure pain-free. The eye is treated with an iodine solution in an effort to prevent infection and an instrument is used to gently keep the lids open during the injection. A pressure sensation is often felt as the implant is injected into the eye with a very thin, short needle. The procedure is very brief.

What are the side effects of Ozurdex?

Although possible, it is rare to see the Ozurdex implant floating in the vision. In about 30-40% of patients, the pressure inside the eye increases and requires eye drops. About 1% of patients require glaucoma surgery to prevent loss of vision. Most eyes develop cataract and eventually require surgery. Rare risks of steroid injection include bleeding, infection, retinal detachment, and loss of vision/loss of the eye. The risk of infection is about one in 1,000 injections. Please report any severe loss of vision to the doctor without delay.

How do I care for the eye?

You may be given eye drops and instructions on how to use them. Physical activity is not limited after Ozurdex injection. Tylenol or Ibuprofen may be used if there is discomfort, but severe pain should be reported to your doctor without delay. It is normal to experience a red area on the white of the eye, which disappears in one to two weeks. If you have any questions or concerns, please call the office. Additional information is available at www.ozurdex.com.

By Scott E. Pautler, MD

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

Copyright © 2015-2022 Designs Unlimited of Florida. All Rights Reserved.

Sub-Tenon’s Steroid Injection

Why is a sub-Tenon’s steroid injection performed?

A sub-Tenon’s steroid injection (STS) is an office procedure performed to decrease inflammation, swelling, or leaky blood vessels inside the eye. The steroid medicine acts to decrease inflammation and leakage from blood vessels from a variety of causes, thereby offering the opportunity for improvement in vision. The effect of STS lasts for several months after which repeated injection may be considered if necessary.

How is a sub-Tenon’s steroid injection performed?

Anesthetic solutions are used to make the procedure pain-free. A pressure sensation is often felt as the steroid is injected next to the eye with a very thin, short needle. The procedure is brief.

Periocular steroid injeciton
Sub-Tenon’s Steroid Injection (click on image to enlarge)

Will the injection affect my vision?

The vision may be slightly blurred immediately after an injection. The anticipated improvement in vision occurs slowly over a period of weeks to months. Sometimes, the pressure inside the eye increases and may require eye drops for several months. There may also be an increased rate of cataract formation. It is common for the upper lid to droop slightly; this improves over several months.  Rare risks of steroid injection include bleeding, infection, retinal detachment, glaucoma, and loss of vision. Please report any severe loss of vision to the doctor without delay.

How do I care for the eye after injection?

If a patch is placed on the eye, keep it on as directed by the doctor, usually 2-3 hours. You may be given eye drops and instructions on how to use them. Physical activity is not limited after STS. Tylenol or Ibuprofen may be used if there is discomfort after the injection, but severe pain should be reported to your doctor without delay. It is normal to experience a red area on the white of the eye, which disappears in one to two weeks. If you have any questions or concerns, please call the office.

By Scott E. Pautler, MD

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

Copyright  © 2013-2022 Designs Unlimited of Florida.  All Rights Reserved.