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Edited By : Sophia Clifton
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What Is Maculopathy?

Maculopathy is a disease that affects the macula, a part in the retina at the back of the eye. The macula is responsible for the ability to see color and have sharp central vision. Maculopathy is also called macular retinopathy or macular degeneration.

People who develop maculopathy don’t go completely blind but they lose central vision. If this gets worse, they may be classified as legally blind.

There are several types of maculopathy, but the most common type of maculopathy is age-related macular degeneration (AMD) — a maculopathy that develops as people age. There is no cure for maculopathy, but treatments include: Lifestyle changes, medications and nutritional supplements.

Causes

The most common cause of maculopathy is age. Over time, the macula degenerates, leading to vision loss. Researchers think that maculopathy may be caused by eye inflammation or abnormal growth of blood vessels that leak fluid into the retina and disrupt vision.

Health conditions, such as diabetes, may increase the risk of developing maculopathy. For example, diabetic maculopathy causes blindness in about 80 percent of people with diabetes, according to a study published on Oxford Medical Online.

Taking certain medications may also damage the retina, leading to maculopathy.

Risk factors and causes of maculopathy include:
  • Age older than 50
  • Obesity
  • Smoking
  • Heart disease
  • Race, Caucasians are more likely to get maculopathy
  • Family history and genes
  • High blood pressure
  • Diabetes
  • Diet high in saturated fat
  • High cholesterol
  • Certain medications, such as Elmiron (pentosan polysulfate sodium) and hydroxychloroquine

In June 2020, Janssen Pharmaceuticals — a subsidiary of Johnson & Johnson — added a pigmentary maculopathy warning to the drug label for the interstitial cystitis drug Elmiron According to studies, pigmentary maculopathy only occurs in people who used Elmiron.

People who took Elmiron and suffered maculopathy and vision loss filed Elmiron lawsuits against Johnson & Johnson claiming the company manufactured a defective drug and failed to warn the public of the risk.

Symptoms

Maculopathy symptoms vary by type and may not be noticeable until the disease progresses. In general, signs of maculopathy include blurry vision, difficulty adjusting to dim light and loss of central vision.

Common symptoms of maculopathy include:
  • Blurry or distorted vision
  • Spotty vision
  • Difficulty adjusting to dim light
  • Difficulty reading
  • Poor night vision
  • Loss of color vision
  • Straight lines that appear wavy
  • Impaired depth perception

Types of Maculopathy

Age-related macular degeneration is the most common type of maculopathy, but other types include: Pigmentary maculopathy, macular pucker, diabetic maculopathy and hereditary maculopathy.

Unique Pigmentary Maculopathy

Pigmentary maculopathy is a unique type of maculopathy linked to Elmiron use with common symptoms such as difficulty reading, adjusting to low light and blurred vision, according to Elmiron’s prescribing information. It has no cure.

Eye doctors began writing about pigmentary maculopathy in medical journals in 2018. Pigmentary maculopathy may be misdiagnosed as age-related macular degeneration, according to Drs. Adam M. Hanif and Nieraj Jain in Review of Ophthalmology. So far, doctors have only found this type of maculopathy in people who used Elmiron.

Eye damage from pigmentary maculopathy usually occurs with long-term use of Elmiron greater than three years, but cases have been reported with a shorter duration of use. Vision damage may continue years after people stopped taking Elmiron. Some people may experience severe, irreversible loss of vision.

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Age Related Macular Degeneration (AMD)

Age-related macular degeneration usually affects people older than 50, according to the American Academy of Ophthalmology. According to the BrightFocus Foundation, as many as 11 million Americans have some form of AMD.

There are two types of AMD: Dry and wet. Dry AMD is the most common and is caused by thinning of the macula with age. Wet AMD is more serious but less common. It occurs when abnormal blood vessels grow and leak fluid into the macula.

People with wet AMD lose vision faster than those with dry AMD.

Macular Pucker

Macular pucker is caused when scar tissue forms on the macula. It pulls on the macula causing it to wrinkle. It causes distorted central vision, blurry vision, a gray spot in the center of your vision and difficulty reading fine print.

This type of maculopathy typically doesn’t get worse and it may affect one or both eyes.

Diabetic Maculopathy

Diabetic maculopathy, also called diabetic retinopathy, occurs when high blood sugar leads to retina damage. When sugar blocks the blood vessels in the eye, the body creates new vessels that may leak or bleed easily. Diabetic maculopathy can occur with any type of diabetes.

Early on, people might not notice symptoms, but when the blood vessels start to bleed, people may see dark floating spots or streaks that look like cobwebs. Later, vision loss becomes more severe. Untreated, it can lead to eye scarring and glaucoma.

Hereditary Maculopathy

Hereditary maculopathy is a type of disease that’s inherited. It’s also called juvenile macular degeneration. Common types of hereditary maculopathy include: Stargardt macular degeneration and vitelliform macular dystrophy (also called Best disease).

A fatty pigment building up in the macula causes Stargardt macular degeneration, and the disease gets progressively worse with time. Bright light can cause more fatty pigment to form.

Vitelliform macular dystrophy is rarer, and is caused by gene mutations that cause fatty buildup in the macula.

Diagnosing Maculopathy

Eye doctors diagnose maculopathy by conducting eye examinations to check for changes in the macula or retina. Depending on what they find, they may perform more detailed, specific tests.

Tests for maculopathy include:
  • Dilated eye exam. During this exam the doctor will put drops in your eye to dilate the pupils. Then, they will look inside the eye with a special lens.
  • Visual field test. Doctors may ask you to look at a grid full of lines called an Amsler grid. Distorted, wavy, blurry or broken lines may indicate worsening disease.
  • Fluorescein angiography. In this test, medical providers will inject a yellow dye into a vein in your arm. When the dye gets into blood vessels in the eye, doctors can see if there are any leaks in the macula.
  • Optical coherence tomography (OCT). A special imaging machine takes images of the back of the eye.
  • Optical coherence tomography angiography (OCTA). Doctors use laser light reflection and a special scanning device to produce 3D images of blood flow in the eye.

Maculopathy Treatments

Maculopathy can’t be cured or reversed. Maculopathy treatment focuses on slowing down disease progression and vision loss.

Most types of maculopathy are treated with medications, nutritional supplements, photodynamic therapy and, in some cases, surgery.

Types of Maculopathy and Treatments
TypeTreatment
Pigmentary maculopathy
  • Avoiding prolonged exposure to Elmiron

  • Drugs such as topical carbonic anhydrase inhibitors, corticosteroids, or nonsteroidal anti-inflammatory agents and oral acetazolamide

  • Anti-VEGF eye injections that slow production of new blood vessels in the eye

Dry age-related macular degeneration
  • Taking special vitamins and minerals called AREDS and AREDS2

  • Nutrient rich diets with dark leafy greens, yellow fruits, vegetables and fish

Wet age-related macular degeneration
  • Anti-VEGF eye injections that slow production of new blood vessels in the eye

  • Photodynamic therapy to clot and seal abnormal blood vessels

Diabetic maculopathy
  • Anti-VEGF eye injections that slow production of new blood vessels in the eye

  • Corticosteroids

  • Laser treatment to reduce swelling

  • Surgery to remove eye scarring

Macular pucker
  • Mild cases don’t need treatment

  • More serious cases may require surgery to remove gel-like fluid in the back of the eye, causing the macula to lie flat and improve vision problems

Hereditary maculopathy
  • Using hats and glasses to keep bright light out of the eyes and prevent excess fatty pigment from developing in the eyes (Stargardt macular degeneration)

  • Anti-VEGF eye injections that slow production of new blood vessels in the eye (vitelliform macular dystrophy)

  • Photodynamic therapy (vitelliform macular dystrophy)

Identifying maculopathy early with regular eye exams allows people to get early treatment and slow vision loss.

If you’re diagnosed with any type of maculopathy, make sure you seek a second or third opinion to prevent misdiagnosis and avoid treatment that isn’t effective or needed. For example, pigmentary maculopathy may be misdiagnosed as another eye condition such as age-related macular degeneration.

Please seek the advice of a qualified professional before making decisions about your health or finances.
Last Modified: September 28, 2021

16 Cited Research Articles

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  2. Astafurov, K.V. et al. (2021, April). Ocular Toxicity Associated With Pentosan Polysulfate Sodium. Retrieved from https://www.mayoclinicproceedings.org/article/S0025-6196(21)00312-8/fulltext
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