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.
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.
- Age older than 50
- Heart disease
- Race, Caucasians are more likely to get maculopathy
- Family history and genes
- High blood pressure
- 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.
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.
- 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.
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 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, 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 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.
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.
- 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 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.
|Dry age-related macular degeneration|
|Wet age-related macular degeneration|
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.
16 Cited Research Articles
Consumernotice.org adheres to the highest ethical standards for content production and references only credible sources of information, including government reports, interviews with experts, highly regarded nonprofit organizations, peer-reviewed journals, court records and academic organizations. You can learn more about our dedication to relevance, accuracy and transparency by reading our editorial policy.
- American Academy of Ophthalmology. (n.d.). Hereditary Maculopathies. Retrieved from https://www.aao.org/focalpointssnippetdetail.aspx?id=04233cba-5975-42fa-81a2-a87ae19edebd
- 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
- Bhagat, N. (2021, June 21). Pentosan Polysulfate Maculopathy. Retrieved from https://eyewiki.aao.org/Pentosan_Polysulfate_Maculopathy
- Boyd, K. (2021, January 26). What is Macular Degeneration? Retrieved from https://www.aao.org/eye-health/diseases/amd-macular-degeneration
- BrightFocus Foundation. (2019, January 5). Age-Related Macular Degeneration: Facts & Figures. Retrieved from https://www.brightfocus.org/macular/article/age-related-macular-facts-figures
- Cleveland Clinic. (n.d.). Age-Related Macular Degeneration. Retrieved from https://my.clevelandclinic.org/health/diseases/15246-age-related-macular-degeneration
- Cleveland Clinic. (n.d.). Macular Pucker. Retrieved from https://my.clevelandclinic.org/health/diseases/14207-macular-pucker
- Doiron, R.C. et al. (2020, January 7). Possible drug-induced, vision-threatening maculopathy secondary to chronic pentosan polysulfate sodium (Elmiron) exposure. Retrieved from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7012292/
- Hanif, A.M. & Jain, N. (2019, July 10). Clinical Pearls for a New Condition. Retrieved from https://www.reviewofophthalmology.com/article/clinical-pearls-for-a-new-condition
- Janssen Pharmaceuticals. (2020, June). Elmiron Prescribing Information. Retrieved from https://www.accessdata.fda.gov/drugsatfda_docs/label/2021/020193Orig1s015lbl.pdf
- Johns Hopkins. (n.d.). Photodynamic Therapy for Age-Related Macular Degeneration. Retrirved from https://www.hopkinsmedicine.org/health/treatment-tests-and-therapies/photodynamic-therapy-for-agerelated-macular-degeneration
- Mayo Clinic. (n.d.). Retinal diseases. Retrieved from https://www.mayoclinic.org/diseases-conditions/retinal-diseases/symptoms-causes/syc-20355825
- Micro Chirurgia Oculare. (n.d.). Maculopathy: Causes and Symptoms. Retrieved from https://www.microchirurgiaoculare.com/en/maculopathy/causes-and-symptoms/
- National Eye Institute. (2019, July 8). Macular Pucker. Retrieved from https://www.nei.nih.gov/learn-about-eye-health/eye-conditions-and-diseases/macular-pucker
- National Eye Institute. (n.d.). Diabetic retinopathy. Retrieved from https://www.nei.nih.gov/learn-about-eye-health/eye-conditions-and-diseases/diabetic-retinopathy
- Oxford Medicine Online. (2008). Maculopathy. Retrieved from https://oxfordmedicine.com/view/10.1093/med/9780199544967.001.0001/med-9780199544967-chapter-6