Dense Deposit Disease

Dense deposit disease (DDD) is a rare and serious condition that mainly affects your kidneys and eyes. DDD belongs to a group of kidney conditions known as complement 3 glomerulopathies (C3G) that affect your immune system and kidneys. Experts think fewer than 5,000 people in the U.S. have this disease. Overall, C3G conditions affect an estimated 2 or 3 people out of every 1 million. Anyone can get DDD, but it most often occurs in children and young adults.

DDD damages the millions of tiny kidney filters (glomeruli) that take out fluids and waste from your blood to produce urine (pee). This glomerular disease can also damage your eyes and increase your risk of developing age-related macular degeneration (AMD).

In about half of all cases, dense deposit disease leads to kidney (renal) failure. DDD has no cure, but early diagnosis and treatment can help slow its progression. In severe cases, when your kidneys stop working, you may need dialysis or a kidney transplant.

It may also be called:

• Membranoproliferative glomerulonephritis type 2
• Mesangiocapillary glomerulonephritis type 2
• MPGN 2

Why is it called dense deposit disease?

In dense deposit disease, immune system proteins attach to your glomeruli in ways they shouldn’t. They form deposits — thick, sausage-shaped patches. Over time, these deposits affect your kidneys’ ability to work as they should.

Dense deposit disease can also cause whitish-yellow deposits (drusen) on an inner layer of your eye called Bruch’s membrane. This membrane divides the colored part of your retina from your choroid. The choroid is a layer of tissue between your retina and the white part of your eye (sclera). These deposits can affect your vision.

Symptoms of dense deposit disease

Dense deposit disease affects your kidneys first. These symptoms may include:

• Blood in your pee (hematuria)
• Change in your peeing habits, especially peeing less frequently than usual (oliguria)
• Confusion or unusual fatigue
• High blood pressure
• High levels of protein in your pee (proteinuria)
• Kidney inflammation (acute nephritis)
• Loss of appetite
• Swelling (edema)

If DDD affects your eyes, you may experience:

• Color blindness
• Loss of vision
• Night blindness (nyctalopia)

Dense deposit disease causes

Healthcare providers don’t know the exact cause of dense deposit disease. They suspect it occurs due to a combination of genetic and immune system problems.

How doctors diagnose it

A kidney biopsy is the only way to diagnose dense deposit disease. Your kidney specialist (nephrologist) removes a small amount of kidney tissue and uses an electron microscope to look for unusual deposits.

What other tests can help with a diagnosis?

You may receive blood tests or antibody tests to figure out if your immune system is working properly. Your provider may also recommend genetic testing to provide more information that can help guide your treatment.

Is there a cure for dense deposit disease?

DDD has no cure, but treatments may slow the disease and treat kidney failure.

What treatments are used for it?

Your treatment for dense deposit disease depends on the severity of the disease and how it affects your kidneys. You may receive:

• ACE inhibitors
• Blood pressure medication
• Plasmapheresis or plasma exchange
• Eculizumab and other complement inhibitors

About half of all people with DDD experience end-stage kidney failure. This life-threatening condition is the last stage of kidney disease. When your kidneys stop working, specialists typically recommend:

• Dialysis
• Kidney transplant

Eye and vision problems often develop later in the disease. If this occurs, an eye care specialist (ophthalmologist) can help treat and manage symptoms.

When should I see my healthcare provider?

Alert your provider if you develop new or worsening symptoms.

You may wish to ask the following questions during your appointments:

• Do you think I’ll have end-stage kidney failure?
• Do you think I’ll develop eye and vision problems?
• What are the side effects of your recommended treatments?
• Are there any clinical trials for DDD that I may qualify for?
• Can you recommend any support groups?

What can I expect if I have dense deposit disease?

The outlook for dense deposit disease is typically poor. Kidney failure is common, usually within 10 years after diagnosis. Even in those who receive kidney transplants, DDD often comes back.

How do I take care of myself or my loved one with this condition?

Keep all medical appointments and closely follow your provider’s instructions. Your care team can support you in your health journey.

What is the difference between dense deposit disease and C3 glomerulonephritis?

Dense deposit disease and C3 glomerulonephritis (C3GN) are both types of C3 glomerulopathies. But they look different under an electron microscope.

DDD has dense deposits that look like ribbons in the glomerular basement membrane. C3GN doesn’t have dense deposits in the glomerular basement membrane.