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Types of Rare Kidney Disease

Gain insight into various types of rare kidney diseases, offering an understanding of each condition. Under the broad umbrella of rare kidney disease, these diseases can affect individuals across all demographics, with each person experiencing unique impacts.

Overview

Diseases that injure the glomeruli, the tiny filtering units within the kidney where blood is cleaned, are called glomerular diseases. Glomerular disease reduces the kidney’s ability to maintain a balance of specific substances in the blood stream. The kidney’s job is to filter the bad toxins in the blood from the good proteins and red blood cells.

Glomerular disease causes the kidney to begin to retain the bad toxins and release the proteins and red blood cells from the body. Laboratory analysis of the urine from people who have glomerular disease often shows protein in the urine (proteinuria) and sometimes blood in the urine (hematuria). Glomerular diseases include many conditions with a variety of genetic and environmental causes.

Most glomerular diseases have specific names but might also be referred to as either:

Glomerulonephritis (gloh-MEHR-yoo-loh-nef-RY-tis) which describes the inflammation of the membrane tissue in the glomerulus of the kidney that serves as a filter, separating wastes and extra fluid from the blood.
Glomerulosclerosis (gloh-MEHR-yoo-loh-skleh-ROH-sis) describes the scarring or permanent damage to the tiny blood vessels within the kidney.

Although glomerular diseases may have different causes, they can all lead to kidney failure. Symptoms of these diseases may be abrupt or slowly progressive. Treatment options are available for some types of glomerular disease, but many of these therapies are toxic, non-specific, and have potential for major side effects. Some forms of glomerular disease do not respond to any therapies. It is important to seek continuous medical attention if you are affected by a glomerular disease.

Learn More About Your Diagnosis

Click on the boxes below to discover more about your rare kidney disease diagnosis.

Nephrotic Syndrome (NS)

Nephrotic Syndrome (NS) is not a disease itself, but rather an umbrella term for the collection of signs and symptoms that result from damage in the kidney’s filters, called glomeruli. This damage causes protein to leak into the urine, a condition called proteinuria.

Focal Segmental Glomerulosclerosis (FSGS)

FSGS is a term that describes a pattern of scarring in the kidneys. This scarring can be seen on a biopsy in patients with increased levels of protein in their urine.

IgA Nephropathy (IgAN)

A kidney disease in which Immunoglobulin A (IgA) builds up in the kidney. IgA is a protein in the blood. IgA is also part of the immune system. Excess IgA can cause inflammation in the kidney. Over time, this leads to scarring in the kidney tissue.

Minimal Change Disease (MCD)

A disorder affecting the filtering units of the kidney (glomeruli) that can lead to symptoms associated with Nephrotic Syndrome. It is one of the most common causes of Nephrotic Syndrome in children worldwide and can also be diagnosed in adults.

Apolipoprotein L1 (APOL1)

A collection of kidney diseases associated with the presence of specific forms of the APOL1 gene in a person’s DNA. These specific APOL1 gene variants are believed to have arisen in people in Western Africa.

Membranous Nephropathy (MN)

A kidney disease that causes injury to the small filters (glomeruli) in kidney tissue. It occurs when the person makes antibodies that damage kidney cells which form part of the kidney filters.

C3 Glomerulopathy (C3G & IC-MPGN)

A very rare and chronic disease that causes the kidneys to not work properly. C3G used to be called Membranoproliferative Glomerulonephritis (MPGN) 1, 2, or 3, but healthcare professionals have changed how they classify and talk about this disease.

Congenital Nephrotic Syndrome (CNS) & Infantile Nephrotic Syndrome (INS)

A rare kidney disorder that begins at birth or within the first 3 months of life. The majority of CNS cases are caused by genetic changes that cause defects in the filtering units of the kidneys.

C1q Nephropathy (C1q)

One of the many diseases causing Nephrotic Syndrome. C1q is a normal protein of the immune system and is found throughout the circulatory system. In an individual with C1q Nephropathy the protein is also found deposited in the kidneys.

IgM Nephropathy (IgM)

An autoimmune disease that affects the filters (gomeruli) of the kidneys. IgM is an immunoglobulin, which is a part of an individual’s healthy immune system. Immunoglobulin M is an antibody produced by B cells.

How Can NephCure Help?

We offer patient navigation services that help patients and their families with healthcare access, insurance headaches, seeking a second opinion, access to medications, school support services, and more. We push RKD research and care forward while connecting patients, families, and caregivers to the resources and support they need. We serve those affected by rare, protein-spilling kidney diseases like Nephrotic syndrome, Focal segmental glomerulosclerosis (FSGS), IgA nephropathy (IgAN), Membranous nephropathy (MN), Minimal change disease (MCD), C3 glomerulopathy (C3G), Immune complex membranoproliferative glomerulonephritis (IC-MPGN), APOL1 kidney disease and other rare, protein-spilling kidney diseases. Learn more about each disease and how we can support you.

Treatment Options

Learn more about the various medications often prescribed to those affected by rare kidney disease, including off label drugs as well as recently FDA-approved treatment options.

Find a Specialist

It is important to work with a nephrologist who specializes in RKD and can provide personalized advice based on your individual health needs and changes in available treatments. NephCure has a curated list of NephCure Specialists nationwide ready to help you.

Find Your Local Communities

Our local NephCure communities are groups of patients, care partners, and community members who want to learn, connect, and take action against rare kidney disease (RKD).

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