The importance of comprehensive care for C3G and primary IC-MPGN

C3G (complement 3 glomerulopathy) and primary IC-MPGN (immune complex membranoproliferative glomerulonephritis) are a pair of rare kidney diseases. Both are caused by an underlying problem with a part of the immune system called the complement system.

The complement system is made up of dozens of proteins that circulate in the body. These proteins switch on when they encounter something that is harmful to the body, like a germ or an infected cell. This starts a chain reaction that activates different components of the immune system, such as white blood cells and inflammatory substances.

When a person has C3G or primary IC-MPGN, abnormal activity in the complement system causes damage to the kidneys. Keep in mind that this is a simplified explanation of a complex biological process.

While C3G and IC-MPGN are distinct conditions, distinguishing between the two can be difficult due to overlapping symptoms. Also, the causes of these conditions are not fully understood. A healthcare provider will always be your best source of information about a diagnosis.

Primary versus secondary IC-MPGN

Some cases of IC-MPGN are associated with infection, autoimmune disorders, certain cancers, or blood disorders. These are known as secondary IC-MPGN because there is an identifiable association with another condition. With primary IC-MPGN, there is no identifiable cause or associated disease. Primary IC-MPGN is also known as idiopathic IC-MPGN.

Why C3G and IC-MPGN require comprehensive care

The kidneys are a pair of fist-sized organs located in the lower back that act as filters for the blood. As blood moves through the kidneys, it passes through a series of smaller blood vessels that remove waste products and excess fluid, which get directed to the bladder. Substances that need to remain in the blood circulate back into the bloodstream.

This is a complex, delicate, and vital process in the body. And when part of this process breaks down or stops working, many different problems can occur.

Symptoms and complications of C3G and IC-MPGN

• Blood in the urine (hematuria)
• Protein in the urine (proteinuria)
• Edema, or swelling in the lower limbs or other parts of the body (edema)
• High blood pressure
• High cholesterol
• Decreased ability for the kidneys to make urine
• Fatigue and weakness
• Difficulty concentrating
• Anemia
• Eye problems
• Heart problems
• Chronic kidney disease
• Kidney failure

There is no cure for either condition, and treatment focuses on controlling symptoms and preventing complications. This often includes medications, including medications to control blood pressure, cholesterol, edema, and protein in the urine, and medications that target the complement system.

Treatment for C3G and primary IC-MPGN is typically overseen by a nephrologist, a medical doctor that specializes in diagnosing and treating kidney diseases. While this specialist oversees treatment, treatment is a coordinated effort among several healthcare providers. This is referred to as multidisciplinary, interprofessional, or comprehensive care.

Other members of a treatment team can include:

• Primary care provider
• Specialty nurses
• Clinical dietitians
• Healthcare social workers
• Genetics counselors
• Pharmacists
• Cardiologists
• Ophthalmologists

Due to the rarity of these conditions, it’s important to seek out healthcare providers and healthcare centers with experience in treating rare kidney disease. To find a healthcare team that has the necessary experience to treat these conditions, look for nephrology departments at major academic medical centers, or check databases available at sites like NephCure and National Organization for Rare Disorders.