Autoimmune Kidney Disease Clinic

Up to two-thirds of children with systemic lupus erythematosus (SLE) develop inflammation (irritation and swelling) in the part of the kidneys that filters blood. This swelling is called glomerulonephritis or nephritis. It may damage kidneys so they no longer can remove waste from the blood or control the amount of fluids in the body. Without the right treatment, nephritis can harm the kidneys so much they can’t get better.

Anti-neutrophil cytoplasmic autoantibodies (ANCAs) are abnormal antibodies that cause infection-fighting cells to mistakenly attack small blood vessels in different parts of the body, including the kidneys. This causes swelling of the walls of blood vessels. It also limits blood flow to important organs like the kidneys. The two main types of ANCA vasculitis are microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA). GPA is also known as Wegener granulomatosis. Your doctor will regularly check your child’s blood and urine for signs of kidney problems. Early treatment can help prevent long-term damage to the kidneys.

Henoch–Schönlein purpura (HSP) causes irritation and swelling (inflammation) of the small blood vessels, which can result in a prominent skin rash, arthritis (inflammation of the joints), abdominal pain and nephritis (inflammation of the blood vessels within the kidney). It is one of the more common types of vasculitis (inflamed blood vessels) in children. Your doctor will measure your child’s blood pressure regularly and check for signs of kidney inflammation, such as blood or protein in the urine. Depending on how serious the condition is, your child may need a kidney biopsy and treatment with medication to calm the immune system. Long-term kidney disease (called glomerulonephritis) develops in about 5% of children with HSP. HSP mostly affects children ages 2 to 10, but it can also affect older children and teens. Early treatment can help prevent long-term damage to the kidneys.

In Goodpasture syndrome, abnormal antibodies (autoantibodies) attack the kidneys and lungs. This causes permanent damage that gets worse over time. This rare condition mostly affects young men but also happens in children. Treatments include medicine that calms down the immune system and a blood-cleaning therapy (called plasmapheresis) to remove the autoantibodies.

In this rare disease, irritation and swelling of the blood vessels (vasculitis) damages different organs in the body. If blood vessels to the kidneys are affected, polyarteritis nodosa causes high blood pressure and affects how well the kidneys work. It is usually treated with medicine to calm down the immune system. Careful monitoring is important because the medicines have side effects.

In sarcoidosis, immune system cells form lumps called granulomas. The lumps collect in various organs, sometimes including the kidneys. Sarcoidosis can harm the kidneys. It also may lead to kidney stones because of extra calcium in the urine. Not everyone has symptoms that need treatment. If your child does, your doctor may use a steroid medicine such as prednisone.

We carefully watch your child for symptoms that may be a sign of a flare-up of their disease. We regularly test their pee (urine) and blood for signs of any harm to the kidneys. We also check that their medicine is working as it should.

It is likely your child will need medicine to calm down their immune system (immunosuppression). It is taken by mouth or through a vein (IV, also called intravenous). IV drugs include cyclophosphamide and rituximab. Mycophenolate (CellCept) and azathioprine (Imuran) are taken in pill form. When possible, we limit use of steroids such as prednisone. They can weaken bones.

We work with your family on a kidney-healthy eating plan. The diet takes into account any medicine your child is taking. Vitamin D is especially important.

At Seattle Children’s we conduct research to help bring promising new treatments to patients faster. Your child may have the chance to take part in a clinical trial testing new ways to treat autoimmune kidney disease in children.

Having an autoimmune disease may affect your child’s view of themselves or their body. Children with these problems may feel they are different from other children, or they may have some different needs than most of their peers. They might feel lonely or depressed at times. Our team can help your child cope with feelings and concerns like these.