Healthcare Professionals Site

Childhood-Onset Idiopathic Nephrotic Syndrome

Childhood-onset idiopathic nephrotic syndrome, also known as childhood-onset INS, is a rare kidney disease most commonly diagnosed in children 2 to 7 years of age. Idiopathic means the cause is unknown. In INS, the immune system can disrupt the kidneys’ filtering system, allowing too much protein to leak from the blood into the urine. Symptoms may include foamy urine, swelling, tiredness, and loss of appetite. Children with INS may also be at increased risk for infections, blood clots, high cholesterol, and long-term kidney problems. Most children initially respond to steroids, but INS often relapses, and repeated or long-term steroid use can lead to slowed growth, high blood pressure, and bone problems.

GAZYVA in Childhood-Onset Idiopathic Nephrotic Syndrome

GAZYVA® (obinutuzumab) is indicated to reduce the risk of relapse in adult and pediatric patients 2 years of age and older with frequently relapsing or steroid dependent childhood-onset idiopathic nephrotic syndrome (INS) who are in complete remission. GAZYVA is the first and only FDA-approved treatment for childhood-onset idiopathic nephrotic syndrome.

GAZYVA is a monoclonal antibody thought to work by targeting B cells, which are a type of immune cell and a main driver of inflammation in childhood-onset idiopathic nephrotic syndrome. GAZYVA is designed to find and attach to CD20, a protein found on the surface of B cells. Based on laboratory studies, GAZYVA destroys B cells more effectively than similar anti-CD20 monoclonal antibodies.

INSHORE Clinical Study

INSHORE was a Phase III randomized clinical study that compared GAZYVA with mycophenolate mofetil, also called MMF, in patients 2 years of age and older with frequently relapsing or steroid-dependent INS. The study was designed to maintain remission in patients at high risk of relapse while reducing steroid use. At Week 52, more patients were in complete remission with GAZYVA than with MMF. Complete remission meant no swelling or fluid buildup, very low protein in the urine, and without a relapse after Week 8. Patients treated with GAZYVA also had fewer total relapses compared with MMF.

How GAZYVA Is Given and Important Safety Information

GAZYVA is given as an intravenous, or IV, infusion by a healthcare professional. Treatment for childhood-onset idiopathic nephrotic syndrome includes 4 infusions over 6 months. Medicines are given before each infusion to help reduce the chance of infusion-related reactions, and blood counts should be checked regularly.

GAZYVA carries Boxed Warnings for hepatitis B virus reactivation and progressive multifocal leukoencephalopathy, a rare but serious brain infection. Other serious risks include infusion-related reactions, allergic reactions, tumor lysis syndrome, serious and fatal infections, low white blood cell counts, low platelet counts, and a serious blood-clotting disorder. Review the full side effects of GAZYVA, including serious side effects.

Important Safety Information and Indication

Indication

GAZYVA® (obinutuzumab) is a prescription medicine used to treat adults and children 2 years of age and older with childhood-onset idiopathic nephrotic syndrome (INS) whose condition relies on regular steroids (steroid dependent) or keeps coming back (frequently relapsing).

What is the most important safety information I should know about GAZYVA?

Tell your doctor right away about any side effects you experience. GAZYVA can cause side effects that can become serious or life-threatening, including:

  • Hepatitis B Virus (HBV): Hepatitis B can cause liver failure and death. If you have a history of hepatitis B infection, GAZYVA could cause it to return. You should not receive GAZYVA if you have active hepatitis B liver disease. Your doctor or healthcare team will need to screen you for hepatitis B before, and monitor you during and after, your treatment with GAZYVA. Sometimes this will require treatment for hepatitis B. Symptoms of hepatitis B include worsening of fatigue and yellow discoloration of skin or eyes
  • Progressive Multifocal Leukoencephalopathy (PML): PML is a rare and serious brain infection caused by a virus. PML can be fatal. Your weakened immune system could put you at risk. Your doctor will watch for symptoms. Symptoms of PML include confusion, difficulty talking or walking, dizziness or loss of balance, and vision problems
Who should not receive GAZYVA?

  • Do NOT receive GAZYVA if you have had an allergic reaction (eg, anaphylaxis or serum sickness) to GAZYVA. Tell your healthcare provider if you have had an allergic reaction to obinutuzumab or any other ingredients in GAZYVA in the past

What are additional possible serious side effects of GAZYVA?

Tell your doctor right away about any side effects you experience. GAZYVA can cause side effects that may become severe or life-threatening, including:

  • Infusion-Related Reactions (IRRs): These side effects may occur during or within 24 hours of any GAZYVA infusion. Some IRRs can be serious, including, but not limited to, severe allergic reactions (anaphylaxis), acute life-threatening breathing problems, or other life-threatening IRRs. If you have a reaction, the infusion is either slowed or stopped until your symptoms are resolved. Most patients are able to complete infusions and receive medication again. However, if the infusion-related reaction is life-threatening, the infusion of GAZYVA will be permanently stopped. Your healthcare team will take steps to help lessen any side effects you may have to the infusion process. You may be given medicines to take before each GAZYVA treatment. Symptoms of IRRs may include fast heartbeat, tiredness, dizziness, headache, redness of the face, nausea, chills, fever, vomiting, diarrhea, rash, high blood pressure, low blood pressure, difficulty breathing, and chest discomfort
  • Hypersensitivity Reactions, Including Serum Sickness: Some people receiving GAZYVA may have severe or life-threatening allergic reactions. This reaction may be severe, may happen during or after an infusion, and may affect many areas of the body. If an allergic reaction occurs, your doctor will stop the infusion and permanently discontinue GAZYVA
  • Serious, Including Fatal, Infections: While you’re taking GAZYVA, you may develop infections. Some of these infections can be fatal and severe, so be sure to talk to your doctor if you think you have an infection. Patients with a history of recurring or chronic infections may be at an increased risk of infection. Patients with an active infection should not be treated with GAZYVA. Patients taking GAZYVA may be at higher risk for severe infections compared to patients taking mycophenolate mofetil. If you develop a serious infection, your doctor will immediately discontinue GAZYVA and begin treatment for the infection
  • Low White Blood Cell Count: When you have an abnormally low count of infection-fighting white blood cells, it is called neutropenia. While you are taking GAZYVA, your doctor will do blood work to check your white blood cell count. Severe and life-threatening neutropenia can develop during or after treatment with GAZYVA. Some cases of neutropenia can last for more than one month. If your white blood cell count is low, your doctor may prescribe medication to help prevent infections
  • Low Platelet Count: Platelets help stop bleeding or blood loss. GAZYVA with chemotherapy may reduce the number of platelets you have in your blood; having low platelet count is called thrombocytopenia. This may affect the clotting process. While you are taking GAZYVA, your doctor will do blood work to check your platelet count. Severe and life-threatening thrombocytopenia can develop during treatment with GAZYVA. Fatal bleeding events have occurred in patients treated with GAZYVA. If your platelet count gets too low, your treatment may be delayed or reduced
  • Disseminated Intravascular Coagulation (DIC): Fatal and severe DIC has been reported in people receiving GAZYVA for chronic lymphocytic leukemia (CLL) and non-Hodgkin lymphoma (NHL). DIC is a rare and serious abnormal blood clotting condition that should be monitored and managed by your doctor, as it can lead to uncontrollable bleeding

The most common side effects of GAZYVA in childhood-onset INS were upper respiratory tract infection, infusion-related reactions, ear infection, pneumonia, influenza, neutropenia, bronchitis, COVID-19, conjunctivitis, and urinary tract infection.

What other information should I tell my doctor before receiving GAZYVA?

You should talk to your doctor about:

  • Immunizations: Before receiving GAZYVA therapy, tell your healthcare provider if you have recently received or are scheduled to receive a vaccine. People who are treated with GAZYVA should not receive live vaccines. In patients with childhood-onset INS, live vaccines should be received at least 28 days prior to treatment with GAZYVA
  • Pregnancy: Tell your doctor if you are pregnant, think that you might be pregnant, or plan to become pregnant. GAZYVA may harm your unborn baby. Speak to your doctor about using GAZYVA while you are pregnant. Talk to your doctor or your child’s doctor about the safety and timing of live virus vaccinations to your infant if you received GAZYVA during pregnancy. Women of childbearing potential should use effective contraception while taking GAZYVA and for 6 months after GAZYVA treatment
  • Breastfeeding: Because of the potential risk of serious side effects in breastfed children, women should not breastfeed while taking GAZYVA and for 6 months after your last dose

Tell your doctor about any side effects.

These are not all of the possible side effects of GAZYVA. For more information, ask your doctor or pharmacist.

GAZYVA is available by prescription only.

You may report side effects to the FDA at (800) FDA-1088 or www.fda.gov/medwatch. You may also report side effects to Genentech at (888) 835-2555.

Please see the full Prescribing Information for additional Important Safety Information, including Serious Side Effects.

 

+