A study to evaluate the safety and effects of inebilizumab in children and adolescents with immunoglobulin G4-related disease

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What is this study about?

This study focuses on children and adolescents with Immunoglobulin G4-related Disease, which is a rare condition where the body’s immune system causes swelling and damage to various organs and tissues. The purpose of the study is to evaluate how the drug inebilizumab works in this specific age group. This medication is an intravenous infusion, meaning it is delivered directly into a vein through a tube.

The research will examine how the body processes the medication, which is known as pharmacokinetics, and how the drug affects the body’s biological functions, known as pharmacodynamics. During the study, researchers will also monitor the safety and how well the body tolerates the treatment. This involves checking how the drug impacts CD20+ B cells, which are a specific type of white blood cell involved in the immune response.

Participants will be monitored over a period of time to see how the medication affects the symptoms of the disease. The study will track how the drug behaves in the blood and observe any changes in the body’s reaction to the treatment. This includes monitoring for any side effects or changes in health indicators to ensure the medication is being used safely in children and teenagers.

Who Can Join the Study?

The person must weigh at least 17 kg.
A legal representative (such as a parent or guardian) must sign a consent form to allow participation, and the child must also agree to join the study by providing assent, which is a child’s way of showing they agree to participate.
The participant must be between 2 and 17 years old at the time they are checked for the study.
The person must have a confirmed medical diagnosis of Immunoglobulin G4-related Disease (IgG4-RD), which is a condition where the body’s immune system causes swelling and damage in various organs.
The patient must meet specific medical standards set by experts to confirm their diagnosis. This includes having the disease affect at least one specific organ, such as the pancreas, lungs, kidneys, or thyroid gland, and meeting a certain number of required medical points.
The participant must have received all vaccinations that are normally required for their age before starting the study.
The participant must need medical treatment for their condition that is either different from or in addition to glucocorticoids (GCs), which are a type of steroid medicine used to reduce swelling and inflammation.
If the participant is already taking glucocorticoids (GCs) for their condition, they must have been taking the same amount (a stable dose) for at least 2 weeks before the study begins.

Who Cannot Join the Study?

People with high levels of certain liver enzymes (proteins in the blood that show how the liver is working), specifically AST, ALT, or total bilirubin, if the disease is affecting the liver or gallbladder.
People who have received treatments like alemtuzumab, total lymphoid irradiation (radiation that targets white blood cells), bone marrow transplant, or T-cell vaccination therapy before the study starts.
People who have had malignancy (cancer), excluding certain types of skin cancer or early-stage breast or cervical cancer, within the last 5 years.
People who have taken specific medications such as azathioprine, mycophenolate mofetil, cyclosporine, methotrexate, cyclophosphamide, tocilizumab, satralizumab, eculizumab, or mitoxantrone within the last 2 months.
People who have used rituximab or other drugs that lower B-cells (a type of white blood cell that helps fight infection) or drugs that target B-cells within the last 6 months, unless their B-cell counts have returned to at least half of the lower limit of normal (the bottom end of a healthy range).
People who have received intravenous immunoglobulin (IVIG) (a treatment containing antibodies) within 1 month before the study.
People who have taken natalizumab within the last 6 months.
People with a history of severe anaphylaxis (a life-threatening allergic reaction) or severe allergies to two or more foods or medicines, including certain pain relievers or allergy medications.
People who have received a live or attenuated vaccine (a vaccine made from a weakened version of a germ) within 4 weeks, the BCG vaccine within 1 year, or a blood transfusion within 4 weeks before the study.
People currently participating in another study testing a new drug or medical device, or those who finished such a study too recently.
People of childbearing potential who are unwilling to use specific contraception (methods to prevent pregnancy) during the study and for 6 months after.
People with a history of immunodeficiency (a condition where the immune system is weak), such as from HIV or having the spleen removed, which makes it harder to fight infections.
People who are breastfeeding or plan to breastfeed during the study or for 6 months after the last dose.
People planning to become pregnant during the study or for 6 months after the last dose.
People who have had major surgery within the 8 weeks before the study begins.
People who have a positive pregnancy test at the start of the study.
People whose partners are unwilling to avoid pregnancy through abstinence or other methods during the study and for 6 months after.
Men assigned male at birth whose partner is pregnant and who are unwilling to use protection or abstain from sex during the study and for 6 months after.
People who are unwilling to stop donating sperm during the study and for 6 months after.
People who are allergic to any of the products used during the study doses.
People who may not be able to attend all required visits or follow all the study rules.
People with any other significant health problems that, in a doctor’s opinion, could make the study unsafe or difficult to complete.
People who test positive for chronic hepatitis B (a long-term liver infection caused by a virus).
People with evidence of significant problems with the liver (organ that cleans blood), kidneys (organs that filter waste), metabolism (how the body uses energy), or hematology (blood health), such as low platelets (cells that help blood clot), low neutrophils (white blood cells that fight infection), low immunoglobulins (antibodies), low CD4 T lymphocytes (specific immune cells), or low hemoglobin (protein in red blood cells that carries oxygen).
People with a low estimated glomerular filtration rate (a measure of how well the kidneys filter blood).
People with low B-cell counts.
People with another autoimmune disease (a condition where the immune system attacks the body) that is not well-controlled.
People with serious active infections (viral, bacterial, or fungal) that require medicine or hospital care within 2 months before the study.
People with a history of untreated hepatitis C (a liver infection) unless they have been cured and the virus is undetectable.
People with a history of tuberculosis (a serious bacterial infection) or a positive TB test unless they have completed treatment.
People who test negative for varicella zoster virus-IgG (antibodies that show past immunity to chickenpox).
People with high liver enzyme levels (AST, ALT, or bilirubin) when the disease is not currently active in the liver or gallbladder.
People with a history of alcohol or drug abuse that could affect their safety or ability to participate.
People who test positive for specific antibodies (proteins that recognize germs) known as anti-neutrophil cytoplasmic antibodies targeting certain proteins.

Where you can join this trial?

Verified and Recommended Sites

No sites found in this category

Verified Sites

Site Name	City	Country	Status
Hospital Universitario Y Politecnico La Fe	Valencia	Spain

Other Sites

Site Name	City	Country
Hospital Sant Joan De Deu Barcelona	Esplugues De Llobregat	Spain
Athens Euroclinic S.A.	Athens	Greece
Athens General Children’s Hospital Panagioti And Aglaia Kyriakou	Athens	Greece
Instytut Pomnik Centrum Zdrowia Dziecka	Warsaw	Poland
Ospedale Pediatrico Bambino Gesu’	Rome	Italy
Ngrlqpmf Izorqfcq Gbdtvhpda Rcixopomsypj I Rtpsgdotacqix Io Pxtri Dw Hmor Mgsm Elsztdis Rvnzkhj	Warsaw	Poland
Ssxsvce Stfgizuujlrcuwc Iwo Az Fepnckwnjoj Wb Wmesprgey scgaduokkmy puudasvym zostzi oasrdw zuztnsmhxp	Wroclaw	Poland

Trial status

Country	Status	Recruitment Start
Greece	Recruiting	15.03.2026
Italy	Not yet recruiting	15.03.2026
Poland	Not yet recruiting	15.03.2026
Spain	Not yet recruiting	15.03.2026

Trial locations

Investigated drugs:

Inebilizumab

Inebilizumab is a medication given through a vein that works by targeting and reducing specific immune cells in the body. In this study, it is being tested to see how it moves through the body, how it affects the immune system, and how safe and well-tolerated it is in children with a condition called Immunoglobulin G4-related disease.

Immunoglobulin G4-related disease – This is a condition where various organs and tissues become swollen due to an overactive immune response. It involves the buildup of specific cells and fibrous tissue in affected areas. The disease can impact several parts of the body, such as the pancreas, salivary glands, or lymph nodes. As it progresses, the inflammation can lead to the hardening of tissues in those locations. This process may cause the affected organs to function less effectively over time.

Trial ID:

2025-520988-41-00

Protocol code:

20240202

NCT ID:

NCT07222553

Trial Phase:

Therapeutic exploratory (Phase II)

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