Overview
Primary immunodeficiency with predisposition to severe viral infection (Orphanet 431156) is a rare inherited disorder of the immune system. In healthy people, the immune system recognizes and fights off viruses quickly. In people with this condition, a specific part of the immune defense — the part that targets viruses — does not work properly. This leaves the body unable to control certain viral infections that most people handle without serious illness. People with this condition may experience repeated, unusually severe, or life-threatening infections caused by viruses such as herpesviruses (including Epstein-Barr virus, cytomegalovirus, and herpes simplex), influenza, and other common viruses. Bacterial and fungal infections may also occur, but viral vulnerability is the defining feature. The condition can vary widely in how severe it is from person to person. Treatment focuses on preventing infections through antiviral medications, immunoglobulin replacement therapy (giving the body protective antibodies from donors), and in some cases bone marrow or stem cell transplantation, which can offer a potential cure. Early diagnosis is critical because it allows protective treatments to begin before a dangerous infection occurs.
Key symptoms:
Repeated severe viral infections that are harder to recover from than normalUnusually severe or prolonged herpesvirus infections (cold sores, chickenpox, shingles, or mono)Severe or life-threatening influenza or other respiratory virus infectionsChronic or recurrent Epstein-Barr virus (EBV) or cytomegalovirus (CMV) illnessSwollen lymph nodes that persist or keep coming backExtreme fatigue that does not improve with restFever that keeps returning without a clear causeFailure to thrive or poor growth in infants and young childrenLung infections or pneumonia caused by virusesInflammation of the brain (encephalitis) from viral infectionLow levels of protective antibodies in the bloodReduced numbers or function of certain white blood cells (T cells or NK cells)
Variable
Can be inherited in different ways depending on the underlying gene
Variable
Can begin at different ages, from infancy through adulthood
Treatments
No FDA-approved treatments are currently listed for Primary immunodeficiency with predisposition to severe viral infection.
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Specialists
View all specialists →No specialists are currently listed for Primary immunodeficiency with predisposition to severe viral infection.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Primary immunodeficiency with predisposition to severe viral infection.
Community
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Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.Which specific gene is causing my (or my child's) immune problem, and what does that mean for treatment choices?,Should I (or my child) be on daily antiviral medication to prevent infections, and if so, which one?,Is immunoglobulin replacement therapy recommended, and would it be given as an infusion at a clinic or at home?,Is stem cell transplantation an option, and what are the risks and benefits in my specific situation?,Which vaccines are safe to receive, and which ones should be avoided?,What are the warning signs that mean I should go to the emergency room immediately?,Are other family members at risk, and should they be tested?
Common questions about Primary immunodeficiency with predisposition to severe viral infection
What is Primary immunodeficiency with predisposition to severe viral infection?
Primary immunodeficiency with predisposition to severe viral infection (Orphanet 431156) is a rare inherited disorder of the immune system. In healthy people, the immune system recognizes and fights off viruses quickly. In people with this condition, a specific part of the immune defense — the part that targets viruses — does not work properly. This leaves the body unable to control certain viral infections that most people handle without serious illness. People with this condition may experience repeated, unusually severe, or life-threatening infections caused by viruses such as herpesviruse