Overview
Immunoglobulin A1 (IgA1) deficiency is a condition where the body does not produce enough of a specific type of antibody called IgA1. IgA is an important part of the immune system that helps protect the surfaces of the body, especially the lining of the nose, throat, lungs, and gut. IgA comes in two subtypes: IgA1 and IgA2. In this condition, the IgA1 subtype is specifically reduced or absent while IgA2 may be normal or also low. It is important to note that this specific diagnosis code (Orphanet 99972) has been marked as obsolete, meaning it is no longer used as a standalone classification. Patients previously diagnosed with IgA1 deficiency are now generally classified under the broader category of Selective IgA Deficiency (SIgAD). Selective IgA deficiency is the most common primary immunodeficiency in humans. People with IgA deficiency may experience frequent infections, particularly of the sinuses, ears, and lungs. Some individuals may also develop allergies, asthma, or autoimmune conditions. However, many people with IgA deficiency have no symptoms at all and may never know they have the condition. There is no cure for IgA deficiency. Treatment focuses on managing infections promptly with antibiotics and addressing any associated conditions like allergies or autoimmune problems. Importantly, IgA cannot be replaced through standard immunoglobulin infusions, so prevention and early treatment of infections are key strategies.
Also known as:
Key symptoms:
Frequent sinus infectionsRecurring ear infectionsRepeated lung infections or pneumoniaChronic diarrheaAllergies such as hay fever or food allergiesAsthmaAutoimmune conditions like celiac disease or lupusFrequent colds and upper respiratory infectionsBronchitisSkin rashes or eczemaDigestive problemsNo symptoms at all in many cases
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 OBSOLETE: Immunoglobulin A1 deficiency.
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Specialists
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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 OBSOLETE: Immunoglobulin A1 deficiency.
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Caregiver Resources
NORD Caregiver Resources
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Mental Health Support
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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.How severe is my IgA deficiency, and do I have any IgA at all?,What is my risk of developing common variable immunodeficiency (CVID) over time?,Should I wear a medical alert bracelet about my IgA deficiency?,Do I need special blood products if I ever need a blood transfusion?,How often should I have my immunoglobulin levels rechecked?,Should my family members be tested for IgA deficiency?,When should I seek medical attention for an infection rather than treating it at home?
Common questions about OBSOLETE: Immunoglobulin A1 deficiency
What is OBSOLETE: Immunoglobulin A1 deficiency?
Immunoglobulin A1 (IgA1) deficiency is a condition where the body does not produce enough of a specific type of antibody called IgA1. IgA is an important part of the immune system that helps protect the surfaces of the body, especially the lining of the nose, throat, lungs, and gut. IgA comes in two subtypes: IgA1 and IgA2. In this condition, the IgA1 subtype is specifically reduced or absent while IgA2 may be normal or also low. It is important to note that this specific diagnosis code (Orphanet 99972) has been marked as obsolete, meaning it is no longer used as a standalone classification.