Selective IgM deficiency

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Overview

Selective IgM deficiency (also called selective immunoglobulin M deficiency or SIgMD) is a rare immune system disorder in which the body produces abnormally low levels of immunoglobulin M (IgM), one of the five main types of antibodies. IgM is the first antibody your immune system makes when it encounters a new infection, so having too little of it can leave you more vulnerable to certain germs. People with this condition often experience repeated infections, particularly of the respiratory tract (such as sinusitis, bronchitis, and pneumonia), skin infections, and sometimes serious bloodstream infections. Some individuals may also develop autoimmune conditions, where the immune system mistakenly attacks the body's own tissues, or allergic diseases like asthma and eczema. The severity of selective IgM deficiency varies widely. Some people have frequent, severe infections that significantly affect their quality of life, while others may have very mild symptoms or even no symptoms at all and are only discovered through routine blood work. The condition can occur on its own (primary) or alongside other medical conditions (secondary). Diagnosis is based on blood tests showing low IgM levels while other immunoglobulin classes (IgG and IgA) remain normal. Treatment focuses on managing and preventing infections. This may include prompt use of antibiotics when infections occur, and in some cases, preventive (prophylactic) antibiotics for people who get infections very frequently. Immunoglobulin replacement therapy, which is commonly used for other antibody deficiencies, is not typically effective for selective IgM deficiency because standard immunoglobulin preparations contain mostly IgG rather than IgM. Vaccination is encouraged to help boost other parts of the immune response. Research into the underlying causes and better treatments is ongoing.

Also known as:

Key symptoms:

Frequent sinus infectionsRecurring ear infectionsRepeated lung infections or pneumoniaBronchitis that keeps coming backSkin infectionsSerious bloodstream infections (sepsis)Allergies or asthmaEczema or skin rashesAutoimmune problems such as joint pain or blood cell destructionChronic fatigueGastrointestinal infections or diarrheaMeningitis in severe casesSlow recovery from common illnesses

Clinical phenotype terms (50)— hover any for plain English
VerrucaeHP:0200043Raynaud phenomenonHP:0030880Decreased circulating total IgMHP:0002850Chronic fatigueHP:0012432Decreased circulating IgA concentrationHP:0002720Decreased circulating IgG concentrationHP:0004315Chronic sinusitisHP:0011109Nontuberculous mycobacterial pulmonary infectionHP:0032261Pulmonary tuberculosisHP:0032262Hashimoto thyroiditisHP:0000872Systemic lupus erythematosusHP:0002725
Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Selective IgM deficiency.

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No actively recruiting trials found for Selective IgM deficiency at this time.

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Search ClinicalTrials.gov ↗Join the Selective IgM deficiency community →

No specialists are currently listed for Selective IgM deficiency.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Selective IgM deficiency.

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Community

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Latest news about Selective IgM deficiency

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Questions for your doctor

Bring these to your next appointment

  • Q1.How severe is my IgM deficiency, and what does that mean for my risk of infections?,Should I be on preventive antibiotics, and if so, for how long?,Are there any vaccines I should receive or avoid?,How often should I have my immunoglobulin levels and immune function checked?,What signs of infection should prompt me to seek urgent medical care?,Could my IgM deficiency be related to any other immune or autoimmune condition?,Should my family members be tested for immune deficiencies?

Common questions about Selective IgM deficiency

What is Selective IgM deficiency?

Selective IgM deficiency (also called selective immunoglobulin M deficiency or SIgMD) is a rare immune system disorder in which the body produces abnormally low levels of immunoglobulin M (IgM), one of the five main types of antibodies. IgM is the first antibody your immune system makes when it encounters a new infection, so having too little of it can leave you more vulnerable to certain germs. People with this condition often experience repeated infections, particularly of the respiratory tract (such as sinusitis, bronchitis, and pneumonia), skin infections, and sometimes serious bloodstream