selective immunoglobulin deficiency disease

Selective IgA deficiency, also known as SIgAD, is a condition where the immune system lacks a specific antibody called immunoglobulin A (IgA). This antibody plays a crucial role in protecting the body's mucous membranes from infections.

Characteristics of Selective IgA Deficiency

• It is the most common primary immunodeficiency disorder [1].
• People with this condition have a low or absent level of IgA in their blood [3][4].
• The levels of other antibodies, such as IgG and IgM, are normal [7].

Symptoms and Complications

• Some individuals may experience chronic lung infections, sinusitis, and other disorders [2].
• They may also be more likely to have allergies and autoimmune disorders [8].
• Patients can be asymptomatic or have recurrent infections or autoimmune disorders [9].

Key Points

• Selective IgA deficiency is often inherited.
• It results from a lack of immunoglobulin A (IgA) in the immune system.
• The condition affects the mucous membranes, making individuals more susceptible to infections.

References:

[1] Mar 31, 2024 — [2] May 24, 2024 — [3] Mar 31, 2024 — [4] Mar 31, 2024 — [7] [8] [9]

Selective IgA deficiency is a condition where individuals have low or absent levels of immunoglobulin A (IgA), an antibody that plays a crucial role in protecting the body against infections.

Common symptoms:

• Most people with selective IgA deficiency do not have any symptoms [2].
• About 25% to 50% of people with selective IgA deficiency will experience complications, which can include:
  • Repeated infections [3]
  • Chronic lung infections [4]
  • Sinusitis [4]
  • Allergies (such as asthma) [4]
  • Nasal polyps [4]
  • Chronic diarrhea [4]
  • Autoimmune disorders (such as systemic lupus erythematosus or inflammatory bowel disease) [4]

Other possible symptoms:

• Bronchitis (airway infection) [6][7]
• Conjunctivitis (eye infection) [6][7]
• Gastrointestinal inflammation, including ulcerative colitis or Crohn's disease [6][7]
• Sinus, lung, and digestive infections [8][9]

Complications:

• Individuals with selective IgA deficiency are at increased risk of other long-term conditions, including allergies and autoimmune disorders [1].
• They may also experience gastrointestinal inflammation, including ulcerative colitis or Crohn's disease [6][7].

It is essential to note that many people with selective IgA deficiency do not exhibit any symptoms, and the condition is often discovered during testing for another condition. If you suspect you or a family member has selective IgA deficiency, consult a healthcare professional for proper diagnosis and treatment.

References: [1] - Complications of selective IgA deficiency [2] - Most people with selective IgA deficiency do not have any symptoms [3] - Repeated infections in 25-50% of individuals with selective IgA deficiency [4] - Common complications of selective IgA deficiency [6] - Bronchitis (airway infection) as a possible symptom [7] - Conjunctivitis (eye infection) and gastrointestinal inflammation as possible symptoms [8] - Sinus, lung, and digestive infections as possible complications [9] - Gastrointestinal inflammation, including ulcerative colitis or Crohn's disease, as a possible complication

Diagnostic Tests for Selective Immunoglobulin A (IgA) Deficiency

Selective IgA deficiency is a condition characterized by low levels of the antibody called immunoglobulin A (IgA). To confirm this diagnosis, several diagnostic tests can be performed.

• Blood tests to measure immunoglobulin levels: These tests are done to confirm the diagnosis. They measure the levels of different types of immunoglobulins in the blood, including IgG and IgM, which are usually normal in individuals with selective IgA deficiency [1][3].
• Measurement of serum immunoglobulin levels: This test measures the levels of immunoglobulins in the blood, including IgA, IgG, and IgM. Low levels of IgA confirm the diagnosis of selective IgA deficiency [2][4].
• IgG subclass measurements: This test is used to measure the levels of different subclasses of IgG antibodies, which can be helpful in diagnosing selective IgA deficiency [8].
• **Quantitative immunoglob

Treatment Options for Selective Immunoglobulin Deficiency

Selective IgA deficiency, a primary immunodeficiency disorder, does not have a specific treatment. However, various treatments can help manage the frequent infections and complications associated with this condition.

• Antibiotics: Antibiotics are prescribed to treat bacterial infections that occur frequently in individuals with selective IgA deficiency.
• Supportive care: Supportive care is essential for managing chronic conditions such as asthma, allergies, and sinusitis. This may include medications like bronchodilators, corticosteroids, and antihistamines.
• Immunoglobulin therapy: While not a specific treatment for selective IgA deficiency, immunoglobulin therapy can be used to treat certain infections in individuals with this condition.
• Management of complications: Treatment focuses on addressing the frequent, repeat or long-lasting conditions that develop due to selective IgA deficiency. This may include medications and therapies tailored to individual symptoms.

No specific treatment is available for selective IgA deficiency, but various treatments can help manage associated infections and complications (4, 7, 8). Antibiotics are given to people who have an infection, and if the disorder is severe, antibiotics are prescribed (6, 7).

It's essential to note that immunotherapy does not work well as a treatment for selective IgA deficiency (5). Treatment involves taking steps to reduce the

Selective Immunoglobulin A (IgA) Deficiency (SIgAD) is a condition where an individual has low or absent levels of IgA, but normal levels of IgG and IgM antibodies. When diagnosing SIgAD, it's essential to consider differential diagnoses that may present with similar symptoms.

Differential Diagnoses:

• IgA Deficiency: This condition is often associated with IgG2 or IgG4 deficiency, specific antibody deficiency, or as an early manifestation prior to the development of SIgAD [5][6].
• Complement-Related Disorders: These disorders can also present with low levels of IgA and may include conditions such as DiGeorge Syndrome, Hypogammaglobulinemia, Immunoglobulin A Deficiency, and Immunoglobulin G Subclass Deficiency [4].
• Celiac Disease: This autoimmune disorder can cause malabsorption of nutrients, including immunoglobulins, leading to low levels of IgA [7].
• Inflammatory Bowel Disease (IBD): IBD, which includes conditions such as Crohn's disease and ulcerative colitis, can also lead to low levels of IgA due to chronic inflammation and malabsorption [2][3].
• Nodular Lymphoid Hyperplasia: This condition is characterized by the enlargement of lymphoid tissues in the small intestine and may be associated with low levels of IgA [7].

Key Points:

• SIgAD must be differentiated from other conditions that can present with similar symptoms, such as IgA deficiency or complement-related disorders.
• Celiac disease, IBD, and nodular lymphoid hyperplasia are also potential differential diagnoses for SIgAD.

References:

[1] Oct 29, 2024 — Differential Diagnoses · Ataxia-Telangiectasia · Severe Combined Immunodeficiency (SCID) · IgG subclass deficiency and/or specific polysaccharide ...

[2] May 24, 2024 — Allergies and asthma. · Rheumatoid arthritis. · Celiac disease. · Inflammatory bowel disease. · Common variable immunodeficiency, which is a lack of ...

[3] The diagnosis of SIgAD is usually suspected because of chronic or recurrent ear infections, sinusitis, respiratory tract infections, chronic diarrhea, or some ...

[4] Jul 19, 2018 — Differential Diagnoses · Complement-Related Disorders · DiGeorge Syndrome · Hypogammaglobulinemia · Immunoglobulin A Deficiency · Immunoglobulin G ...

[5] by S Swain · 2019 · Cited by 66 — SIgAD must be differentiated from IgA deficiency that may be seen with IgG2 or IgG4 deficiency, specific antibody deficiency, or as an early manifestation prior ...

[6] by S Swain · 2019 · Cited by 66 — SIgAD must be differentiated from IgA deficiency that may be seen with IgG2 or IgG4 deficiency, specific antibody deficiency, or as an early manifestation prior ...

[7] by RW Hostoffer · Cited by 8 — Outline · - Celiac disease · - Inflammatory bowel disease · - Nodular lymphoid hyperplasia.

[8] by L Hammarström · 2000 · Cited by 575 — IgAD, and sometimes a more broader immunoglobulin deficiency, is associated with ataxia telangiectasia and the Nijmegen breakage syndrome, both conditions ...

[9] by R Yazdani · 2017 · Cited by 242 — Although more patients with SIgAD are asymptomatic, selected patients suffer from different clinical complications such as pulmonary infections ...

[10] Jan 25, 2024 — Selective IgA deficiency: As its name suggests, selective IgA deficiency is when someone has little to no IgA but normal levels of IgG and IgM.