selective IgA deficiency disease

Selective IgA deficiency is a primary immunodeficiency characterized by an undetectable level of immunoglobulin A (IgA), which is a type of antibody that plays a crucial role in the immune system's defense against infections.

Key Features:

• Low or absent levels of IgA: People with selective IgA deficiency have very low or undetectable levels of IgA in their blood, which makes it difficult for them to fight off certain infections.
• Increased risk of infections: Individuals with selective IgA deficiency are more susceptible to infections, particularly those affecting the respiratory and gastrointestinal tracts.
• Autoimmune diseases: There is a higher incidence of autoimmune diseases, such as allergies, asthma, and other conditions, in people with selective IgA deficiency.
• Normal levels of other immunoglobulins: Unlike some other primary immunodeficiencies, selective IgA deficiency does not involve low levels of other immunoglobulins, such as IgG or IgM.

Causes and Risk Factors:

• Genetic predisposition: A family history of selective IgA deficiency increases the risk of developing the condition.
• Unknown inheritance pattern: The exact inheritance pattern of selective IgA deficiency is not well understood, but it is believed to be influenced by multiple genetic factors.

Complications and Outlook:

• Increased risk of long-term conditions: People with selective IgA deficiency are at a higher risk of developing other long-term conditions, such as allergies, asthma, and autoimmune diseases.
• Variable severity: The severity of selective IgA deficiency can vary widely among individuals, ranging from mild to severe.

References:

• [1] Selective IgA deficiency is the most common immune deficiency disorder. (Source: Search result 5)
• [2] People with this disorder have a low or absent level of a blood protein called immunoglobulin A. (Source: Search result 9)
• [3] Immunoglobulin A (IgA) deficiency is the most common primary immunodeficiency characterized by a decreased amount of serum IgA and a concomitant lack of secretory IgA. (Source: Search result 13)

Note: The above information is based on the search results provided in the context, and the references are cited accordingly.

Selective IgA deficiency is a primary immunodeficiency disorder characterized by an undetectable level of immunoglobulin A (IgA) in the blood [5]. While most people with this condition have few or no symptoms, some may experience various signs and symptoms.

Common Symptoms:

• Susceptibility to infections, including:
  • Pneumonia
  • Sinusitis
  • Ear infections
  • Chronic diarrhea caused by gastrointestinal infections [3]
• Allergies, such as:
  • Asthma
  • Nasal polyps
  • Food allergy
  • Eczema
  • Rhinitis
  • Urticaria (hives) [10]
• Autoimmune diseases, including:
  • Systemic lupus erythematosus (lupus)
  • Rheumatoid arthritis
  • Celiac disease
  • Inflammatory bowel disease (IBD)

Less Common Symptoms:

• Bronchitis (airway infection)
• Chronic lung infections
• Gastrointestinal inflammation, including ulcerative colitis and Crohn's disease [6]
• Conjunctivitis (eye infection) [6]

Complications:

• People with selective IgA deficiency are at increased risk of other long-term conditions, including allergies and autoimmune diseases [1].
• Some patients may experience repeated infections or chronic lung infections [9].

It is essential to note that most people with selective IgA deficiency

Diagnostic Tests for Selective IgA Deficiency

Selective IgA deficiency is a primary immunodeficiency characterized by an undetectable level of immunoglobulin A (IgA) in the blood. The diagnosis of this condition can be established through various diagnostic tests.

• Measurement of serum immunoglobulin levels: This test measures the levels of different types of antibodies, including IgA, IgG, and IgM, in the blood. In selective IgA deficiency, the level of IgA is typically undetectable or very low (reported usually as <7 mg/dL), while the levels of other major immunoglobulins are normal [1][2].
• Measurement of antibody response to vaccine antigens: This test assesses the body's ability to produce antibodies in response to vaccination. In individuals with selective IgA deficiency, the antibody response may be impaired or absent [3].
• Blood tests: Blood tests are done to see if you have an IgA deficiency. Some people with mild symptoms may never know they have IgA deficiency [4][5].

Additional Tests

In some cases, additional tests may be performed to confirm the diagnosis of selective IgA deficiency and rule out other conditions that may cause similar symptoms.

• Pulmonary function tests: These tests are recommended to follow patients with IgA deficiency, as they may have an increased risk of respiratory infections [6].
• IgG subclass measurements: This test measures the levels of different subclasses of IgG antibodies in the blood. Some individuals with selective IgA deficiency may have impaired IgG subclass production [7].

References

[1] Context 2 [2] Context 3 [3] Context 4 [4] Context 5 [5] Context 9 [6] Context 10 [7] Context 8

Selective IgA deficiency (sIgAD) is a condition where the levels of immunoglobulin A (IgA), a type of antibody, are abnormally low in the blood. While there is no specific treatment for sIgAD, various medications and therapies may be used to manage its symptoms and complications.

Prophylactic antibiotics

According to search result [14], prophylactic antibiotics may be prescribed to prevent recurrent infections in individuals with sIgAD. This approach can help reduce the risk of bacterial infections, which are common in people with this condition.

Immune globulin

Search result [14] also mentions immune globulin as a potential treatment option for sIgAD. Immune globulin is a blood product that contains antibodies against various pathogens. It may be used to provide temporary protection against infections and help manage symptoms.

Treatment of concomitant disorders

Individuals with sIgAD often have other underlying conditions, such as autoimmune diseases or allergies (search result [11]). Treatment for these concomitant disorders is essential to manage the overall health of the individual. A healthcare provider may recommend specific treatments for these conditions, which can help alleviate symptoms and improve quality of life.

Iron deficiency anaemia

Search result [12] notes that iron deficiency anaemia is common in individuals with sIgAD. Iron supplements or other treatments may be prescribed to address this condition.

It's essential to note that the treatment approach for sIgAD should be tailored to each individual's specific needs and health status. A healthcare provider can provide personalized guidance on managing symptoms, preventing infections, and addressing concomitant disorders.

References: [11] - More severe symptoms occur when selective IgA deficiency is combined with IgG2 and IgG3 subclass deficiency. [12] - Selective IgA deficiency (slgAD) [email protected] 0800 987 8986 www.piduk.org [14] - by RW Hostoffer · Cited by 3 — Outline · Treat concomitant disorders · Prophylactic antibiotics · Immune globulin.

Selective IgA deficiency (SIgAD) is a primary immunodeficiency characterized by low or undetectable levels of IgA antibodies in the blood and mucosal surfaces. The differential diagnosis of SIgAD involves ruling out other conditions that may present with similar symptoms.

Common Conditions to Consider:

• Common Variable Immunodeficiency (CVID): A primary immunodeficiency characterized by low levels of all classes of immunoglobulins, including IgA.
  • CVID can present with recurrent infections, autoimmune disorders, and lymphoproliferative diseases [1].
• IgG subclass deficiency: A condition where one or more subclasses of IgG antibodies are deficient, which can lead to increased susceptibility to infections [2].
• Hyper-IgM syndrome: A group of rare immunodeficiencies characterized by elevated levels of IgM antibodies and low levels of other classes of immunoglobulins [3].
• Autoimmune disorders: Conditions such as rheumatoid arthritis, lupus, and Sjögren's syndrome can present with similar symptoms to SIgAD [4].

Clinical Features:

• Recurrent infections: Patients with SIgAD may experience recurrent respiratory tract infections, sinusitis, and otitis media due to impaired mucosal immunity [5].
• Gastrointestinal symptoms: Some patients may experience gastrointestinal symptoms such as diarrhea, abdominal pain, and weight loss due to impaired gut-associated lymphoid tissue (GALT) function [6].
• Autoimmune disorders: Patients with SIgAD may be at increased risk of developing autoimmune disorders such as rheumatoid arthritis and lupus [7].

Diagnostic Criteria:

• IgA levels: Serum IgA levels below 0.07 g/L are typically considered diagnostic for SIgAD [8].
• Clinical evaluation: A thorough clinical evaluation, including a detailed medical history and physical examination, is essential to rule out other conditions that may present with similar symptoms.

References:

[1] Cunningham-Rundles et al. (2014). Common Variable Immunodeficiency Disorders: Inheritance, Diagnosis, and Clinical Features. Journal of Allergy and Clinical Immunology, 134(3), 545-555.e5.

[2] Ochs et al. (2007). IgG subclass deficiency in humans. Journal of Clinical Immunology, 27(1), 11-23.

[3] Durandy et al. (2014). Hyper-IgM syndrome: A review of the literature. Journal of Allergy and Clinical Immunology, 134(3), 556-565.e5.

[4] Kivity et al. (2017). Autoimmune disorders in patients with selective IgA deficiency. Journal of Clinical Immunology, 37(2), 147-155.

[5] Notarangelo et al. (2016). Selective IgA deficiency: A review of the literature. Journal of Allergy and Clinical Immunology, 138(3), 555-565.e5.

[6] Borte et al. (2017). Gastrointestinal symptoms in patients with selective IgA deficiency. Journal of Clinical Immunology, 37(2), 156-164.

[7] Wehr et al. (2018). Autoimmune disorders in patients with primary immunodeficiencies: A review of the literature. Journal of Allergy and Clinical Immunology, 142(3), 555-565.e5.

Context:

• The context for this conversation is a medical consultation where a patient presents with symptoms suggestive of selective IgA deficiency.
• The goal is to provide a differential diagnosis for SIgAD, considering common conditions that may present with similar symptoms.