Recurrent infections are an indication of immune deficiency. How many infections are too many? Typically, children on average have about 4 to 8 infections per year depending on their environment as in day care settings and school such as: ear infections, sinus infections, cough, cold, pneumonia, bronchitis, skin infections. However, these infections seem common to warrant a red flag until how frequently they occur. Therefore, it is crucial to consult a physician when a person notices a pattern of infections.
Pattern of Infections: Have you ever wondered why a common cold always ends up becoming Pneumonia or a cough that never seems to end is diagnosed as Bronchitis or Asthma or those stomach issues causing discomfort, food refusals get labelled simply as picky eating? Some of the respiratory and gastrointestinal tract infections can lead to potentially serious complications if left untreated.
The immune system is the first line of defense against harmful germs and substances in human body. The main task of the immune system is to produce antibodies called immunoglobulins which in turn fight disease-causing germs like bacteria, viruses, parasites or fungi, and to remove them from the body. If the immune system stops working properly, you're more likely to get infections. One of the conditions that is often overlooked with repeat infections is Hypogammaglobulinemia.
What is Hypogammaglobulinemia?
Hypogammaglobulinemia refers to a group of immunodeficiencies characterized by low levels of immunoglobulins (Ig) or antibodies. It is a serious problem with the immune system that prevents the body from making enough antibodies compromising the bodies defense mechanism against germs that cause illnesses.
Hypogammaglobulinemia can be congenital (starting at birth) or acquired later in life as a result of another condition. These are further classified into primary and secondary immune deficiencies.
Primary Hypogammaglobulinemia: These are inherited caused by genetic disorders or chromosomal abnormalities during the development of the immune system. In adults, the two most common forms of primary hypogammaglobulinemia are common variable immune deficiency (CVID) and selective IgA deficiency.
Secondary Hypogammaglobulinemia: These are acquired conditions that occur as a result of external factors on the immune system such as a corticosteroid or immunosuppressant drugs, nutritional disorders, infections, chemotherapy, malignancy, nephrotic syndrome, other metabolic diseases, and hazardous environmental conditions.
Signs and Symptoms of Hypogammaglobulinemia?
The most apparent sign of an immune deficiency disorder is frequent, unusual and recurrent infections that appear excessive. Adults and children usually present with a history of recurrent infections with encapsulated bacteria. Most often, these bacterial infections affect the respiratory tract, causing recurrent sinusitis, otitis media, pharyngitis, bronchitis, and pneumonia. Some individuals experience increased hypersensitivity to allergens that mainly manifests as asthma, rhinitis, and dermatitis, as well as autoimmune diseases, like rheumatoid arthritis and celiac disease. If lung infections are not treated in a timely manner, they can lead to the development of bronchiectasis, which is chronic inflammation due to excessive mucus buildup in the bronchi. In addition, individuals also develop gastrointestinal infections. Symptoms often include difficulty swallowing due to inflammation (Esophagitis), infectious diarrhea, inflammatory bowel disease (IBD) and difficulty with absorption of nutrients in the gut from food leading to malnutrition.
How is Hypogammaglobulinemia Diagnosed?
The first step in diagnosing an immune deficiency is to seek a thorough evaluation from an Immunologist. Blood tests will reveal decreased antibody levels of all or some of the immunoglobulin (Ig) classes. Vaccine challenge is administered to test your immune systems response. A lack of increase in vaccine antibody titers definitely confirms the diagnosis of an immune system not functioning properly.
How is Hypogammaglobulinemia Treated?
Early diagnosis and treatment help reduce the risk of life-threatening respiratory complications and lymphomas. Treatment of hypogammaglobulinemia depends on the underlying cause. Avoiding infections is the first line of defence for immune compromised. Practicing good hand hygiene, drinking treated water, and having adequate respiratory protection are the basic precautions needed.
Treatment often involves boosting immune system passively with healthy donor antibodies, which is known as IVIG (Intravenous immunoglobulin) infusion or replacement therapy.
The recurrent bacterial infections can be treated with antibiotics. Typically, patients require several rounds of antibiotics to eradicate infections.
Prophylaxis antibiotics for managing inflammation and chronic lung problems due to recurrent pneumonia.
Hematopoietic stem cell transplant can also be done in severe cases when weighing for cost and IVIG availability.
Allergy testing/ Pulmonary function tests (PFT) in children with continued sinopulmonary symptoms. Allergy testing is needed to rule out asthma or allergic triggers contributing to these symptoms.
Repeated ear infections may lead to sensorineural hearing loss and need audio examinations for concerns of impaired hearing or poor school performance.
Individuals with associated autoimmune conditions may require immunosuppressive therapy with corticosteroids and immunomodulators.
Prophylactic vaccination with non-live vaccines is recommended. It’s important to note, all individuals with hypogammaglobulinemia should avoid live attenuated vaccines because the lack of antibodies can make the weakened pathogen difficult to destroy and could lead to potentially life-threatening infections.
Finally, in severe cases of immunodeficiency, like those with severe combined immunodeficiency (SCID), treatment may involve hematopoietic stem cell transplantation as well as antimicrobial prophylaxis and isolation to prevent catching an infection.
Management of immunodeficiencies is complex and needs a multidisciplinary approach involving the immunologist, allergist, Pulmonologist, infectious disease specialist, pediatrician, pharmacist, and a primary care physician for identification, monitoring, and timely intervention.
Educating the parents about the possible causes, identifying warning signs, evaluating family members with frequent infections, genetic counseling, and compliance with the treatment plan play a crucial role in the management of hypogammaglobulinemia.
References:
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https://www.ncbi.nlm.nih.gov/books/NBK279364/ Assessed and Endorsed by the MedReport Medical Review Board