Immunodeficiency
This article needs additional citations for verification. (November 2007) |
Immunodeficiency | |
---|---|
Other names | Immunocompromisation, immune deficiency |
Specialty | Immunology |
Medication | Imuran |
Immunodeficiency, also known as immunocompromisation, is a state in which the
In clinical settings,
A person who has an immunodeficiency of any kind is said to be immunocompromised. An immunocompromised individual may particularly be vulnerable to
Types
By affected component
- agammaglobulinemia (lack of all or most antibody production) which results in frequent severe infections and is often fatal.[6]
- acquired immune deficiency syndrome (AIDS).[7]
- Granulocyte deficiency, including decreased numbers of neutrophil granulocytes (termed neutropenia). Granulocyte deficiencies also include decreased function of individual granulocytes, such as in chronic granulomatous disease.
- Asplenia, where there is no function of the spleen
- Complement deficiency is where the function of the complement system is deficient
In reality, immunodeficiency often affects multiple components, with notable examples including
Affected components | Main causes[8] | Main pathogens of resultant infections[8] | |
---|---|---|---|
Humoral immune deficiency
B cell deficiency
|
B cells, plasma cells or antibodies |
|
|
T cell deficiency | T cells |
|
fungal infections.[8]
|
Neutropenia | Neutrophil granulocytes
|
|
|
Asplenia | Spleen |
|
|
Complement deficiency | Complement system |
|
|
Primary or secondary
The distinction between primary versus secondary immunodeficiencies is based on, respectively, whether the cause originates in the immune system itself or is, in turn, due to insufficiency of a supporting component of it or an external decreasing factor of it.
Primary immunodeficiency
A number of
The treatment of primary immunodeficiencies depends on the nature of the defect, and may involve antibody infusions, long-term antibiotics and (in some cases) stem cell transplantation. The characteristics of lacking and/or impaired antibody functions can be related to illnesses such as X-Linked Agammaglobulinemia and Common Variable Immune Deficiency [13]
Secondary immunodeficiencies
Secondary immunodeficiencies, also known as acquired immunodeficiencies, can result from various
Many specific diseases directly or indirectly cause immunosuppression. This includes many types of
Various hormonal and metabolic disorders can also result in immune deficiency including anemia, hypothyroidism and hyperglycemia.
Smoking, alcoholism and drug abuse also depress immune response.
Heavy schedules of training and competition in athletes increases their risk of immune deficiencies.[14]
Causes
The cause of immunodeficiency varies depending on the nature of the disorder. The cause can be either genetic or acquired by malnutrition and poor sanitary conditions.[15][16] Only for some genetic causes, the exact genes are known.[17]
Immunodeficiency and autoimmunity
This section needs additional citations for verification. (November 2016) |
There are a large number of immunodeficiency syndromes that present clinical and laboratory characteristics of autoimmunity. The decreased ability of the immune system to clear infections in these patients may be responsible for causing autoimmunity through perpetual immune system activation.[18] One example is common variable immunodeficiency (CVID) where multiple autoimmune diseases are seen, e.g., inflammatory bowel disease, autoimmune thrombocytopenia, and autoimmune thyroid disease.
Diagnosis
Patients with immune deficiencies can present with variable clinical phenotypes. This often translates into a significant delay in their diagnosis, and resultant patient morbidity. A structured approach on when to suspect an immunodeficiency and the initial investigations pathway is given in the publication by Grammatikos et al.[19]
People who suffer from chronic infections such as candida or Lyme disease have predisposed or enhanced immune responses. So, individuals with underlying conditions such as periodic(chronic) bacterial pneumonias, yeast infections, bacterial sinus infections, and other continuous infections are at a very high risk of being diagnosed with an immunodeficiency disorder. By undermining the immune system, people susceptible to chronic illnesses suffer from existing illnesses constantly, forming a sick circle. Besides that, they may also suffer from chronic abdominal pain and this will further complicate their condition while as the time passes they lead to weight loss due to impaired metabolism and maintenance of overall wellbeing.[20]
If a doctor suspects for a immunodeficiency, they will proceed with conducting multiple different effectual tests. The tests depend on the type of immunodeficiency you may have. Primary immunodeficiencies are in most cases hereditary, more specifically X-linked. Secondary immunodeficiencies are brought about by malnutrition, aging, and certain medications. In most cases, the doctor begins from the review of the patient's medical record, followed by a comprehensive physical exam. A laboratory assay to test for the fraction of white blood cell, T cell, and immunoglobulin amounts will be crucial for diagnosis. Skin tests may be used as the investigative tool in situations where a T cell abnormality is suspected. Making biopsies of lymph nodes or marrow may be the case when determining the underlying causes of the disease. A genetic test involves a blood sample usually ordered to look for the specific gene mutations ratifying the influence to the immune disorder. Such diagnostic tests consist of instruments that individually give the clue on the specific disorder and assist in the development of effective treatment regimens.[21]
Treatment
Available treatment falls into two modalities: treating infections and boosting the immune system.
Prevention of Pneumocystis pneumonia using trimethoprim/sulfamethoxazole is useful in those who are immunocompromised.[22] In the early 1950s Immunoglobulin(Ig) was used by doctors to treat patients with primary immunodeficiency through intramuscular injection. Ig replacement therapy are infusions that can be either subcutaneous or intravenously administered, resulting in higher Ig levels for about three to four weeks, although this varies with each patient.[13]
Prognosis
Prognosis depends greatly on the nature and severity of the condition. Some deficiencies cause early mortality (before age one), others with or even without treatment are lifelong conditions that cause little mortality or morbidity. Newer stem cell transplant technologies may lead to gene based treatments of debilitating and fatal genetic immune deficiencies. Prognosis of acquired immune deficiencies depends on avoiding or treating the causative agent or condition (like AIDS).
See also
- Acquired immune deficiency syndrome(AIDS)
- Immune disorder
- Autoimmune disease, immune response to self-proteins
- Allergy, immune response to harmless non-self proteins
- Immunosenescence, age-associated immune deficiency
- Steroids, commonly administered drugs like prednisone that suppress the immune system
- Human genetic enhancement
- Immune system
- Immunology
References
- PMID 20042227.
- ^ "Primary immunodeficiency". Mayo Clinic. 30 January 2020. Retrieved 13 May 2020.
- PMID 25002900.
- PMID 35236664.
- PMID 24491087.
- ^ Greenberg S. "Immunodeficiency". University of Toronto. Archived from the original on 10 July 2013.
- ^ Schwartz RA (2019-10-22). Jyonouchi H (ed.). "T-cell Disorders". Medscape.
- ^ ISBN 978-1-4051-2665-6.
- ISBN 978-1-4051-2665-6.
- ^ PMID 11272299.
- ^ a b Basic Immunology: Functions and Disorders of the Immune System, 3rd Ed. 2011.
- S2CID 39699189.
- ^ a b "Immune Deficiency Foundation". primaryimmune.org. Retrieved 2017-04-17.
- S2CID 84378380.
- ^ "Nutrition and Immunity". The Nutrition Source. Harvard T.H. Chan School of Public Health. May 2020. Retrieved 8 November 2020.
- PMID 27237815.
- ^ Charles A Janeway, Jr; Travers, Paul; Walport, Mark; Shlomchik, Mark J. (2001). "Inherited immunodeficiency diseases". Immunobiology. Garland Science.
- PMID 22177735.
- S2CID 221143773.
- PMID 33679719.
- PMID 33679719.
- PMID 25269391.