Immunodeficiency

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Immunocompromised
)
Immunodeficiency
Other namesImmunocompromisation, immune deficiency
SpecialtyImmunology
MedicationImuran

Immunodeficiency, also known as immunocompromisation, is a state in which the

infectious diseases and cancer is compromised or entirely absent. Most cases are acquired ("secondary") due to extrinsic factors that affect the patient's immune system. Examples of these extrinsic factors include HIV infection and environmental factors, such as nutrition.[1]
Immunocompromisation may also be due to
genetic diseases/flaws such as SCID
.

In clinical settings,

organ transplant surgery as an anti-rejection measure and in patients with an overactive immune system, as in autoimmune diseases. Some people are born with intrinsic defects in their immune system, or primary immunodeficiency.[2]

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

In reality, immunodeficiency often affects multiple components, with notable examples including

acquired immune deficiency syndrome
(which is secondary).

Comparison of immunodeficiencies by affected component
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
  • Congenital deficiencies

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

autosomal recessive or X-linked. There are over 95 recognised primary immunodeficiency syndromes; they are generally grouped by the part of the immune system that is malfunctioning, such as lymphocytes or granulocytes.[12]

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

petrochemicals like styrene, dichlorobenzene, xylene, and ethylphenol. For medications, the term immunosuppression
generally refers to both beneficial and potential adverse effects of decreasing the function of the immune system, while the term immunodeficiency generally refers solely to the adverse effect of increased risk for infection.

Many specific diseases directly or indirectly cause immunosuppression. This includes many types of

acquired immunodeficiency syndrome (AIDS),[11] caused by the human immunodeficiency virus (HIV). HIV directly infects a small number of T helper cells
, and also impairs other immune system responses indirectly.

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

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.

perforin
are thought to be responsible. In addition to chronic and/or recurrent infections many autoimmune diseases including arthritis, autoimmune hemolytic anemia, scleroderma and type 1 diabetes are also seen in X-linked agammaglobulinemia (XLA). Recurrent bacterial and fungal infections and chronic inflammation of the gut and lungs are seen in chronic granulomatous disease (CGD) as well. CGD is caused by a decreased production of nicotinamide adenine dinucleotide phosphate (NADPH) oxidase by neutrophils. Hypomorphic RAG mutations are seen in patients with midline granulomatous disease; an autoimmune disorder that is commonly seen in patients with granulomatosis with polyangiitis and NK/T cell lymphomas. Wiskott–Aldrich syndrome (WAS) patients also present with eczema, autoimmune manifestations, recurrent bacterial infections and lymphoma. In
autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy
(APECED) also autoimmunity and infections coexist: organ-specific autoimmune manifestations (e.g., hypoparathyroidism and adrenocortical failure) and chronic mucocutaneous candidiasis. Finally, IgA deficiency is also sometimes associated with the development of autoimmune and atopic phenomena.

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]

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.[20] 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

References

  1. PMID 20042227
    .
  2. ^ "Primary immunodeficiency". Mayo Clinic. 30 January 2020. Retrieved 13 May 2020.
  3. PMID 25002900
    .
  4. .
  5. .
  6. ^ Greenberg S. "Immunodeficiency". University of Toronto. Archived from the original on 10 July 2013.
  7. ^ Schwartz RA (2019-10-22). Jyonouchi H (ed.). "T-cell Disorders". Medscape.
  8. ^ .
  9. .
  10. ^ .
  11. ^ a b Basic Immunology: Functions and Disorders of the Immune System, 3rd Ed. 2011.
  12. S2CID 39699189
    .
  13. ^ a b "Immune Deficiency Foundation". primaryimmune.org. Retrieved 2017-04-17.
  14. S2CID 84378380
    .
  15. ^ "Nutrition and Immunity". The Nutrition Source. Harvard T.H. Chan School of Public Health. May 2020. Retrieved 8 November 2020.
  16. PMID 27237815
    .
  17. ^ Charles A Janeway, Jr; Travers, Paul; Walport, Mark; Shlomchik, Mark J. (2001). "Inherited immunodeficiency diseases". Immunobiology. Garland Science.
  18. PMID 22177735
    .
  19. .
  20. .

External links