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Related Concept Videos

Immunodeficiency Diseases01:25

Immunodeficiency Diseases

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Immunodeficiency disorders are conditions in which the immune system's ability to fight infectious disease and cancer is compromised or entirely absent. The immune system comprises a complex network of cells, tissues, and organs that work together to protect the body from potentially harmful invaders. When this system is deficient or not functioning properly, it leaves the body susceptible to infections, diseases, or other complications.
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Factors Affecting the Risk of Infection01:26

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The hosts' susceptibility to infection depends on several factors. The integrity of the skin and mucous membranes helps protect the body against microbial attacks. When the skin is altered, the chance of infection, limb loss, and even death increases.
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Disorders of Leukocytes01:27

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Leukocyte disorders can lead to either leukopenia, characterized by an abnormally low leukocyte count, or leukocytosis, marked by a very high leukocyte number.
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Skin Diseases and Disorders01:23

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Skin is the first line of defense and encounters a variety of microbes. Some pathogenic strains are often the cause of a broad range of infections of the skin and other body systems. These conditions can affect people of all ages and may have different causes, including genetic factors, infections, autoimmune reactions, environmental factors, and lifestyle choices.
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Pulmonary Tuberculosis I01:29

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Tuberculosis, often called TB, is a contagious illness primarily caused by Mycobacterium tuberculosis. It mainly affects the lung parenchyma but can also impact other body parts.
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Mode of...
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Infection01:20

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When a pathogen enters the body and reproduces, it can cause an infection, damage body cells, and cause illness symptoms that eventually lead to disease. Therefore, its prevention requires breaking the chain of infection.
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Infections in Disorders of Immune Regulation.

Abarna Thangaraj1, Reva Tyagi1, Deepti Suri1

  • 1Pediatric Allergy Immunology Unit, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India.

Pathogens (Basel, Switzerland)
|March 27, 2024
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Summary

Primary immune regulatory disorders (PIRDs) increase infection risk, mainly bacterial sinopulmonary infections and EBV-related issues. Understanding these infections is crucial for timely diagnosis and preventing organ damage in PIRD patients.

Keywords:
ALPSSTAT3 GOFTregautoimmunityimmune dysregulationlymphoproliferationopportunistic infection

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Area of Science:

  • Immunology
  • Genetics
  • Infectious Diseases

Background:

  • Primary immune regulatory disorders (PIRDs) are inborn errors of immunity (IEIs) characterized by autoimmunity, lymphoproliferation, atopy, and malignancy.
  • Infections are less common in PIRDs than other IEIs but can arise from antibody deficiency, innate immune defects, or T/NK cell issues.
  • Immunotherapy and chemotherapy complicate immune status, increasing infection risks.

Purpose of the Study:

  • To review the infection profile in specific PIRDs: Autoimmune Lymphoproliferative Syndrome (ALPS), Tregopathies, and other autoimmune IEIs.
  • To highlight the importance of recognizing infection patterns for early diagnosis and management.
  • To address the scarcity of comprehensive reports on infections in PIRDs.

Main Methods:

  • Literature review focusing on infection spectrum in ALPS, Tregopathies, and related autoimmune disorders.
  • Analysis of reported infectious manifestations, including common and rare pathogens.
  • Synthesis of information within the broader context of Primary Immune Regulatory Disorders.

Main Results:

  • Recurrent bacterial sinopulmonary infections (Staphylococcal, Streptococcal) are most common.
  • Increased susceptibility to viral infections, particularly Epstein-Barr virus (EBV), leading to lymphoproliferation and malignancy.
  • Mycobacterial and invasive fungal infections are rarely documented.

Conclusions:

  • A comprehensive understanding of the infection spectrum in PIRDs is vital for preventing diagnostic delays and organ damage.
  • Specific PIRD subtypes like ALPS and Tregopathies have distinct infectious risks that require targeted awareness.
  • Further research and reporting are needed to fully elucidate the infectious complications in these complex immune disorders.