Autoimmunity in patients with primary immunodeficiency
- Markus G Seidel, MD
Markus G Seidel, MD
- Professor of Translational Pediatric Hematology and Immunology
- Medical University of Graz
- Section Editor
- E Richard Stiehm, MD
E Richard Stiehm, MD
- Editor-in-Chief — Allergy and Immunology
- Section Editor — Immunology and Immunodeficiency
- Distinguished Research Professor of Pediatrics
- David Geffen School of Medicine at UCLA
- Deputy Editors
- Anna M Feldweg, MD
Anna M Feldweg, MD
- Senior Deputy Editor — UpToDate
- Deputy Editor — Allergy and Immunology
- Instructor in Medicine
- Harvard Medical School
- Monica Ramirez Curtis, MD, MPH
Monica Ramirez Curtis, MD, MPH
- Deputy Editor — Rheumatology
- Instructor of Medicine
- Harvard Medical School
Patients with primary immunodeficiency (PID) have dysregulated immune processes, which can result in an increased susceptibility to infectious diseases, autoimmune disorders, and malignancies.
This topic will discuss the most common manifestations of autoimmunity in patients with PID and review the autoimmune disorders associated with the most prevalent PID disorders. Malignancies in patients with PID are reviewed separately. (See "Malignancy in primary immunodeficiency".)
CLASSIFICATION OF PID
Primary immunodeficiencies (PID) are classified into nine major categories of disorders :
●Combined immunodeficiencies without syndromic features. (See "Combined immunodeficiencies" and "Severe combined immunodeficiency (SCID): An overview".)
●Combined immunodeficiencies with syndromic features. (See "Syndromic immunodeficiencies".)
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- CLASSIFICATION OF PID
- MANIFESTATIONS OF AUTOIMMUNITY AND IMMUNE DYSREGULATION IN PID
- GENERAL PRINCIPLES OF AUTOIMMUNITY IN PID
- Autoimmune disorders in common forms of PID
- - Cytopenias
- - Endocrinopathies
- - Enteropathies
- WHEN TO SUSPECT AN UNDERLYING PID
- Early-onset autoimmunity
- Multiorgan involvement
- WHEN TO REFER
- POSSIBLE PATHOGENIC MECHANISMS
- Immune dysregulation
- MONITORING FOR AUTOIMMUNITY
- Considering alternative etiologies
- SUMMARY AND RECOMMENDATIONS