Editing Autoimmunity (section)

== 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.<ref name="pmid22177735">{{cite journal | vauthors = Grammatikos AP, Tsokos GC | title = Immunodeficiency and autoimmunity: lessons from systemic lupus erythematosus | journal = Trends in Molecular Medicine | volume = 18 | issue = 2 | pages = 101–108 | date = February 2012 | pmid = 22177735 | pmc = 3278563 | doi = 10.1016/j.molmed.2011.10.005 }}</ref>

One example is [[common variable immunodeficiency]], in which multiple autoimmune diseases are seen, e.g., inflammatory bowel disease, autoimmune thrombocytopenia and autoimmune thyroid disease.<ref>{{Cite journal |last1=Tam |first1=Jonathan S. |last2=Routes |first2=John M. |date=March 2013 |title=Common Variable Immunodeficiency |journal=American Journal of Rhinology & Allergy |language=en |volume=27 |issue=4 |pages=260–265 |doi=10.2500/ajra.2013.27.3899 |issn=1945-8924 |pmc=3901442 |pmid=23883805}}</ref>

Familial hemophagocytic [[lymphohistiocytosis]], an autosomal recessive primary immunodeficiency, is another example. [[Pancytopenia]], rashes, [[lymphadenopathy|swollen lymph nodes]] and [[hepatosplenomegaly|enlargement of the liver and spleen]] are commonly seen in such individuals. Presence of multiple uncleared viral infections due to lack of 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 mellitus]] 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 a caused by 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]] 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.<ref>{{Cite journal |last1=Vosughimotlagh |first1=Ahmad |last2=Rasouli |first2=Seyed Erfan |last3=Rafiemanesh |first3=Hosein |last4=Safarirad |first4=Molood |last5=Sharifinejad |first5=Niusha |last6=Madanipour |first6=Atossa |last7=Dos Santos Vilela |first7=Maria Marluce |last8=Heropolitańska-Pliszka |first8=Edyta |last9=Azizi |first9=Gholamreza |date=2023-08-28 |title=Clinical manifestation for immunoglobulin A deficiency: a systematic review and meta-analysis |journal=Allergy, Asthma & Clinical Immunology |language=en |volume=19 |issue=1 |page=75 |doi=10.1186/s13223-023-00826-y |doi-access=free |issn=1710-1492 |pmc=10463351 |pmid=37641141}}</ref>