Adult-onset Immunodeficiency Syndrome
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Adult-onset immunodeficiency syndrome is a type of
immunodeficiency Immunodeficiency, also known as immunocompromisation, is a state in which the immune system's ability to fight infectious diseases and cancer is compromised or entirely absent. Most cases are acquired ("secondary") due to extrinsic factors that a ...
. It is linked to vulnerability to disseminated infections brought on by
opportunistic pathogens An opportunistic infection is an infection caused by pathogens (bacteria, fungi, parasites or viruses) that take advantage of an opportunity not normally available. These opportunities can stem from a variety of sources, such as a weakened immune ...
. People with this condition have increased levels of anti-interferon-gamma
autoantibodies An autoantibody is an antibody (a type of protein) produced by the immune system that is directed against one or more of the individual's own proteins. Many autoimmune diseases (notably lupus erythematosus) are associated with such antibodies. Pr ...
. These particular
immune system The immune system is a network of biological processes that protects an organism from diseases. It detects and responds to a wide variety of pathogens, from viruses to parasitic worms, as well as cancer cells and objects such as wood splinte ...
proteins mistakenly target an individual's own tissues. It is most commonly reported among previously healthy Southeast Asians. The average age of onset is between 30 and 50 years. Non-tuberculous mycobacteria, non-typhoidal
salmonella ''Salmonella'' is a genus of rod-shaped (bacillus) Gram-negative bacteria of the family Enterobacteriaceae. The two species of ''Salmonella'' are ''Salmonella enterica'' and ''Salmonella bongori''. ''S. enterica'' is the type species and is fur ...
,
cytomegalovirus ''Cytomegalovirus'' (''CMV'') (from ''cyto-'' 'cell' via Greek - 'container' + 'big, megalo-' + -''virus'' via Latin 'poison') is a genus of viruses in the order ''Herpesvirales'', in the family ''Herpesviridae'', in the subfamily ''Betaherpe ...
,
Penicillium marneffei ''Talaromyces marneffei'', formerly called ''Penicillium marneffei'', was identified in 1956. The organism is endemic to southeast Asia where it is an important cause of opportunistic infections in those with HIV/AIDS-related immunodeficiency. ...
, and
varicella zoster virus Varicella-zoster virus (VZV), also known as human herpesvirus 3 (HHV-3, HHV3) or ''Human alphaherpesvirus 3'' (taxonomically), is one of nine known herpes viruses that can infect humans. It causes chickenpox (varicella) commonly affecting chil ...
are some of the
pathogen In biology, a pathogen ( el, πάθος, "suffering", "passion" and , "producer of") in the oldest and broadest sense, is any organism or agent that can produce disease. A pathogen may also be referred to as an infectious agent, or simply a germ ...
s that can cause infections in patients with this illness. Symptoms vary depending on the infection(s) present in each individual. It is unknown what causes the development of anti-interferon-gamma
autoantibodies An autoantibody is an antibody (a type of protein) produced by the immune system that is directed against one or more of the individual's own proteins. Many autoimmune diseases (notably lupus erythematosus) are associated with such antibodies. Pr ...
, however,  genetic factors are thought to be implicated. A number of studies indicate a link with specific HLA genes. These
gene In biology, the word gene (from , ; "...Wilhelm Johannsen coined the word gene to describe the Mendelian units of heredity..." meaning ''generation'' or ''birth'' or ''gender'') can have several different meanings. The Mendelian gene is a ba ...
s aid the
immune system The immune system is a network of biological processes that protects an organism from diseases. It detects and responds to a wide variety of pathogens, from viruses to parasitic worms, as well as cancer cells and objects such as wood splinte ...
in distinguishing between proteins produced by the body and those produced by outside threats. Some have proposed that an infection may first initiate the formation of
autoantibodies An autoantibody is an antibody (a type of protein) produced by the immune system that is directed against one or more of the individual's own proteins. Many autoimmune diseases (notably lupus erythematosus) are associated with such antibodies. Pr ...
, and that subsequent infections increase their activity. There is currently no standard medication for Adult-onset immunodeficiency syndrome, and treatment is dependent on the infection(s) present. Long-term
antibiotic therapy An antibiotic is a type of antimicrobial substance active against bacteria. It is the most important type of antibacterial agent for fighting bacterial infections, and antibiotic medications are widely used in the treatment and prevention of ...
and
rituximab Rituximab, sold under the brand name Rituxan among others, is a monoclonal antibody medication used to treat certain autoimmune diseases and types of cancer. It is used for non-Hodgkin lymphoma, chronic lymphocytic leukemia (in non-geriatric p ...
therapy have been used to manage Adult-onset immunodeficiency syndrome. Multiple treatments may be required at the same time.


Signs and symptoms

At least one physician associates the
symptom Signs and symptoms are the observed or detectable signs, and experienced symptoms of an illness, injury, or condition. A sign for example may be a higher or lower temperature than normal, raised or lowered blood pressure or an abnormality showin ...
s with
tuberculosis Tuberculosis (TB) is an infectious disease usually caused by '' Mycobacterium tuberculosis'' (MTB) bacteria. Tuberculosis generally affects the lungs, but it can also affect other parts of the body. Most infections show no symptoms, in ...
. Some lethal overwhelming infections are reported, aggravating people who already suffer other conditions such as
HIV/AIDS Human immunodeficiency virus infection and acquired immunodeficiency syndrome (HIV/AIDS) is a spectrum of conditions caused by infection with the human immunodeficiency virus (HIV), a retrovirus. Following initial infection an individual ...
. Clinical manifestations can involve symptoms similar to
Generalized pustular psoriasis Generalized pustular psoriasis (GPP) is an extremely rare type of psoriasis that can present in a variety of forms. Unlike the most general and common forms of psoriasis, GPP usually covers the entire body and with pus-filled blisters rather than p ...
.


Cause

Little is publicly known about the underlying factors causing the disease. Genetic factors are suspected, but the disease does not appear to be heritable. One specific gene hypothesized to be involved in the disorder is SERPINA1. Also, something in the
environment Environment most often refers to: __NOTOC__ * Natural environment, all living and non-living things occurring naturally * Biophysical environment, the physical and biological factors along with their chemical interactions that affect an organism or ...
may trigger the disease.


Mechanism

An elevated concentration of
autoantibodies An autoantibody is an antibody (a type of protein) produced by the immune system that is directed against one or more of the individual's own proteins. Many autoimmune diseases (notably lupus erythematosus) are associated with such antibodies. Pr ...
that block
interferon-gamma Interferon gamma (IFN-γ) is a dimerized soluble cytokine that is the only member of the type II class of interferons. The existence of this interferon, which early in its history was known as immune interferon, was described by E. F. Wheelock ...
was detected in most patients.


Diagnosis


Treatment


Society and culture

The swash.com website uses AIDS 2.0 as the moniker for maybe another, apparently highly contagious AIDS-like condition described by
The Epoch Times ''The Epoch Times'' is a far-right international multi-language newspaper and media company affiliated with the Falun Gong new religious movement. The newspaper, based in New York City, is part of the Epoch Media Group, which also operates New ...
.
The Daily Beast ''The Daily Beast'' is an American news website focused on politics, media, and pop culture. It was founded in 2008. It has been characterized as a "high-end tabloid" by Noah Shachtman, the site's editor-in-chief from 2018 to 2021. In a 20 ...
has described this disease emphatically as not AIDS 2.0.


References


External links

{{Medical resources , ICD10 = D84.8 , ICD9 = , ICDO = , OMIM = , DiseasesDB = , MedlinePlus = , eMedicineSubj = , eMedicineTopic = , MeSH = , GeneReviewsNBK = , GeneReviewsName = , Orphanet = 306431 Immunodeficiency Syndromes affecting immunity Health in Thailand