Adult-onset immunodeficiency with anti-interferon-gamma autoantibodies
Synonyms: Acquired adult-onset immunodeficiency · Adult-onset immunodeficiency with acquired anti-interferon-gamma autoantibodies
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare acquired immunodeficiency disorder characterized by the appearance of susceptibility to disseminated opportunistic infections (in particular, disseminated nontuberculous mycobacterial infection, salmonellosis, penicillosis, and varicella zoster virus infection) in previously healthy (HIV-negative) adults, associated with the presence of acquired autoantibodies to interferon gamma. Typical clinical manifestation includes lymphadenopathy (cervical or generalized), fever, weight loss and/or reactive skin lesions.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Adult-onset immunodeficiency with anti-interferon-gamma autoantibodies | Acquired adult-onset immunodeficiency · Adult-onset immunodeficiency with acquired anti-interferon-gamma autoantibodies |
| fr | Déficit immunitaire de l'adulte avec autoanticorps anti-interféron-gamma | Déficit immunitaire acquis de l'adulte · Déficit immunitaire de l'adulte avec autoanticorps anti-interféron-gamma acquis |
| de | Immundefekt des Erwachsenen, mit anti-Interferon gamma-Antikörpern | Immundefekt des Erwachsenen, erworbener · Immundefekt des Erwachsenen, mit erworbenen anti-Interferon gamma-Antikörpern |
| es | Inmunodeficiencia con autoanticuerpos anti-interferón-gamma de inicio en el adulto | Inmunodeficiencia adquirida de inicio en el adulto · Inmunodeficiencia adquirida de inicio en el adulto con autoanticuerpos anti-interferón-gamma |
| it | Immunodeficienza ad esordio nell'età adulta con autoanticorpi anti-interferone gamma | |
| nl | Imuundeficiëntie met anti-interferon-gamma auto-antilichamen met aanvang op volwassen leeftijd | Imuundeficiëntie met verworven anti-interferon-gamma auto-antilichamen met aanvang op volwassen leeftijd · Verworven immuundeficiëntie met aanvang op volwassen leeftijd |
| pt | Imunodeficiência com autoanticorpos anti-interferon-gama de início no adulto | |
| pl | Niedobór odporności wieku dorosłego z obecnością przeciwciał przeciwko interferonowi gamma | Niedobór odporności o początku w wieku dorosłym z autoprzeciwciałami anty interferon gamma |
| cs | Získaná imunodeficience s nástupem v dospělém věku | Imunodeficience s anti-interferon-gama protilátkami s nástupem v dospělém věku |
| uk | Імунодефіцит у дорослих з аутоантитілами до антиінтерферону-гамма | Набутий імунодефіцит у дорослих · Імунодефіцит, що починається у дорослих, з набутими аутоантитілами до антиінтерферону-гамма |
| zh | 抗干扰素-γ自身抗体的成人免疫缺陷 | 成人免疫缺陷伴抗干扰素-γ自身抗体 |
| tr | Anti-interferon-gama otoantikorları ile seyreden yetişkin başlangıçlı immün yetmezlik | Erişkin başlangıçlı edinsel immün yetmezlik · Edinsel anti-interferon-gama otoantikorları ile seyreden erişkin başlangıçlı immün yetmezlik |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D84.8 | NTBT | attributed | validated |
| ICD-11 | 4A20.0 | NTBT | attributed | validated |
| MONDO | 0017617 | E | validated | |
| UMLS | C5191336 | E | validated | |
| GARD | 11992 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:310050Rare acquired immunodeficiency
rare immunological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/306431
- This record as JSON: /data/d/306431.json