Idiopathic subglottic stenosis
Synonyms: iSGS
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Status
- active
A rare otorhinolaryngologic disease characterized by recurrent and severe obstruction of the central airway in the region bounded superiorly by a plane below the glottis and inferiorly by the first two tracheal rings. Symptoms may involve respiratory distress, dysphonia, exertional dyspnea, stridor, chronic cough and wheezing. Diagnosis is challenging due to the lack of apparent etiology and nonspecific respiratory symptoms that can be confounded with other common respiratory diseases and may also occur in late course of the disease. Thorough clinical and laboratory evaluation aims to exclude other causes of subglottic stenosis is therefore essential. Affected patients are almost exclusively Caucasian women between 30-60 years old.
Names in Orphadata languages
10 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Idiopathic subglottic stenosis | iSGS |
| fr | Sténose sous-glottique idiopathique | = en |
| de | Idiopathische Subglottisstenose | IPSS · iSGS |
| es | Estenosis subglótica idiopática | = en |
| it | Stenosi idiopatica subglottica | = en |
| nl | Idiopathische subglottische stenose | Idiopathische stenose van subglottis · iSGS |
| pt | Estenose subglótica idiopática | = en |
| pl | Idiopatyczne zwężenie podgłośniowe | = en |
| cs | Idiopatická subglotická stenóza | = en |
| uk | Ідіопатичний субглотковий стеноз | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | J38.6 | NTBT | attributed | validated |
| ICD-11 | CA0H.5 | NTBT | attributed | validated |
| MONDO | 0958099 | E | validated | |
| MONDO | 958099 | E | validated | |
| UMLS | C5925119 | 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:98036Rare otorhinolaryngologic disease
rare otorhinolaryngological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/652681
- This record as JSON: /data/d/652681.json