IgG4-related submandibular gland disease
Synonyms: Küttner tumor
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
A rare IgG4-related disease characterized by a benign tumor-like chronic inflammatory lesion of the submandibular gland. Histologic features are periductal fibrosis, acinar atrophy, obliterative phlebitis, dense lymphoplasmacytic infiltrates rich in IgG4-positive plasma cells, and formation of lymphoid follicles. Lobular architecture is preserved. Patients most commonly present with unilateral, painless swelling of the submandibular gland. Serologic analysis reveals elevated IgG4 levels.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | IgG4-related submandibular gland disease | Küttner tumor |
| fr | Maladie de la glande submandibulaire associée à IgG4 | Maladie de la glande sous mandibulaire associée à IgG4 · Tumeur de Küttner |
| de | IgG4-assoziierte chronisch-sklerosierende Sialadenitis | Chronisch-sklerosierende Sialadenitis der Glandula submandibularis · IgG4-assoziierte Adenitis der Glandula submandibularis · IgG4-assoziierte Speicheldrüsenentzündung |
| es | Enfermedad de la glándula submandibular asociada a IgG4 | Tumor de Küttner |
| it | Malattia della ghiandola sottomandibolare correlata alle IgG4 | Tumore di Küttner |
| nl | IgG4-gerelateerde aandoening van submandibulaire klier | Küttner-tumor |
| pt | Doença da glândula submandibular IgG4-relacionada | Tumor Küttner |
| pl | Choroba ślinianki podżuchwowej związana z IgG4 | = en |
| cs | IgG4 asociované postižení submandibulární slinné žlázy | IgG4 asociovaná sialoadenitida · Küttnerův nádor |
| uk | Захворювання підщелепної залози, повязане з IgG4 | Пухлина Кюттнера |
| zh | IgG4相关的下颌下腺疾病 | IgG4相关涎腺炎 |
| tr | IgG4-ilişkili submandibuler bez hastalığı | Küttner tümörü · IgG4-ilişkili sialadenit |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | K11.2 | NTBT | attributed | validated |
| ICD-11 | 4A43.0 | NTBT | index | validated |
| MONDO | 0018674 | E | validated | |
| UMLS | C5679995 | 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 (2):
- ORPHA:596448IgG4-related systemic disease
rare systemic and rheumatological diseases - ORPHA:98036Rare otorhinolaryngologic disease
rare otorhinolaryngological 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 | Validated | ORPHANET_24844187[PMID] |
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
25 annotations · validated 2021-05-26| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0010287 | Abnormality of the submandibular glands | |
| HP:0002960 | Autoimmunity | |
| HP:0003237 | Increased circulating IgG level | |
| HP:0032300 | Increased circulating IgG4 level | |
| Frequent (79-30%) | ||
| HP:0010286 | Abnormal salivary gland morphology | |
| HP:0004431 | Complement deficiency | |
| HP:0007734 | Enlarged lacrimal glands | |
| HP:0003212 | Increased circulating IgE concentration | |
| HP:0010702 | Increased circulating immunoglobulin concentration | |
| HP:0001880 | Increased total eosinophil count | |
| HP:0002716 | Lymphadenopathy | |
| HP:0031281 | Sialadenitis | |
| HP:0000217 | Xerostomia | |
| Occasional (29-5%) | ||
| HP:0012090 | Abnormal pancreas morphology | |
| HP:0000077 | Abnormality of the kidney | |
| HP:0000820 | Abnormality of the thyroid gland | |
| HP:0011801 | Enlargement of parotid gland | |
| HP:0000282 | Facial edema | |
| HP:0100539 | Periorbital edema | |
| HP:0025439 | Pharyngitis | |
| HP:0000083 | Renal insufficiency | |
| HP:0005200 | Retroperitoneal fibrosis | |
| Very rare (<4-1%) | ||
| HP:0025426 | Abnormal bronchus morphology | |
| HP:0030151 | Cholangitis | |
| HP:0000024 | Prostatitis | |
Run the differential on the 4 obligate / very frequent terms
Annotation source: 24332556[PMID]_33827676[PMID]_27007955[PMID]_27193771[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/449432
- This record as JSON: /data/d/449432.json