IgG4-related dacryoadenitis and sialadenitis
Synonyms: Chronic dacryoadenitis and sialadenitis · Mikulicz disease
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
IgG4-related dacryoadenitis and sialoadenitis (Mikulicz disease) is an IgG4-related sclerosing disease characterized by persistent, usually painless, bilateral enlargement of the lacrimal, parotid, and submandibular glands associated with elevated levels of serum immunoglobulin (Ig) G4 and with lymphocyte and IgG4-positive plasmacyte infiltration. It predominantly causes mouth and eye dryness but can also affect other organs such as the lungs, liver, and kidneys, and be accompanied by complications such as autoimmune pancreatitis (AIP), retroperitoneal fibrosis, and tubulointerstitial nephritis.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | IgG4-related dacryoadenitis and sialadenitis | Chronic dacryoadenitis and sialadenitis · Mikulicz disease |
| fr | Dacryoadénite et sialadénite à IgG4 | Dacryoadénite et sialadénite chroniques · Maladie de Mikulicz |
| de | IgG4-assoziierte Dakryoadenitis und Sialoadenitis | Mikulicz-Krankheit · Mikulicz-Syndrom · Tränen- und Speicheldrüsenentzündung, chronische |
| es | Dacrioadenitis y sialoadenitis asociada a IgG4 | Dacrioadenitis y sialoadenitis crónicas · Enfermedad de Mikulicz |
| it | Dacrioadenite e scialoadenite correlate alle IgG4 | Malattia di Mikulicz |
| nl | IgG4-gerelateerde dacryoadenitis en sialoadenitis | Chronische dacryoadenitis en sialoadenitis · Ziekte van Mikulicz |
| pt | Sialadenite e dacrioadenite IgG4-relacionada | Doença Mickulicz · Sialadenite e dacrioadenite crónica |
| pl | Zapalenie gruczołów łzowych i ślinianek związane z IgG4 | Choroba Mikulicza · Przewlekłe zapalenie gruczołów łzowych i ślinianek |
| cs | IgG4 asociovaná sialo-dakryoadenitida | Chronická dakryoadenitida a sialoadenitida · Mikuliczova choroba |
| uk | Дакріоаденіт та сіаладеніт, повязані з IgG4 | Хронічний дакріоаденіт та сіаладеніт · Хвороба Мікуліча |
| zh | IgG4相关性泪腺和唾液腺炎 | 慢性泪腺和唾液腺炎 |
| tr | IgG4-ilişkili dakriyoadenit ve siyaladenit | Mikulicz hastalığı · Kronik dakriyoadenit ve sialadenit |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | K11.8 | NTBT | inclusion | validated |
| ICD-11 | 4A43.0 | NTBT | index | validated |
| MONDO | 0019191 | E | validated | |
| MeSH | D008882 | E | validated | |
| UMLS | C0026103 | E | validated | |
| GARD | 7043 | E | validated | |
| MedDRA | 10051457 | 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 (3):
- ORPHA:596448IgG4-related systemic disease
rare systemic and rheumatological diseases - ORPHA:519264Inflammatory/autoimmune disorder involving the lacrimal system
rare ophthalmic disorders - 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 | 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
33 annotations · validated 2021-09-22| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0010286 | Abnormal salivary gland morphology | |
| HP:0010287 | Abnormality of the submandibular glands | |
| HP:0007734 | Enlarged lacrimal glands | |
| HP:0032300 | Increased circulating IgG4 level | |
| Frequent (79-30%) | ||
| HP:0011801 | Enlargement of parotid gland | |
| Occasional (29-5%) | ||
| HP:0000587 | Abnormal optic nerve morphology | |
| HP:0008049 | Abnormality of the extraocular muscles | |
| HP:0000315 | Abnormality of the orbital region | |
| HP:0003493 | Antinuclear antibody positivity | |
| HP:0002960 | Autoimmunity | |
| HP:0032230 | Cytoplasmic antineutrophil antibody positivity | |
| HP:0000282 | Facial edema | |
| HP:0012378 | Fatigue | |
| HP:0001945 | Fever | |
| HP:0003324 | Generalized muscle weakness | |
| HP:0003261 | Increased circulating IgA level | |
| HP:0003496 | Increased circulating IgM level | |
| HP:0001097 | Keratoconjunctivitis sicca | |
| HP:0100614 | Myositis | |
| HP:0100540 | Palpebral edema | |
| HP:0000629 | Periorbital fullness | |
| HP:0000520 | Proptosis | |
| HP:0001824 | Weight loss | |
| HP:0000217 | Xerostomia | |
| Very rare (<4-1%) | ||
| HP:0011481 | Abnormal lacrimal duct morphology | |
| HP:0000618 | Blindness | |
| HP:0002716 | Lymphadenopathy | |
| HP:0005994 | Nodular goiter | |
| HP:0007807 | Optic nerve compression | |
| HP:0005200 | Retroperitoneal fibrosis | |
| HP:0001873 | Thrombocytopenia | |
| HP:0100646 | Thyroiditis | |
| HP:0001970 | Tubulointerstitial nephritis | |
Run the differential on the 4 obligate / very frequent terms
Annotation source: 31598602[PMID]_26016066[PMID]_33598067[PMID]_29675343[PMID]_23242814[PMID]_32921832[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/79078
- This record as JSON: /data/d/79078.json