Kawasaki disease
Synonyms: Mucocutaneous lymph node syndrome
- Type
- Disease
- Group
- Disorder
- Inheritance
- Multigenic/multifactorial
- Age of onset
- Adolescent, Adult, Childhood, Infancy
- Status
- active
A rare inflammatory disease characterized by an acute febrile, systemic, self-limiting, medium-vessel vasculitis primarily affecting children. It often causes acute coronary arteritis which is associated with coronary arterial aneurysms (CAA) that may be life threatening when untreated.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Kawasaki disease | Mucocutaneous lymph node syndrome |
| fr | Maladie de Kawasaki | Syndrome lympho-cutanéo-muqueux |
| de | Kawasaki-Krankheit | Kawasaki-Syndrom · Mukokutanes Lymphknotensyndrom |
| es | Enfermedad de Kawasaki | Síndrome linfo-cutáneo-mucoso |
| it | Malattia di Kawasaki | Sindrome linfo-cutaneo-mucosa |
| nl | Ziekte van Kawasaki | Mucocutaan lymfekliersyndroom |
| pt | Doença de Kawasaki | Síndrome de nódulos linfáticos mucocutâneos |
| pl | Choroba Kawasakiego | Zespół skórno-śluzówkowy węzłów chłonnych |
| cs | Kawasakiho syndrom | Mukokutánní lymfatický syndrom |
| uk | Хвороба Кавасакі | Синдром підшкірних лімфатичних вузлів |
| zh | 川崎病 | 皮肤粘膜淋巴结综合征 |
| tr | Kawasaki hastalığı | Mukokutanöz lenf nodu sendromu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 611775 | E | validated | |
| ICD-10 | M30.3 | E | specific | validated |
| ICD-11 | 4A44.5 | E | specific | validated |
| MONDO | 0012727 | E | validated | |
| MeSH | D009080 | E | validated | |
| UMLS | C0026691 | E | validated | |
| GARD | 6816 | E | validated | |
| MedDRA | 10023320 | 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:156143Predominantly medium-vessel vasculitis
rare systemic and rheumatological diseases; rare circulatory system diseases - ORPHA:280369Rare pediatric vasculitis
rare systemic or rheumatologic diseases of childhood
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
46 annotations · validated 2023-11-10| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0025289 | Cervical lymphadenopathy | Diagnostic criterion |
| HP:0100825 | Cheilitis | Diagnostic criterion |
| HP:0000509 | Conjunctivitis | Diagnostic criterion |
| HP:0025493 | Palmoplantar erythema | Diagnostic criterion |
| HP:0000093 | Proteinuria | |
| HP:0100776 | Recurrent pharyngitis | |
| HP:0000988 | Skin rash | Diagnostic criterion |
| HP:0002633 | Vasculitis | |
| Frequent (79-30%) | ||
| HP:0002027 | Abdominal pain | |
| HP:0001654 | Abnormal heart valve morphology | |
| HP:0100643 | Abnormality of nail color | |
| HP:0001369 | Arthritis | |
| HP:0030953 | Conjunctival hyperemia | Diagnostic criterion |
| HP:0002014 | Diarrhea | |
| HP:0000969 | Edema | |
| HP:0011227 | Elevated circulating C-reactive protein concentration | |
| HP:0003565 | Elevated erythrocyte sedimentation rate | |
| HP:0012378 | Fatigue | |
| HP:0001945 | Fever | Diagnostic criterion |
| HP:0000206 | Glossitis | Diagnostic criterion |
| HP:0003073 | Hypoalbuminemia | |
| HP:0001974 | Increased total leukocyte count | |
| HP:0031250 | Lip fissure | Diagnostic criterion |
| HP:0025538 | Palmar edema | Diagnostic criterion |
| HP:0001701 | Pericarditis | |
| HP:0025537 | Plantar edema | Diagnostic criterion |
| HP:0025525 | Scaling skin on fingertip | Diagnostic criterion |
| HP:0031042 | Strawberry tongue | Diagnostic criterion |
| HP:0001894 | Thrombocytosis | |
| Occasional (29-5%) | ||
| HP:0006530 | Abnormal pulmonary interstitial morphology | |
| HP:0011675 | Arrhythmia | |
| HP:0002829 | Arthralgia | |
| HP:0004970 | Ascending tubular aorta aneurysm | |
| HP:0001082 | Cholecystitis | |
| HP:0001635 | Congestive heart failure | |
| HP:0006824 | Cranial nerve paralysis | |
| HP:0011658 | Double outlet right ventricle with subpulmonary ventricular septal defect without pulmonary stenosis | |
| HP:0012115 | Hepatitis | |
| HP:0000737 | Irritability | |
| HP:0000952 | Jaundice | |
| HP:0001287 | Meningitis | |
| HP:0002076 | Migraine | |
| HP:0012819 | Myocarditis | |
| HP:0002017 | Nausea and vomiting | |
| HP:0000508 | Ptosis | |
| HP:0100586 | Sterile pyuria | |
Run the differential on the 8 obligate / very frequent terms
Annotation source: 15505111[PMID]_24162006[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/2331
- This record as JSON: /data/d/2331.json