Loeffler endocarditis
Synonyms: Eosinophilic endocarditis
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adolescent, Adult, Childhood
- Status
- active
A rare restrictive cardiomyopathy characterized by hypereosinophilia and fibrous thickening of the endocardium, with usually large thrombi against the ventricle walls, that can lead to cardiovascular complications such as heart failure and thromboembolism. It manifests with symptoms like edema, fatigue and shortness of breath. It is usually secondary to eosinophil-associated tissue damage and is associated with idiopathic hypereosinophilic syndrome, chronic eosinophilic leukemia, carcinoma, or lymphoma.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Loeffler endocarditis | Eosinophilic endocarditis |
| fr | Endocardite de Loeffler | Endocardite à éosinophiles |
| de | Löffler-Endokarditis | Endokarditis, eosinophile |
| es | Endocarditis de Loeffler | Endocarditis eosinofílica |
| it | Endocardite di Loeffler | Endocardite eosinofila |
| nl | Loeffler-endocarditis | Eosinofiele endocarditis |
| pt | Endocardite de Loeffler | Endocardite eosínofílica |
| pl | Zapalenie wsierdzia Loefflera | Eozynofilowe zapalenie wsierdzia |
| cs | Löfflerova endokarditida | Eozinofilní endokarditida |
| uk | Ендокардит Леффлера | Еозинофільний ендокардит |
| zh | 嗜酸细胞增多性心肌心内膜炎 | 嗜酸性粒细胞增多性心内膜炎 |
| tr | Loeffler endokarditi | Eozinofilik endokardit |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | I42.3 | NTBT | inclusion | validated |
| ICD-11 | BC43.20 | NTBT | index | validated |
| MONDO | 0019159 | E | validated | |
| UMLS | C0206143 | E | validated | |
| MedDRA | 10052841 | 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:217720Non-familial restrictive cardiomyopathy
rare cardiac 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
27 annotations · validated 2021-09-21| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0001977 | Abnormal thrombosis | |
| HP:0001880 | Increased total eosinophil count | |
| HP:0031323 | Myocardial eosinophilic infiltration | |
| HP:0001723 | Restrictive cardiomyopathy | |
| Frequent (79-30%) | ||
| HP:0031331 | Abnormal cardiomyocyte morphology | |
| HP:0001654 | Abnormal heart valve morphology | |
| HP:0100749 | Chest pain | |
| HP:0001635 | Congestive heart failure | |
| HP:0002094 | Dyspnea | |
| HP:0012378 | Fatigue | |
| HP:0025168 | Left ventricular diastolic dysfunction | |
| HP:0001712 | Left ventricular hypertrophy | |
| HP:0001962 | Palpitations | |
| HP:0001824 | Weight loss | |
| Occasional (29-5%) | ||
| HP:0025523 | Abnormal morphology of the chordae tendinae of the mitral valve | |
| HP:0031442 | Abnormal tricuspid chordae tendinae morphology | |
| HP:0011675 | Arrhythmia | |
| HP:0012735 | Cough | |
| HP:0006685 | Endocardial fibrosis | |
| HP:0031295 | Left atrial enlargement | |
| HP:0001653 | Mitral regurgitation | |
| HP:0001685 | Myocardial fibrosis | |
| HP:0011712 | Right bundle branch block | |
| HP:0010872 | T-wave inversion | |
| Very rare (<4-1%) | ||
| HP:0001659 | Aortic regurgitation | |
| HP:0001650 | Aortic valve stenosis | |
| HP:0001701 | Pericarditis | |
Run the differential on the 4 obligate / very frequent terms
Annotation source: 32999784[PMID]_33117933[PMID]_33129649[PMID]_30521271[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/75566
- This record as JSON: /data/d/75566.json