ORPHA:47612
Felty syndrome
Synonyms: Splenomegaly-neutropenia-rheumatoid arthritis syndrome
- Type
- Disease
- Group
- Disorder
- Inheritance
- Unknown
- Age of onset
- Adult
- Status
- active
Felty syndrome (FS), also known as ''super rheumatoid'' disease, is a severe form of rheumatoid arthritis (RA), characterized by a triad of RA, splenomegaly and neutropenia, resulting in susceptibility to bacterial infections.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Felty syndrome | Splenomegaly-neutropenia-rheumatoid arthritis syndrome |
| fr | Syndrome de Felty | |
| de | Felty-Syndrom | Splenomegalie-Neutropenie-rheumatoide Arthritis-Syndrom |
| es | Síndrome de Felty | Síndrome de esplenomegalia-neutropenia-artritis reumatoide |
| it | Sindrome di Felty | Sindrome splenomegalia-neutropenia-artrite reumatoide |
| nl | Syndroom van Felty | Splenomegalie - neutropenie - reumatoïde artritis-syndroom |
| pt | Felty syndrome | |
| pl | Zespół Felty'ego | Zespół splenomegalia-neutropenia-reumatoidalne zapalnie stawów |
| cs | Feltyho syndrom | Syndrom zahrnující splenomegalii, neutropenii a revmatoidní artritidu |
| uk | Синдром Фелті | Синдром спленомегалії-нейтропенії-ревматоїдного артриту |
| zh | Felty综合征 | 脾大-嗜中性白血球减少症-风湿性关节炎综合征 |
| tr | Felty sendromu | Splenomegali-nötropeni-romatoid artrit sendromu |
Definition in:
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Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 134750 | E | validated | |
| ICD-10 | M05.0 | NTBT | attributed | validated |
| ICD-11 | FA20.0 | NTBT | index | validated |
| MONDO | 0007603 | E | validated | |
| MeSH | D005258 | E | validated | |
| UMLS | C0015773 | E | validated | |
| GARD | 8234 | E | validated | |
| MedDRA | 10016386 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (2):
- ORPHA:178996Acquired neutropenia
rare immunological diseases - ORPHA:182231Rare rheumatologic disease
rare systemic and rheumatological diseases
No children.
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Genes
0 directOrphanet records no gene directly on this code.
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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.
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HPO phenotypes
36 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0001367 | Abnormal joint morphology | |
| HP:0002829 | Arthralgia | |
| HP:0001369 | Arthritis | |
| HP:0002960 | Autoimmunity | |
| HP:0001875 | Decreased total neutrophil count | |
| HP:0002721 | Immunodeficiency | |
| HP:0001376 | Limitation of joint mobility | |
| HP:0002797 | Osteolysis | |
| HP:0002719 | Recurrent infections | |
| HP:0002205 | Recurrent respiratory infections | |
| HP:0001482 | Subcutaneous nodule | |
| HP:0100769 | Synovitis | |
| Frequent (79-30%) | ||
| HP:0004332 | Abnormal lymphocyte morphology | |
| HP:0001903 | Anemia | |
| HP:0000389 | Chronic otitis media | |
| HP:0002716 | Lymphadenopathy | |
| HP:0100776 | Recurrent pharyngitis | |
| HP:0006532 | Recurrent pneumonia | |
| HP:0012384 | Rhinitis | |
| HP:0000246 | Sinusitis | |
| HP:0001744 | Splenomegaly | |
| HP:0001824 | Weight loss | |
| Occasional (29-5%) | ||
| HP:0005528 | Bone marrow hypocellularity | |
| HP:0100658 | Cellulitis | |
| HP:0100534 | Episcleritis | |
| HP:0007440 | Generalized hyperpigmentation | |
| HP:0002240 | Hepatomegaly | |
| HP:0007400 | Irregular hyperpigmentation | |
| HP:0002665 | Lymphoma | |
| HP:0001701 | Pericarditis | |
| HP:0009830 | Peripheral neuropathy | |
| HP:0002102 | Pleuritis | |
| HP:0002206 | Pulmonary fibrosis | |
| HP:0000010 | Recurrent urinary tract infections | |
| HP:0100806 | Sepsis | |
| HP:0001873 | Thrombocytopenia | |
Run the differential on the 12 obligate / very frequent terms
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/47612
- This record as JSON: /data/d/47612.json