ORPHA:188
Systemic capillary leak syndrome
Synonyms: Capillary hyperpermeability syndrome · Capillary leak syndrome · Clarkson disease · Idiopathic capillary leak syndrome · SCLS
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
Systemic capillary leak syndrome (SCLS) is a severe systemic disease due to increased capillary permeability, characterized by episodes of hypotension, edema and hypovolemia.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Systemic capillary leak syndrome | Capillary hyperpermeability syndrome · Capillary leak syndrome · Clarkson disease · Idiopathic capillary leak syndrome · SCLS |
| fr | Syndrome de fuite capillaire systémique | Syndrome d'hyperperméabilité capillaire · Syndrome de fuite capillaire · Syndrome de fuite capillaire idiopathique · Maladie de Clarkson · SCLS · SFC · SFCI |
| de | Capillary-Leak-Syndrom, systemisches | Clarkson-Krankheit · Kapillarleck-Syndrom |
| es | Síndrome de extravasación capilar sistémica | SCLS · Síndrome de Clarkson · Síndrome de fuga capilar · Síndrome de fuga capilar idiopática · Síndrome de hiperpermeabilidad capilar |
| it | Sindrome sistemica da aumentata permeabilità capillare | Malattia di Clarkson · SCLS · Sindrome da aumentata permeabilità capillare · Sindrome da iperpermeabilità capillare · Sindrome idiopatica da aumentata permeabilità capillare |
| nl | Systemisch capillair leksyndroom | Capillair leksyndroom · Capillaire hyperpermeabiliteitsyndroom · Idiopathisch capillair leksyndroom · SCLS · Ziekte van Clarkson |
| pt | Síndrome de derrame capilar sistémico | Doença de Clarkson · SCLS · Síndrome de derrame capilar · Síndrome de derrame capilar idiopático · Síndrome de hiperpermeabilidade capilar |
| pl | Uogólniony zespół przesiąkania włośniczek | Choroba Clarksona · Idiopatyczny zespół przesiąkania włośniczek · SCLS · Zespół przesiąkania włośniczek · Zespół zwiększonej przepuszczalności naczyń włosowatych |
| cs | Systémový syndrom děravých kapilár | Syndrom hyperpermeability kapilár · Clarksonova choroba · SCLS · Syndrom děravých kapilár · Idiopatický syndrom děravých kapilár |
| uk | Синдром системного капілярного просочування | Синдром гіперпроникності капілярів · Синдром капілярного витоку · Хвороба Кларксона · Синдром ідіопатичного капілярного просочування · SCLS |
| zh | 系统性毛细血管渗漏综合征 | 毛细血管高渗透性综合征 |
| tr | Sistemik kılcal sızıntı sendromu | SCLS · Clarkson hastalığı · Kılcal sızıntı sendromu · İdiyopatik kılcal sızıntı sendromu · Kılcal aşırı geçirgenlik sendromu |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | I78.8 | NTBT | attributed | validated |
| ICD-11 | BD5Y | NTBT | index | validated |
| MONDO | 1956 | E | validated | |
| MeSH | D019559 | E | validated | |
| UMLS | C0343084 | E | validated | |
| GARD | 1084 | E | validated | |
| MedDRA | 10007196 | 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 (1):
- ORPHA:182222Rare systemic 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
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 150 cases | Worldwide | Case(s) | Validated | [EXPERT] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | 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
23 annotations · validated 2018-01-22| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0001974 | Increased total leukocyte count | |
| HP:0010741 | Pedal edema | |
| Frequent (79-30%) | ||
| HP:0002027 | Abdominal pain | |
| HP:0025142 | Constitutional symptom | |
| HP:0002014 | Diarrhea | |
| HP:0012378 | Fatigue | |
| HP:0002615 | Hypotension | |
| HP:0003326 | Myalgia | |
| HP:0001733 | Pancreatitis | |
| HP:0100598 | Pulmonary edema | |
| HP:0031417 | Rhinorrhea | |
| HP:0001824 | Weight loss | |
| Occasional (29-5%) | ||
| HP:0000091 | Abnormal renal tubule morphology | |
| HP:0011675 | Arrhythmia | |
| HP:0006543 | Cardiorespiratory arrest | |
| HP:0012735 | Cough | |
| HP:0006775 | Multiple myeloma | |
| HP:0012819 | Myocarditis | |
| HP:0100520 | Oliguria | |
| HP:0001701 | Pericarditis | |
| HP:0002202 | Pleural effusion | |
| HP:0000083 | Renal insufficiency | |
| HP:0004936 | Venous thrombosis | |
Run the differential on the 2 obligate / very frequent terms
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/188
- This record as JSON: /data/d/188.json