Systemic capillary leak syndrome

General Information (adopted from Orphanet):

Synonyms, Signs: SCLS
Idiopathic capillary leak syndrome
Capillary hyperpermeability syndrome
Capillary leak syndrome
Clarkson disease
Number of Symptoms 24
OrphanetNr: 188
OMIM Id:
ICD-10: I78.8
UMLs: C0343084
MeSH: D019559
MedDRA: 10007196
Snomed: 87730004

Prevalence, inheritance and age of onset:

Prevalence: < 150 cases [Orphanet]
Inheritance: Not applicable
[Orphanet]
Age of onset: Adult
[Orphanet]

Disease classification (adopted from Orphanet):

Parent Diseases: Rare systemic disease
 -Rare systemic or rheumatologic disease

Symptom Information: Sort by abundance 

1
 (HPO:0011037) Decreased urine output Occasional [Orphanet] 47 / 7739
2
 (HPO:0000083) Renal insufficiency Occasional [Orphanet] 232 / 7739
3
 (HPO:0000124) Renal tubular dysfunction Occasional [Orphanet] 46 / 7739
4
 (HPO:0000246) Sinusitis Frequent [Orphanet] 73 / 7739
5
 (HPO:0001250) Seizures Occasional [Orphanet] 1245 / 7739
6
 (HPO:0002027) Abdominal pain Frequent [Orphanet] 184 / 7739
7
 (HPO:0001733) Pancreatitis Frequent [Orphanet] 46 / 7739
8
 (HPO:0002014) Diarrhea Frequent [Orphanet] 225 / 7739
9
 (HPO:0004325) Decreased body weight Frequent [Orphanet] 492 / 7739
10
 (HPO:0001697) Abnormality of the pericardium Occasional [Orphanet] 52 / 7739
11
 (HPO:0002615) Hypotension Frequent [Orphanet] 52 / 7739
12
 (HPO:0001695) Cardiac arrest Occasional [Orphanet] 87 / 7739
13
 (HPO:0001637) Abnormality of the myocardium Occasional [Orphanet] 76 / 7739
14
 (HPO:0011675) Arrhythmia Occasional [Orphanet] 226 / 7739
15
 (HPO:0001974) Leukocytosis Very frequent [Orphanet] 33 / 7739
16
 (HPO:0006775) Multiple myeloma Occasional [Orphanet] 10 / 7739
17
 (HPO:0004936) Venous thrombosis Occasional [Orphanet] 41 / 7739
18
 (HPO:0100598) Pulmonary edema Frequent [Orphanet] 17 / 7739
19
 (HPO:0010741) Edema of the lower limbs Very frequent [Orphanet] 34 / 7739
20
 (HPO:0001945) Fever Occasional [Orphanet] 218 / 7739
21
 (HPO:0002103) Abnormality of the pleura Occasional [Orphanet] 58 / 7739
22
 (HPO:0003326) Myalgia Frequent [Orphanet] 143 / 7739
23
 (HPO:0001324) Muscle weakness Frequent [Orphanet] 859 / 7739
24
 ([DEL]MedDRA:10011224) Cough Occasional [Orphanet] 70 / 7739

Associated genes:

ClinVar (via SNiPA)

Gene symbol Variation Clinical significance Reference

Additional Information: