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Systemic lupus erythematosus

ORPHA:536DiseaseNot applicableAdolescent, Adult, Childhood, Infancy

Ассоциированные гены (30)

BANK1
B cell scaffold protein with ankyrin repeats 1
Major susceptibility factor in
OMIM: 610292
PXK
PX domain containing serine/threonine kinase like
Major susceptibility factor in
OMIM: 611450
IGHG1
immunoglobulin heavy constant gamma 1 (G1m marker)
Modifying germline mutation in
OMIM: 147100
CTLA4
cytotoxic T-lymphocyte associated protein 4
Major susceptibility factor in
OMIM: 123890
TREX1
three prime repair exonuclease 1
Major susceptibility factor in
OMIM: 606609
HLA-DRB1
major histocompatibility complex, class II, DR beta 1
Major susceptibility factor in
OMIM: 142857
IL10
interleukin 10
Major susceptibility factor in
OMIM: 124092
MECP2
methyl-CpG binding protein 2
Major susceptibility factor in
OMIM: 300005
FCGR3B
Fc fragment of IgG receptor IIIb
Major susceptibility factor in
OMIM: 610665
C4A
complement C4A (Chido/Rodgers blood group)
Major susceptibility factor in
OMIM: 120810
C4B
complement C4B (Chido/Rodgers blood group)
Major susceptibility factor in
OMIM: 120820
CR2
complement C3d receptor 2
Major susceptibility factor in
OMIM: 120650
IRF5
interferon regulatory factor 5
Major susceptibility factor in
OMIM: 607218
PDCD1
programmed cell death 1
Major susceptibility factor in
OMIM: 600244
PTPN22
protein tyrosine phosphatase non-receptor type 22
Major susceptibility factor in
OMIM: 600716
STAT4
signal transducer and activator of transcription 4
Major susceptibility factor in
OMIM: 600558
FCGR2B
Fc fragment of IgG receptor IIb
Major susceptibility factor in
OMIM: 604590
DNASE1
deoxyribonuclease 1
Major susceptibility factor in
OMIM: 125505
BLK
BLK proto-oncogene, Src family tyrosine kinase
Major susceptibility factor in
OMIM: 191305
ITGAM
integrin subunit alpha M
Major susceptibility factor in
OMIM: 120980
KIAA0319L
KIAA0319 like
Major susceptibility factor in
OMIM: 613535
JAZF1
JAZF zinc finger 1
Major susceptibility factor in
OMIM: 606246
TNIP1
TNFAIP3 interacting protein 1
Major susceptibility factor in
OMIM: 607714
TNFAIP3
TNF alpha induced protein 3
Major susceptibility factor in
OMIM: 191163
UBE2L3
ubiquitin conjugating enzyme E2 L3
Major susceptibility factor in
OMIM: 603721
TNFSF4
TNF superfamily member 4
Major susceptibility factor in
OMIM: 603594
SPP1
secreted phosphoprotein 1
Major susceptibility factor in
OMIM: 166490
IRAK1
interleukin 1 receptor associated kinase 1
Major susceptibility factor in
OMIM: 300283
ETS1
ETS proto-oncogene 1, transcription factor
Major susceptibility factor in
OMIM: 164720
TLR7
toll like receptor 7
Disease-causing germline mutation(s) in
OMIM: 300365

Фенотипы (44)

Очень частый (80–99%)7
HP:0000093Proteinuria
HP:0001824Weight loss
HP:0001945Fever
HP:0002039Anorexia
HP:0003493Antinuclear antibody positivity
HP:0012378Fatigue
HP:0033834Malaise
Частый (30–79%)16
HP:0033726Lupus nephritis
HP:0034076Anti-ribosome Po antibody positivity
HP:0034077Anti-complement component C1q antibody positivity
HP:0045042Decreased circulating complement C4 concentration
HP:5000038Anti-titin antibody positivity
HP:0000790Hematuria
HP:0000822Hypertension
HP:0001369Arthritis
HP:0001596Alopecia
HP:0001882Leukopenia
HP:0003453Antineutrophil antibody positivity
HP:0005421Decreased circulating complement C3 concentration
HP:0005764Polyarticular arthritis
HP:0012085Pyuria
HP:0025300Malar rash
HP:0030880Raynaud phenomenon
Периодический (5–29%)19
HP:0000155Oral ulcer
HP:0000716Depression
HP:0000992Cutaneous photosensitivity
HP:0001250Seizure
HP:0001873Thrombocytopenia
HP:0001878Hemolytic anemia
HP:0002716Lymphadenopathy
HP:0007417Discoid lupus rash
HP:0020151Anti-dsDNA antibody positivity
HP:0032235Anti-La/SS-B antibody positivity
HP:0033028Anti-U1 ribonucleoprotein antibody positivity
HP:0033040Anti-Sm antibody positivity
HP:0033556Anti-nucleoporin 62 antibody positivity
HP:0034093Anti-Ro52/TRIM21 antibody positivity
HP:0034096Anti-phosphatidic acid antibody positivity
HP:0045073Serositis
HP:0100669Abnormal pigmentation of the oral mucosa
HP:0100755Abnormality of salivation
HP:0100825Cheilitis
Очень редкий (1–4%)2
HP:0000488Retinopathy
HP:0002072Chorea

Эпидемиология (11)

Point prevalence
1-5 / 10 000
France
Point prevalence
6-9 / 10 000
Martinique
Point prevalence
1-5 / 10 000
New Zealand
Point prevalence
1-5 / 10 000
Korea, Republic of
Point prevalence
1-5 / 10 000
United States
Point prevalence
1-5 / 10 000
Taiwan, Province of China
Point prevalence
1-5 / 10 000
Europe
Point prevalence
1-5 / 10 000
Finland
Annual incidence
1-9 / 100 000
Worldwide
Point prevalence
1-5 / 10 000
Worldwide
Annual incidence
6-9 / 10 000
Poland

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы