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Limited cutaneous systemic sclerosis

ORPHA:220402Clinical subtypeMultigenic/multifactorial, Not applicableAdult

Ассоциированные гены (6)

CAV1
caveolin 1
Major susceptibility factor in
OMIM: 601047
HLA-DRB1
major histocompatibility complex, class II, DR beta 1
Major susceptibility factor in
OMIM: 142857
CCN2
cellular communication network factor 2
Major susceptibility factor in
OMIM: 121009
IRF5
interferon regulatory factor 5
Major susceptibility factor in
OMIM: 607218
KIAA0319L
KIAA0319 like
Major susceptibility factor in
OMIM: 613535
CCR6
C-C motif chemokine receptor 6
Major susceptibility factor in
OMIM: 601835

Фенотипы (15)

Очень частый (80–99%)5
HP:0000951Abnormality of the skin
HP:0001000Abnormality of skin pigmentation
HP:0001053Hypopigmented skin patches
HP:0002960Autoimmunity
HP:0100958Narrow foramen obturatorium
Частый (30–79%)6
HP:0002015Dysphagia
HP:0002017Nausea and vomiting
HP:0002020Gastroesophageal reflux
HP:0100579Mucosal telangiectasiae
HP:0100585Telangiectasia of the skin
HP:0200042Skin ulcer
Периодический (5–29%)4
HP:0002092Pulmonary arterial hypertension
HP:0002206Pulmonary fibrosis
HP:0008366Foot joint contracture
HP:0009473Joint contracture of the hand

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы