IgG4-related systemic disease
ORPHA:596448DiseaseNot applicableAdolescent, Adult, Childhood, Elderly
Фенотипы (HPO)30
Частый (30–79%)11
HP:0000872Hashimoto thyroiditis
HP:0001733Pancreatitis
HP:0003493Antinuclear antibody positivity
HP:0005200Retroperitoneal fibrosis
HP:0005421Decreased circulating complement C3 concentration
HP:0030991Sclerosing cholangitis
HP:0030992Abnormal pancreatic duct morphology
HP:0031281Sialadenitis
HP:0032300Increased circulating IgG4 level
HP:0045042Decreased circulating complement C4 concentration
HP:0000315Abnormality of the orbital region
Периодический (5–29%)6
HP:0002923Rheumatoid factor positive
HP:0003212Increased circulating IgE level
HP:0003237Increased circulating IgG level
HP:0003565Elevated erythrocyte sedimentation rate
HP:0005339Abnormality of complement system
HP:0011227Elevated circulating C-reactive protein concentration
Очень редкий (1–4%)1
HP:0032252Granuloma
Исключён (0%)12
HP:0001744Splenomegaly
HP:0001873Thrombocytopenia
HP:0001880Eosinophilia
HP:0001882Leukopenia
HP:0001945Fever
HP:0032229Perinuclear antineutrophil antibody positivity
HP:0032235Anti-La/SS-B antibody positivity
HP:0033028Anti-U1 ribonucleoprotein antibody positivity
HP:0033040Anti-Sm antibody positivity
HP:0033555Anti-Ro/SS-A antibody positivity
HP:0100280Crohn's disease
HP:0100778Cryoglobulinemia
Эпидемиология6
| Тип оценки | Класс распространённости | Среднее (на 100к) | Регион | Квалификация |
|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only |
| Annual incidence | Unknown | — | Worldwide | Class only |
| Point prevalence | 1-9 / 100 000 | — | Japan | Class only |
| Annual incidence | 1-9 / 100 000 | 1.026 | Japan | Value and class |
| Point prevalence | 1-9 / 1 000 000 | 0.576 | Denmark | Value and class |
| Annual incidence | 1-9 / 1 000 000 | 0.6885 | Denmark | Value and class |
Лекарства и терапия
Источник: Open Targets Platform (в реальном времени)
Клинические исследования
Источник: ClinicalTrials.gov (в реальном времени)