IgG4-related dacryoadenitis and sialadenitis
ORPHA:79078Clinical subtypeNot applicableAll ages
Фенотипы (HPO)33
Очень частый (80–99%)4
HP:0007734Enlarged lacrimal glands
HP:0010286Abnormal salivary gland morphology
HP:0010287Abnormality of the submandibular glands
HP:0032300Increased circulating IgG4 level
Частый (30–79%)1
HP:0011801Enlargement of parotid gland
Периодический (5–29%)19
HP:0000217Xerostomia
HP:0000282Facial edema
HP:0000315Abnormality of the orbital region
HP:0000520Proptosis
HP:0000587Abnormal optic nerve morphology
HP:0000629Periorbital fullness
HP:0001097Keratoconjunctivitis sicca
HP:0001824Weight loss
HP:0001945Fever
HP:0002960Autoimmunity
HP:0003261Increased circulating IgA level
HP:0003324Generalized muscle weakness
HP:0003493Antinuclear antibody positivity
HP:0003496Increased circulating IgM level
HP:0008049Abnormality of the extraocular muscles
HP:0012378Fatigue
HP:0032230Cytoplasmic antineutrophil antibody positivity
HP:0100540Palpebral edema
HP:0100614Myositis
Очень редкий (1–4%)9
HP:0000618Blindness
HP:0001873Thrombocytopenia
HP:0001970Tubulointerstitial nephritis
HP:0002716Lymphadenopathy
HP:0005200Retroperitoneal fibrosis
HP:0005994Nodular goiter
HP:0007807Optic nerve compression
HP:0011481Abnormal lacrimal duct morphology
HP:0100646Thyroiditis
Эпидемиология1
| Тип оценки | Класс распространённости | Среднее (на 100к) | Регион | Квалификация |
|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only |
Лекарства и терапия
Источник: Open Targets Platform (в реальном времени)
Клинические исследования
Источник: ClinicalTrials.gov (в реальном времени)