Primary Sjögren disease
ORPHA:289390DiseaseNot applicableAdult
Фенотипы (HPO)82
Очень частый (80–99%)2
HP:0000217Xerostomia
HP:0001097Keratoconjunctivitis sicca
Частый (30–79%)17
HP:0000077Abnormality of the kidney
HP:0000707Abnormality of the nervous system
HP:0000739Anxiety
HP:0000951Abnormality of the skin
HP:0001970Tubulointerstitial nephritis
HP:0002829Arthralgia
HP:0003011Abnormality of the musculature
HP:0004431Complement deficiency
HP:0005195Polyarticular arthropathy
HP:0011850Parotitis
HP:0012378Fatigue
HP:0012532Chronic pain
HP:0031950Usual interstitial pneumonia
HP:0033555Anti-Ro/SS-A antibody positivity
HP:0034064Anti-salivary protein antibody positivity
HP:0034066Anti-carbonic anhydrase VI antibody positivity
HP:0034069Anti-parotid secretory protein antibody positivity
Периодический (5–29%)54
HP:0000083Renal insufficiency
HP:0000099Glomerulonephritis
HP:0000708Atypical behavior
HP:0000716Depression
HP:0000958Dry skin
HP:0000965Cutis marmorata
HP:0000979Purpura
HP:0001045Vitiligo
HP:0001250Seizure
HP:0001287Meningitis
HP:0001317Abnormal cerebellum morphology
HP:0001324Muscle weakness
HP:0001369Arthritis
HP:0001871Abnormality of blood and blood-forming tissues
HP:0001873Thrombocytopenia
HP:0001882Leukopenia
HP:0001888Lymphopenia
HP:0001895Normochromic anemia
HP:0001897Normocytic anemia
HP:0002011Morphological central nervous system abnormality
HP:0002613Biliary cirrhosis
HP:0002633Vasculitis
HP:0002665Lymphoma
HP:0002716Lymphadenopathy
HP:0003326Myalgia
HP:0003474Somatic sensory dysfunction
HP:0004302Functional motor deficit
HP:0004313Decreased circulating antibody level
HP:0005421Decreased circulating complement C3 concentration
HP:0005523Lymphoproliferative disorder
HP:0006527Lymphoid interstitial pneumonia
HP:0006530Abnormal pulmonary interstitial morphology
HP:0006536Airway obstruction
HP:0010702Increased circulating antibody level
HP:0012089Arteritis
HP:0012219Erythema nodosum
HP:0012387Bronchitis
HP:0020151Anti-dsDNA antibody positivity
HP:0030880Raynaud phenomenon
HP:0031088Vaginal dryness
HP:0031246Nonproductive cough
HP:0031452Lichenoid skin lesion
HP:0031983Abnormal pulmonary thoracic imaging finding
HP:0032218Decreased proportion of CD4-positive T cells
HP:0034076Anti-ribosome Po antibody positivity
HP:0034093Anti-Ro52/TRIM21 antibody positivity
HP:0045042Decreased circulating complement C4 concentration
HP:0100614Myositis
HP:0100646Thyroiditis
HP:0100653Optic neuritis
HP:0100778Cryoglobulinemia
HP:0200042Skin ulcer
HP:0200123Chronic hepatitis
HP:0410008Abnormality of the peripheral nervous system
Очень редкий (1–4%)9
HP:0000726Dementia
HP:0002072Chorea
HP:0002143Abnormality of the spinal cord
HP:0007141Sensorimotor neuropathy
HP:0009830Peripheral neuropathy
HP:0032018Multiple mononeuropathy
HP:0100543Cognitive impairment
HP:0100583Corneal perforation
HP:0200120Chronic active hepatitis
Эпидемиология14
| Тип оценки | Класс распространённости | Среднее (на 100к) | Регион | Квалификация |
|---|---|---|---|---|
| Point prevalence | 1-5 / 10 000 | 11.343 | France | Value and class |
| Point prevalence | 1-5 / 10 000 | 48.99 | Europe | Value and class |
| Point prevalence | 6-9 / 10 000 | 86.398 | Greece | Value and class |
| Point prevalence | 1-5 / 10 000 | 49.745 | Norway | Value and class |
| Point prevalence | 1-5 / 10 000 | 45.47 | Denmark | Value and class |
| Annual incidence | 1-9 / 100 000 | 3.95 | United States | Value and class |
| Point prevalence | 1-5 / 10 000 | 37.15 | Taiwan, Province of China | Value and class |
| Point prevalence | >1 / 1000 | 207.83 | Turkey | Value and class |
| Point prevalence | 1-5 / 10 000 | 30.91 | Italy | Value and class |
| Point prevalence | >1 / 1000 | 165.98 | Brazil | Value and class |
| Annual incidence | 1-9 / 100 000 | 9.5 | Taiwan, Province of China | Value and class |
| Annual incidence | 1-9 / 100 000 | 5.3 | Greece | Value and class |
| Annual incidence | 1-9 / 100 000 | 3.9 | Slovenia | Value and class |
| Annual incidence | 1-9 / 100 000 | 6.92 | Worldwide | Value and class |
Лекарства и терапия
Источник: Open Targets Platform (в реальном времени)
Клинические исследования
Источник: ClinicalTrials.gov (в реальном времени)