Dermatomyositis
ORPHA:221DiseaseNot applicableAdult, Elderly
Фенотипы (HPO)77
Очень частый (80–99%)10
HP:0000492Abnormal eyelid morphology
HP:0000969Edema
HP:0002960Autoimmunity
HP:0003325Limb-girdle muscle weakness
HP:0003326Myalgia
HP:0003457EMG abnormality
HP:0003701Proximal muscle weakness
HP:0009071Inflammatory myopathy
HP:0010783Erythema
HP:0100539Periorbital edema
Частый (30–79%)32
HP:0001824Weight loss
HP:0002093Respiratory insufficiency
HP:0002205Recurrent respiratory infections
HP:0002206Pulmonary fibrosis
HP:0002207Diffuse reticular or finely nodular infiltrations
HP:0002747Respiratory insufficiency due to muscle weakness
HP:0002829Arthralgia
HP:0002910Elevated circulating hepatic transaminase concentration
HP:0003236Elevated circulating creatine kinase concentration
HP:0003458EMG: myopathic abnormalities
HP:0006530Abnormal pulmonary interstitial morphology
HP:0011362Abnormal hair quantity
HP:0012378Fatigue
HP:0012544Elevated circulating aldolase concentration
HP:0025435Increased circulating lactate dehydrogenase concentration
HP:0025508Gottron's papules
HP:0025535Shawl sign
HP:0034140Anti-SUMO-activating enzyme subunit 1 antibody positivity
HP:0034141Anti-SUMO-activating enzyme subunit 2 antibody positivity
HP:0040324Heliotrope rash
HP:0100614Myositis
HP:0200034Papule
HP:0200042Skin ulcer
HP:0000934Chondrocalcinosis
HP:0000958Dry skin
HP:0000988Skin rash
HP:0000989Pruritus
HP:0001041Facial erythema
HP:0001063Acrocyanosis
HP:0001252Hypotonia
HP:0001369Arthritis
HP:0001597Abnormality of the nail
Периодический (5–29%)34
HP:0000992Cutaneous photosensitivity
HP:0001029Poikiloderma
HP:0001596Alopecia
HP:0001618Dysphonia
HP:0001658Myocardial infarction
HP:0001701Pericarditis
HP:0001879Abnormality of eosinophils
HP:0001945Fever
HP:0002015Dysphagia
HP:0002092Pulmonary arterial hypertension
HP:0002633Vasculitis
HP:0002664Neoplasm
HP:0002665Lymphoma
HP:0003002Breast carcinoma
HP:0008065Aplasia/Hypoplasia of the skin
HP:0008872Feeding difficulties in infancy
HP:0011675Arrhythmia
HP:0011703Sinus tachycardia
HP:0012819Myocarditis
HP:0025536V-sign
HP:0030078Lung adenocarcinoma
HP:0030880Raynaud phenomenon
HP:0033033Anti-MDA5 antibody positivity
HP:0033554Anti-Mi2 antibody positivity
HP:0034105Anti-transcription intermediary factor-1gamma antibody positivity
HP:0034139Anti-SUMO-activating enzyme antibody positivity
HP:0034142Anti-nuclear matrix protein-2 antibody positivity
HP:0034152Anti-histidyl tRNA synthetase antibody positivity
HP:0100585Telangiectasia of the skin
HP:0100658Cellulitis
HP:0100723Gastrointestinal stroma tumor
HP:0100758Gangrene
HP:0430033Gottron sign
HP:6000006Holster sign
Очень редкий (1–4%)1
HP:0010765Palmar hyperkeratosis
Эпидемиология14
| Тип оценки | Класс распространённости | Среднее (на 100к) | Регион | Квалификация |
|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.1 | Australia | Value and class |
| Point prevalence | 1-9 / 100 000 | 1.97 | Australia | Value and class |
| Annual incidence | 1-9 / 1 000 000 | 0.32 | Argentina | Value and class |
| Point prevalence | 1-5 / 10 000 | 10.2 | Argentina | Value and class |
| Annual incidence | 1-9 / 1 000 000 | 0.49 | Spain | Value and class |
| Annual incidence | 1-9 / 100 000 | 1.1 | United States | Value and class |
| Point prevalence | 1-5 / 10 000 | 13 | United States | Value and class |
| Point prevalence | 1-9 / 100 000 | 4.9172 | Norway | Value and class |
| Annual incidence | 1-9 / 1 000 000 | 0.32 | Sweden | Value and class |
| Point prevalence | 1-9 / 100 000 | 3.8 | Sweden | Value and class |
| Annual incidence | 1-9 / 100 000 | 3.5 | Germany | Value and class |
| Point prevalence | 1-5 / 10 000 | 11.3 | Germany | Value and class |
| Annual incidence | 1-9 / 100 000 | 0.9704 | Worldwide | Value and class |
| Point prevalence | 1-9 / 100 000 | 7.5312 | Worldwide | Value and class |
Лекарства и терапия
Источник: Open Targets Platform (в реальном времени)
Клинические исследования
Источник: ClinicalTrials.gov (в реальном времени)