IgG4-related retroperitoneal fibrosis
ORPHA:49041Clinical subtypeNot applicable, UnknownAdult
Фенотипы (HPO)43
Очень частый (80–99%)1
HP:0012531Pain
Частый (30–79%)18
HP:0002027Abdominal pain
HP:0002039Anorexia
HP:0003138Increased blood urea nitrogen
HP:0003259Elevated circulating creatinine concentration
HP:0003419Low back pain
HP:0003565Elevated erythrocyte sedimentation rate
HP:0005310Large vessel vasculitis
HP:0011227Elevated circulating C-reactive protein concentration
HP:0012378Fatigue
HP:0012583Unilateral renal hypoplasia
HP:0025379Anti-thyroid peroxidase antibody positivity
HP:0030157Flank pain
HP:0031191Deep dermal perivascular inflammatory infiltrate
HP:0000083Renal insufficiency
HP:0000126Hydronephrosis
HP:0000822Hypertension
HP:0001824Weight loss
HP:0001897Normocytic anemia
Периодический (5–29%)14
HP:0000074Ureteropelvic junction obstruction
HP:0000872Hashimoto thyroiditis
HP:0001370Rheumatoid arthritis
HP:0001919Acute kidney injury
HP:0001945Fever
HP:0002017Nausea and vomiting
HP:0002019Constipation
HP:0002725Systemic lupus erythematosus
HP:0002923Rheumatoid factor positive
HP:0003262Smooth muscle antibody positivity
HP:0003453Antineutrophil antibody positivity
HP:0003765Psoriasiform dermatitis
HP:0010741Pedal edema
HP:0012578Membranous nephropathy
Очень редкий (1–4%)10
HP:0000034Hydrocele testis
HP:0000100Nephrotic syndrome
HP:0000790Hematuria
HP:0000802Impotence
HP:0002639Budd-Chiari syndrome
HP:0008682Renal tubular epithelial necrosis
HP:0012871Varicocele
HP:0012877Retrograde ejaculation
HP:0100518Dysuria
HP:0100817Renovascular hypertension
Эпидемиология3
| Тип оценки | Класс распространённости | Среднее (на 100к) | Регион | Квалификация |
|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.35 | Europe | Value and class |
| Point prevalence | 1-9 / 100 000 | — | Finland | Class only |
| Point prevalence | 1-9 / 100 000 | — | Europe | Class only |
Лекарства и терапия
Источник: Open Targets Platform (в реальном времени)
Клинические исследования
Источник: ClinicalTrials.gov (в реальном времени)