Nature clinical practice. Rheumatology · 2008
A case of Erdheim-Chester disease initially mistaken for Ormond's disease.
Клинический случайСтатьяНазвание и аннотация: Перевод пока не подготовлен
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Loddenkemper Konstanze, Hoyer Bimba, Loddenkemper Christoph, Hermann Kay-Geert, Rogalla Patrick, Förster Gregor, Buttgereit Frank, Hiepe Falk, Burmester Gerd-Rüdiger
Аннотация
BACKGROUND: A 54-year-old man presented with fever, abdominal pain, anemia, elevated C-reactive protein level and decreased renal function. Idiopathic retroperitoneal fibrosis (Ormond's disease) had been diagnosed in the past, leading to surgical ureterolysis. Re-evaluation of the CT scans and histological biopsy samples revealed unusual sclerosis of the long bones and diffuse infiltrates of foamy histiocytes in the bone marrow and the retroperitoneum.
INVESTIGATIONS: Physical examination, laboratory tests including hemoglobin concentration, erythrocyte sedimentation rate, C-reactive protein level, beta2-microglobulin level, creatinine level, CT of the chest and abdomen, bone scintigraphy, bone marrow and soft tissue biopsies and immunohistochemistry.
DIAGNOSIS: Erdheim-Chester disease with retroperitoneal fibrosis and bone sclerosis.
MANAGEMENT: Treatment with glucocorticoids failed. The patient's symptoms improved significantly after initiation of interferon-alpha therapy.
INVESTIGATIONS: Physical examination, laboratory tests including hemoglobin concentration, erythrocyte sedimentation rate, C-reactive protein level, beta2-microglobulin level, creatinine level, CT of the chest and abdomen, bone scintigraphy, bone marrow and soft tissue biopsies and immunohistochemistry.
DIAGNOSIS: Erdheim-Chester disease with retroperitoneal fibrosis and bone sclerosis.
MANAGEMENT: Treatment with glucocorticoids failed. The patient's symptoms improved significantly after initiation of interferon-alpha therapy.