BILATERAL HYDRONEPHROSIS IN A PATIENT WITH ERDHEIM-CHESTER DISEASE

Abstract

Erdheim-Chester disease is a rare systemic disorder, which involves the retroperitoneum and kidneys in almost a third of the cases. CASE REPORT A 68-year-old white man presented in October 1997 with persistent edema of the lower limbs associated with acute pulmonary edema. He had a history of breathlessness on exertion, which led to the diagnosis of histiocytosis X (Langerhans cell granulomatosis) in 1983, although lung biopsy failed to demonstrate X bodies. Hemoglobin was 10.2 gm./dl. (normal 12 to 16), erythrocyte sedimentation rate 100 (normal 4 to 8) and serum creatinine 130 mmol./l. (normal 50 to 100). Thoracic computerized tomography (CT) showed diffuse interstitial fibrosis, periaortic fibrosis of the descending thoracic aorta and bilateral adrenal hyperplasia. Abdominal CT and magnetic resonance imaging revealed bilateral hydronephrosis associated with right hilar lymphadenopathy (part A of figure), nonspecific adrenal hyperplasia (part B of figure) and retroperitoneal fibrosis at the level of L1 and L2 vertebrae. Excretory urography demonstrated a 3-hour delay in excretion from the left kidney, nonvisualization of the ureters and a heterogeneous appearance of the hips. Bilateral retrograde pyelography revealed bilateral lumbar ureteral stenosis extending 3 to 4 cm. below the ureteropelvic junction. A silicone double pigtail ureteral stent was inserted on both sides. Erdheim-Chester disease was confirmed on radiographs of the humerus, which showed specific signs, such as bilateral and symmetrical metaphyseal sclerosis, cortical thickening and medullary sclerosis. Endocrine tests were normal. CT guided biopsies failed to demonstrate specific histological features of Erdheim-Chester fibrosis. The patient received 1 mg./kg. prednisone daily for 6 months because of persistently elevated erythrocyte sedimentation rate and stable appearance of the fibrosis. He died of myocardial infarction in July 1998. Autopsy findings confirmed the diagnosis of Erdheim-Chester disease with specific pathological characteristics.