Erdheim-Chester disease (ECD) is a rare, non-Langerhans form of histiocytosis of unknown origin. It is characterized by xanthomatous or xanthogranulomatous infiltration of tissues by spumous ("foamy") histiocytes. As of this writing, 178 cases have been reported. ECD is characterized by heterogeneous systemic manifestations. Bone pain is the most frequent symptom. About half of all patients have extraskeletal manifestations. Cardiovascular manifestations of ECD remain underestimated. We report 6 new cases of ECD associated with periaortic fibrosis. In 4 of these cases, the whole aorta had a "coated" aspect. A literature review revealed 66 cases of ECD with cardiovascular involvement. We therefore analyzed 72 ECD patients with cardiovascular involvement: 40 (55.6%) had periaortic "fibrosis," 32 (44.4%) had pericardial involvement, and 22 (30.6%) had myocardial involvement. Six had a right atrial tumor. Symptomatic valvular heart disease (3 aortic and 3 mitral regurgitations) was found in 6 patients. Nineteen patients (26.4%) had heart failure, leading to death in 8 cases. Six patients had renovascular hypertension related to perirenal artery stenosis. Data concerning follow-up were available for 58 (80.6%) patients. Of these, 35 (60.3%) patients died, confirming the severe prognosis of ECD. Cardiovascular complications were responsible for the death of 11 of the 35 patients (31.4%).

Objectives: To determine the range of normal radiographic joint space width (JSW) values and the shape of the normal hip, and the influence of age, sex, dysplasia, coxa profunda, and acetabular roof curve abnormalities on these values. Methods: On routine conventional pelvic radiographs taken in the supine position in patients with no history of hip or lumbar pain, JSW was measured at three points (superolateral, apical, superomedial), together with the VCE, HTE, and neck shaft angles; acetabular depth; and femoral head diameter. Results: 223 radiographs (446 hips) from 127 women and 96 men (mean age 51.3 years) were examined. Interindividual variations in JSW were large (apical site: 4.19 (0.92) mm; range 2-7). Mean JSW values were higher at the superolateral site than at the apical and superomedial sites in nearly 80% of cases. Women had lower JSW values than men. JSW values did not fall with age. Marked right/left JSW asymmetry was seen in 13/221 (5.9%) subjects. Eight cases of acetabular dysplasia (7 unilateral) and 16 cases of coxa profunda were found, but no cases of acetabular protrusion. The JSW was thicker in dysplastic hips, and thinner in hips with coxa profunda. A roof curve abnormality was found in 96/446 (21.5%) hips. Conclusions: Normal JSW values vary widely; the JSW is commonly narrower at the superomedial site than at the apical site, and is sometimes asymmetric. The roof curve is dysmorphic in about 20% of patients. These findings may have important implications for epidemiological studies and early diagnosis of osteoarthritis of the hip.

This series provides a detailed description of bone involvement in Erdheim-Chester disease. Periostitis and partial epiphyseal involvement of the long bones are also features of this disease. (c) RSNA, 2005.

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