Background and Purpose-Advanced cerebrovascular wall calcification was recently observed at autopsy in 2 patients with systemic sclerosis. To further investigate this issue, we conducted a prospective CT study of scleroderma patients to detect intracerebral calcification. Methods-Thirty-seven consecutive patients with systemic sclerosis underwent unenhanced brain CT. Images were blindly interpreted, together with those of 2 age-matched (Ϯ1 year) and sex-matched control subjects per patient. Results-Intracerebral calcification was found in 12 patients (32.4%) and 7 controls (9.5%) (Pϭ0.006). Among the patients, intracerebral calcification was associated with the duration of Raynaud's phenomenon (Pϭ0.005) and not with age (Pϭ0.086). Conclusions-Intracerebral calcification is closely associated with scleroderma, which suggests that scleroderma causes primary cerebrovascular changes. (Stroke. 1999;30:2183-2185.)Key Words: basal ganglia Ⅲ calcification Ⅲ scleroderma, systemic Ⅲ tomography, x-ray computed S mall-artery changes have been observed in nearly every organ in scleroderma. 1 One exception is the brain, where the distinction between primary lesions and lesions secondary to arterial hypertension and renal disease could not be established in the few available pathological reports. 2 We recently described 2 patients with scleroderma, central nervous system (CNS) manifestations, and autopsy evidence of small cerebral artery wall calcification, mainly in the basal ganglia. 3 Both patients also had mild basal ganglia calcification on brain CT, which is the most frequently observed CT abnormality in the brain of patients with cerebral lupus. 4 Because of the young age of the 2 patients and the absence of other known causes of cerebrovascular calcification, we postulated that the latter were primarily related to scleroderma. 3 To confirm this we conducted a prospective CT study to detect intracerebral calcification in patients with systemic sclerosis.
Subjects and Methods
SubjectsBetween October 1997 and June 1998, 37 consecutive scleroderma patients hospitalized in our department, mainly for elective investigations, underwent unenhanced brain CT. There were 31 women and 6 men, aged 21 to 78 years (meanϮSD 54Ϯ14 years). The main characteristics of the study population are shown in the Table. Sixteen patients (43%) had diffuse scleroderma according to the American Rheumatism Association (ARA) criteria, 5 and 21 (57%) had the limited form of systemic sclerosis. Among the ARA diagnostic criteria: all the patients had Raynaud's phenomenon, 68% had sclerodactyly, 49% had a history of digital ulceration, 35% had pulmonary fibrosis, and 16% had cutaneous sclerosis proximal to the knees and/or elbows. Screening for antinuclear antibodies was positive in all 37 patients, at a dilution of 1/500 or more in 32 (86%).
MethodsBrain CT studies were performed with an electron beam CT (Evolution, Siemens Inc) with 6-mm unenhanced contiguous slices through the whole brain. Between January 1997 and June 1998, 1240 patients underwent br...