Review of fracture experience during treatment of Paget's disease of bone with etidronate disodium (EHDP).
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Biomedical subjects
Publications and source records attributed to R D Altman.
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Thirty-two patients with systemic lupus erythematosus were evaluated clinically and with computed tomography in order to determine whether the occurrence of cerebral atrophy in systemic lupus erythematosus was due to the steroid therapy or the cerebral manifestations of the disease itself. Of these patients, 14 had central nervous system manifestations of the disease (lupus cerebritis) and 12 of the 14 were on long-term steroid therapy. Eighteen patients had no clinical evidence of lupus cerebritis and all were on long-term steroids. Of the 14 lupus cerebritis patients, 10 showed moderate cerebral atrophy, four minimal atrophy, and none were normal. Of the 18 patients without lupus cerebritis, none had moderate atrophy, six (33%) showed minimal atrophy, and 12 (67%) had normal CT scans. This data suggest that it is the lupus cerebritis rather than the steroid therapy that is responsible for the moderate cerebral atrophy. In patients suspected of lupus cerebritis, steroids should not be withheld because of concern for steroid-induced atrophy. Rather, the dose may need to be increased.
Prominent scalp vein dilatation was observed in 55% of 71 patients with Paget's disease of bone involving the skull. None of 137 pagetic patients without skull involvement showed visible dilatation of their scalp veins. Following disease suppression with oral etidronate disodium therapy, 21 of 30 patients showed diminution in their scalp vein distension. This "scalp vein sign" may serve as 1 marker of pagetic skull disease and provides another clinical feature of disease activity.
Systemic lupus erythematosus (SLE) and steroid effects on the brain were measured by computed tomography (CT). Of 14 patients with SLE cerebritis, 10 (71%) had marked cortical atrophy and 4 (29%) minimal atrophy. None were normal by CT. Controls included 22 patients with SLE without cerebritis receiving corticosteroids; this group had normal CT scans in 16 (73%) and minimal cortical atrophy in the remaining 6 (27%). Follow-up CT on 5 patients with cerebritis was unchanged. CT of the brain is a minimally invasive technique for documenting SLE cerebritis. CT may also help differentiate cerebritis from the neuropsychiatric side effects of corticosteroids.
We report a case of a patient who presented with symptoms of acute debilitating proximal muscle weakness, which mimicked acute polymyositis, accompanied by a hypopigmented papular rash. The histology of the skin and muscle demonstrated sarcoidosis. A review of the literature of acute sarcoid myositis and the various other myopathies of sarcoidosis is presented.