Objective: to investigate the clinical characteristics of systemic lupus erythematosus with diffuse intracranial calcification.

Methods: The clinical characteristics of one case of systemic lupus erythematosus with diffuse intracranial calcification were analyzed, and 12 cases in related literatures were reviewed by searching Medline and Wanfang database.

Results: Our case and 12 cases reviewed were all female. With the exception of one case, the course of SLE was more than 5 years. The clinical manifestations of the nervous system are diverse, including epilepsy, hemiplegia, cognitive impairment, and mental abnormalities. In the presence of neuropsychiatric manifestations, this case and six cases reviewed had SLE activity. Cerebrospinal fluid (CSF) examination was performed in seven patients, including four patients with CSF protein elevation, two patients with IL-6 elevation, and one patient with anti-ribosomal antibody elevation. This case and 10 of 12 cases reviewed had bilateral basal ganglia calcification. Intracranial calcification was very high density on CT and showed high T1WI and low T2WI signal on MRI.

Conclusion: Systemic lupus erythematosus with intracranial calcification is a rare and severe manifestation of SLE, which is not completely parallel to SLE activity. The clinical manifestations of the nervous system are diverse, and bilateral basal ganglia calcification is the most common in imaging. High T1WI signal and low T2WI signal may be used as one of the imaging features to identify intracranial calcification.

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http://dx.doi.org/10.1177/09612033221122674DOI Listing

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