Intracranial hypertension in systemic lupus erythematosus.
OBJECTIVE
Intracranial hypertension (IH) mimicking pseudotumor cerebri (i.e., idiopathic IH) has been reported in individuals with systemic lupus erythematosus (SLE) since the 1960s. Although various mechanisms have been proposed (e.g., venous thrombosis, medication side effect, and immunologic or inflammatory disease) none have been proven to be causal.


METHODS
Retrospective chart review of patients with IH and SLE at a single tertiary care institution.


RESULTS
In a cohort of IIH patients the percentage of patients in our study with SLE was 1% (4 in 410). Three out of the four patients had serologic evidence for a hypercoagulable state but only one had cerebral venous sinus thrombosis. Two patients had onset or worsening of IH in close temporal relationship to steroid withdrawal. The course, treatment, and prognosis of our patients with SLE and intracranial hypertension did not differ significantly from our clinical experience with IIH or with the published natural history data for IIH.


CONCLUSIONS
The association of SLE and IH may occur more than by chance alone. Steroid withdrawal in the treatment of the SLE may be a predisposing or precipitating factor in the development of IH in these patients rather than the inflammatory effects of SLE per se. The hypercoagulable state in some patients with SLE may also produce cerebral venous sinus thrombosis as an additional potential mechanism of IH.
Dave
Sarita
S
Department of Ophthalmology, University of Iowa Hospitals and Clinics, Iowa City, IA 52242, USA.
Longmuir
Reid
R
Shah
Vinay A
VA
Wall
Michael
M
Lee
Andrew G
AG
D000086:Acetazolamide / Q000627:therapeutic use; D000328:Adult; D001778:Blood Coagulation Disorders / Q000150:complications; D004232:Diuretics / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D019586:Intracranial Hypertension / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D007427:Intracranial Pressure; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D008875:Middle Aged; D009765:Obesity / Q000150:complications; D011239:Prednisolone / Q000627:therapeutic use; D012189:Retrospective Studies
D004232:Diuretics; D005938:Glucocorticoids; D011239:Prednisolone; D000086:Acetazolamide