Intracranial Hypertension in Systemic Lupus Erythematosus

Department of Ophthalmology, University of Iowa Hospitals and Clinics, Iowa City, IA 52242, USA.
Seminars in Ophthalmology (Impact Factor: 0.86). 07/2009; 23(2):127-33. DOI: 10.1080/08820530801888188
Source: PubMed


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

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    • "The proposed mechanisms include immune-mediated injury within the arachnoid villi and consequent reduction in CSF absorption or probable hypercoagulable state without overt vascular thrombosis giving rise to micro-obliteration of cerebral arteriolar and venous systems.[279] Steroid withdrawal in the treatment of the SLE may be a predisposing or precipitating factor in the development of IH in patients.[10] In a review of 127 patients with LN over 11 years, six patients had IH, which gave a disease prevalence of 4.7% in those with LN. "
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    ABSTRACT: A 14-year-old male presented with bilateral papilledema, growth retardation and absent secondary sexual characters. He had a past history of fever, headache and fatigue of 6 months duration. The diagnosis of intracranial hypertension (IH) was confirmed by an increased intracranial pressure and normal neuroimaging studies of the brain, except for partial empty sella, prominent perioptic cerebrospinal fluid (CSF) spaces and buckling of optic nerves. Evaluation showed erythrocyte sedimentation rate (ESR) of 150 mm/hr, positive antinuclear antibody (ANA), anti dsDNA and anti ribosomal P protein. Renal biopsy revealed diffuse segmental proliferative lupus nephritis (LN) class IV S (A) confirming the diagnosis of systemic lupus erythematosus (SLE). Treatment of LN with intravenous pulse methyl prednisolone and cyclophosphamide was effective in normalizing the CSF pressure, resulting in express and dramatic resolution of symptomatology. In a case of IH, SLE must be considered. IH, growth retardation and absence of sexual characters may be presenting manifestations of a chronic systemic inflammatory disease like SLE. These manifestations may act as a pointer to associated advanced grades of LN, which can be totally asymptomatic and missed without a renal biopsy.
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