Idiopathic intracranial hypertension as a significant cause of intractable headache in patients with systemic lupus erythematosus: a 15-year experience

Abstract

Objective: To evaluate the occurrence of idiopathic intracranial hypertension (IIH) in patients with systemic lupus erythematosus (SLE) and to describe the manifestations, treatments and outcomes in these patients. Methods: We reviewed the medical records of 1084 patients with SLE followed up from January 1997 to June 2011 in our unit. We identified patients with IIH and analyzed the demographic, clinical and laboratory characteristics of these patients. Results: Among the 1084 SLE patients, 47 underwent cerebrospinal fluid studies because of their intractable headache and eight (17%) of these were diagnosed as IIH. All were females aged 14 to 32 years. Nobody belonged to the obesity group. Headache, nausea, vomiting and blurred vision were the most common presenting symptoms. All patients had active SLE at the time of admission (SLE disease activity index ≥6). Five patients had lupus nephritis. In eight patients, there were two with antiphospholipid antibodies, two with anti-ribosomal P antibodies and six with anti-Ro antibodies. All subjects recovered without any complication after high dose steroid therapy. Conclusions: IIH accounts for a considerable part of the causes of intractable headache in SLE patients and steroids should be considered as a first-line treatment.