Systemic lupus erythematosus

Idiopathic intracranial hypertension as an initial presentation of systemic lupus erythematosus

Authors: Maloney K.

A 14-year-old girl with no known illness presented with a several week history of headaches and vomiting. The patient also reported having joint pain and swelling to the wrists and knees. She had no prior history of headaches, use of hormonal contraception or other medications, recent weight changes or family history of autoimmune disease. Blood pressure temperature, height and weight were normal. She was alert, there was alopecia, cervical lympadenopathy, symmetrical synovitis to the wrists, bilateral papilloedema and cranial nerve VI palsy. Laboratory investigations revealed a normochromic normocytic anaemia, leucopenia and lymphopenia. Serum chemistries were normal. CT of the brain was normal. Lumbar puncture revealed an opening pressure of greater than 300 mm H2O; cerebrospinal fluid (CSF) analysis was normal. HIV antibodies were non-reactive. Despite treatment with acetazolamide she developed somnolence. Hence MR venography was performed which showed no evidence of cerebral vein thrombosis. Further investigations revealed a positive direct coombs test, positive antinuclear antibodies (ANA) positive antidouble-stranded DNA (dsDNA) and false positive VDRL. Complement levels were reduced. Anti-Smith, anticardiolipin antibodies and lupus anticoagulant were negative.

Idiopathic intracranial hypertension as the initial presentation of systemic lupus erythematosus

Authors: Rajasekharan C, Renjith SW, Marzook A, Parvathy R.

A 14-year-old girl was referred for evaluation of headache with episodes of transient blurring of vision, and intermittent fever for 4 weeks. On examination she was conscious and febrile, with multiple annular purpuric skin lesions present over the face and back. Neurological examination revealed a bilaterally extensor plantar response, with bilateral papilloedema. Lumbar puncture yielded clear spinal fluid with a very high opening pressure with a normal biochemistry and cytology. Neuroimaging showed evidence of raised intracranial tension. She was provisionally diagnosed to have idiopathic intracranial hypertension (IIH) and started on anticerebral oedema measures. Despite medication, she continued to be symptomatic. On the sixth day of admission, her antinuclear antibody and antidouble-stranded DNA registered positively in high titres. She was diagnosed with systemic lupus erythematosus (SLE) with IIH and was started on corticosteroids, with dramatic recovery of her symptoms and clinical signs. Reports of SLE, the maiden presentation of which is IIH, are rare in the literature.

Intracranial hypertension associated with systemic lupus erythematosus in a young male patient

Authors: Tse C, Klein R.

Systemic lupus erythematosus (SLE) is a chronic disease that affects multiple organ systems including the central nervous system. We present a case of a young male with a new diagnosis of SLE presenting as intracranial hypertension (IH). Review of the literature suggests an association between SLE and IH and prompts a reconsideration of the exclusion of IH from the American College of Rheumatology's accepted neuropsychiatric manifestations. In addition, our case is one of the few cases of IH and SLE in males. While the data are limited, SLE-associated IH in males may be different from that in females with respect to presentation and severity.

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

Authors: Kim JM, Kwok SK, Ju JH, Kim HY, Park SH.

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.

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