A severe case of systemic lupus erythematosus with cerebral involvement.
We describe an 18-year-old girl who presented with severe systemic lupus erythematosus with multiple organ involvement. The disease was further complicated by recurrent seizures and intracerebral left parieto-occipital bleeding that required neurosurgical treatment. Postoperative rebleeding occurred due to disseminated intravascular coagulation and platelet dysfunction. Catastrophic antiphospholipid syndrome was suspected, but could not be confirmed during follow-up. Additional treatment with plasmapheresis and intravenous pulse cyclophosphamide in combination with corticosteroids was started. Liquor drainage via a ventriculo-peritoneal (vp)-shunt was necessary because of a hydrocephalus malresorptivus. The patient's recovery was slow and incomplete (cachexia and amaurosis persisted). Follow-up was further complicated by an intraperitoneal vp-shunt cyst, which was initially treated conservatively, but finally had to be revised operatively.
Wiedmann
M
M
Department of Internal Medicine II (Gastroenterology), Center of Internal Medicine, University of Leipzig, Leipzig, Germany. wiedm@medizin.uni-leipzig.de
Seidel
W
W
Mende
L
L
Petros
S
S
Engelmann
L
L
D000293:Adolescent; D016736:Antiphospholipid Syndrome / Q000175:diagnosis* / Q000628:therapy*; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000175:diagnosis* / Q000628:therapy*; D020945:Lupus Vasculitis, Central Nervous System / Q000175:diagnosis / Q000628:therapy; D009102:Multiple Organ Failure / Q000175:diagnosis* / Q000628:therapy*; D012720:Severity of Illness Index; D016896:Treatment Outcome