A 41 year-old African-American woman with recently diagnosed pericarditis presented actively seizing with fixed gaze deviation to the right. When levetiracetam and lorazepam did not break seizure activity, she required sedation with propofol and intubation. Within 2 days she was successfully extubated, but began to develop a fever, confusion, agitation and visual hallucinations. Upon further questioning, the patient’s mother reported similar strange behaviors in the week preceding the seizure.
Electrolytes were within normal limits. Infectious work-up of the blood, urine and cerebrospinal fluid was negative. Electroencephalogram was negative for continued seizure activity. 24-hour urine protein excretion rate was 940 mg/day. Contrasted magnetic resonance imaging of the brain revealed bilateral cortical and thalamic hyper intensities, as well as an acute left-sided occipital infarct. SS-B antibody was positive and antinuclear antibody (ANA) was reactive with a titer of 1:640. The patient was promptly started on systemic corticosteroids with eventual improvement in confusion, agitation and hallucinations.
Systemic lupus erythematosus (SLE) is a complex autoimmune disease that afflicts many patients in care of the internist. It can be diagnosed using the 2012 Systemic Lupus International Collaborating Clinics (SLICC) classification, which details the need for 4 of 17 criteria, including at least 1 clinical and 1 immunologic criterion or biopsy-proven lupus nephritis in the presence of autoantibodies. Alternative diagnoses such as medication effect, infection or electrolyte abnormalities must first be excluded.
Neuropsychiatric lupus (NPSLE), formerly known as “lupus cerebritis,” encompasses the many manifestations of the disease within the central nervous system (CNS). In some studies, the frequency of cerebrovascular disease, aseptic meningitis, cognitive disorders, headaches and psychiatric disorders attributed to lupus may approach 30 percent. Diagnosis of NPSLE is clinical and must be suspected if any of these is present in a patient with lupus.
Our patient meets the required criteria for new SLE diagnosis given evidence of pericarditis, proteinuria over 500 mg/day, thrombocytopenia and significantly elevated ANA titer. She also exhibits many of the syndromes of NPSLE including delirium, psychosis, seizure and stroke.
Prompt treatment of NPSLE has shown significant improvement in morbidity and mortality. Though corticosteroids are the mainstay of therapy, psychotropic agents can be used as an adjunct for associated psychotic and affective disorders. Severe, life-threatening NSPLE that does not respond to steroids should be treated with cyclophosphamide, with consideration for autologous hematopoietic stem cell transplant in refractory cases.
SLE is a complex and variable autoimmune disease. NPSLE can have significant prognostic consequences and thus must be recognized quickly by the hospitalist in order to avoid reduced quality of life, potentially permanent organ damage or even death.
To cite this abstract:Cannon C, Putnam P. ‘roids for the Rage. Abstract published at Hospital Medicine 2016, March 6-9, San Diego, Calif. Abstract 455. Journal of Hospital Medicine. 2016; 11 (suppl 1). https://www.shmabstracts.com/abstract/roids-for-the-rage/. Accessed April 3, 2020.