Discussion
This patient had presented with a septic clinical picture and acute encephalopathy that , coupled with her history of psychiatric co-morbidity and history of IV drug use , masked an acute exacerbation of SLE with neuropsychiatric manifestation . The acute encephalopathic presentation of this patient in the emergency room may have been a reflection of the patient ’ s post-ictal state . The new-onset seizures during the inpatient course were a key factor for considering an alternate diagnosis ; they prompted an autoimmune workup and eventually confirmed the diagnosis of SLE .
Systemic lupus erythematosus ( SLE ) is a multi-organ , chronic , autoimmune disorder with widespread inflammation in affected tissues . It is characterized by production of pathogenic autoantibodies against the nucleic material and binding proteins of one ’ s own cells , thereby resulting in the loss of self-tolerance and over-activation of the immune system . 3 Consequently , there is widespread inflammation , which leads to tissue injury and end-organ damage . Clinical presentation can vary depending on what systems are involved in SLE ; these range from dermatologic / cutaneous ( malar rash , photosensitivity ), renal ( glomerulonephritis , acute renal failure ), hematologic ( anemia , thrombocytopenia ), cardiac ( pericarditis ), pulmonary ( pleural effusion , pleurisy ), or central nervous involvement ( aseptic meningitis , cerebritis , seizure ). 3
For our patient , the clinical presentation of SLE exacerbation was with shock , acute encephalopathy , new-onset seizure disorder , and acute renal failure with metabolic acidosis necessitating management with hemodialysis . Initially an infectious etiology was considered , but failure to improve with therapy prompted expansion of the differential diagnosis to include autoimmune disease , considering her clinical picture in combination with her demographics and laboratory and radiologic evidence of multi-system organ involvement ( renal , cardiac , CNS , dermatologic , and hematologic ). SLE was confirmed by laboratory testing ( elevation of ESR and CRP , positive pANCA , decreased C3 and C4 ; positive ANA , and anti-DS-DNA antibodies ).
Neuropsychiatric syndrome in SLE ( NPSLE ) refers to the subgroup of patients presenting with neurologic or psychiatric symptoms of SLE ( see Table 1 ) and is the second leading cause of mortality and morbidity in patients with SLE . 4 The neuropsychiatric manifestations can be primary ( related to the disease activity itself ) or secondary ( related to treatment [ steroid-induced ], infections , metabolic , or other systemic manifestations not related to SLE ). 5 Neurologic and / or psychiatric manifestations can precede , occur simultaneously , or follow the diagnosis of SLE ; in some patients , it may serve as the only or the initial finding , thereby making diagnosis further difficult . Nonetheless , the presence of neuropsychiatric symptoms identifies those individuals with a higher mortality and a poor prognosis versus those without . 4
The presenting neuropsychiatric manifestations of NPSLE can be nonspecific ( headache , cognitive dysfunction , psychosis , seizure ) thus posing a challenge in identifying patients with primary NPSLE as there is no gold standard criteria to support the diagnosis of NPSLE and exclude confounding diagnoses . 2 As previously noted , this patient ’ s past medical history was significant for bipolar disorder and aggressive behavior since the age of 21 years , IV drug abuse , and prior multiple admissions to the psychiatric unit for acute psychosis . She was on anti-psychotic medication for bipolar mood disorder . Acutely , her neuropsychiatric manifestations were masked by her past medical history of bipolar disorder and IV drug abuse . Appropriate treatment for SLE resulted in control and improvement in neuropsychiatric symptoms , and she no longer required therapy with anti-epileptics and anti-psychotic medications .
The presenting neuropsychiatric manifestations of NPSLE can be nonspecific ( headache , cognitive dysfunction , psychosis , seizure ) thus posing a challenge in identifying patients with primary
NPSLE as there is no gold standard criteria to support the diagnosis of NPSLE and exclude confounding diagnoses . 2 As previously noted , this patient ’ s past medical history was significant for bipolar disorder and aggressive behavior since the age of 21 years , IV drug abuse , and prior multiple admissions to the psychiatric unit for acute psychosis . She was on anti-psychotic medication for bipolar mood disorder . Acutely , her neuropsychiatric manifestations were masked by her past medical history of bipolar disorder and IV drug abuse . Appropriate treatment for SLE resulted in control and improvement in neuropsychiatric symptoms , and she no longer required therapy with anti-epileptics and anti-psychotic medications .
Diagnostic Evaluation in NPSLE
The initial step toward defining a uniform diagnostic criterion for NPSLE was the 1999 American College of Rheumatology guidelines . Since that time , numerous studies applying the ACR criteria have been published to compare cohorts for the presentation of neurologic manifestations of SLE . There are relatively few population-based estimates of the prevalence of NPSLE . A recent attempt to quantify the prevalence of NPSLE evaluated nine studies comprising 5057 SLE patients and estimated the overall prevalence of NPSLE to vary from 11 % to 60 % of all SLE patients , with the most common disorders being major depression ( 17 % - 52 %), headaches ( 38.8 % -60 %), seizures ( 26.4 % -63 %), and cerebrovascular diseases ( 26 % -38.8 %). 6 By design , the 1999 ACR criteria are used for diagnosis and other authors have endorsed modifications to develop a more comprehensive guideline to support clinical decision-making and improve patient care . 7
Evaluation of patients with manifestations of NPSLE consists of investigations that first establish the diagnosis of SLE and exclude non-SLE etiologies . The diagnosis is based on clinical picture , cerebrospinal fluid ( CSF ) analysis , electrophysiological studies , neuroimaging , and neuropsychological testing . Current serologic tests are neither sufficiently accurate for diagnosis of NPSLE nor for as-
132 • The Journal of the Arkansas Medical Society www . ArkMed . org