NEUROPSYCHIATRIC SYSTEMIC LUPUS ERYTHEMATOSUS: A NOVEL APPROACH TO REFRACTORY DISEASE

Abstract

TOPIC: Critical Care TYPE: Medical Student/Resident Case Reports INTRODUCTION: Neuropsychiatric Systemic Lupus Erythematosus (NPSLE) refers to involvement of the nervous system in patients with Systemic Lupus Erythematosus (SLE) either by inflammation or thrombosis. (1) Neuropsychiatric manifestations in SLE are prevalent with affecting up to 40 percent of patients. (2) We present a case of NPSLE presenting as Lupus cerebritis requiring pulse dose steroids and Immunosuppressive therapy. CASE PRESENTATION: A 40 year old female with a history of SLE presented with altered mental status. She had a dental procedure done 1 week ago. Her vitals on presentation were unremarkable. On physical examination she was an ill appearing female oriented to self with deep red confluent plaques with some crusting on her face. Initial work up showed leukopenia. Blood cultures were drawn. CT head was normal and a panorex showed periodontitis at the site of recent dental procedure. Workup was negative for any ischemic, metabolic, or toxic etiology for her confusion. A lumbar puncture was performed and the patient was started on empiric antibiotics. Spinal fluid analysis was unremarkable, including culture and viral encephalitis panel. ESR and CRP were elevated at 68 mm/hr and 1.4 mg/dl respectively. Autoimmune studies showed low C3 and C4 levels of 59 mg/dl and 3 mg/dl while dsDNA was 32 IU/ml. Diffusion Weighted images on MRI showed patchy cortical and subcortical lesions consistent with lupus cerebritis. EEG was negative for any seizure activity.The patient was diagnosed with Lupus Cerebritis (NPSLE) and was started on pulse dose steroids 1gm Methylprednisolone for 3 days and mycophenolate. There was significant improvement in her mental status and rash. Following her improvement steroids were decreased to 1mg/kg/day prednisone. Upon decreasing her steroids, she again developed worsening delirium. She was again started on a second course of pulse dose steroids with subsequent return to her baseline mental status. She was discharged on mycophenalate, hydroxychloroquine and tapering dose of prednisone starting at 1mg/kg with rheumatology follow up. DISCUSSION: NPSLE is a complication of SLE with substantial mortality. There is no gold standard test to diagnose it, other etiologies delirium must be ruled out. (3) NSPLE can be associated with cutaneous involvement. Generalized involvement of the central nervous system with inflammation leads to an acute state of confusion (Delirium). Treatment is with pulse dose steroids, methylprednisolone at 1 gm/day for 3 days followed by 1mg/kg/day. (3) In certain cases, additional immunosuppressive agents may be needed. Further, if there is incomplete response to pulse dose steroids, repeating or prolonging the course may be beneficial as was the case in our patient. CONCLUSIONS: In patients with NPSLE, physicians should consider prolonging the pulse dose steroids or repeating the course of pulse dose steroids if inadequate response to initial therapy. REFERENCE #1: The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes. Arthritis Rheum. 1999 Apr;42(4):599-608. doi: 10.1002/1529-0131(199904)42:4<599::AID-ANR2>3.0.CO;2-F. PMID: 10211873. REFERENCE #2: Hanly JG, Urowitz MB, Su L, et al. ; Systemic Lupus International Collaborating Clinics (SLICC). Prospective analysis of neuropsychiatric events in an international disease inception cohort of patients with systemic lupus erythematosus. Ann Rheum Dis. 2010 Mar;69(3):529-35. doi: 10.1136/ard.2008.106351. Epub 2009 Apr 8. PMID: 19359262; PMCID: PMC2929162. REFERENCE #3: Kivity, S., Agmon-Levin, N., Zandman-Goddard, G. et al. Neuropsychiatric lupus: a mosaic of clinical presentations. BMC Med 13, 43 (2015). https://doi.org/10.1186/s12916-015-0269-8 DISCLOSURES: No relevant relationships by Benjamin Goodman, source=Web Response No relevant relationships by Muhammad Ibrar Islam, source=Web Response