Catastrophic neuropsychiatric lupus erythematosus flare with multiple cns manifestations

Background: Systemic Lupus Erythematosus (SLE) is known to cause a wide array of neuropsychiatric (NPSLE) issues, impacting a substantial proportion of affected individuals (pooled prevalence ~30% in SLE populations). Although severe events such as myelitis (seen in ~0.5-2% of SLE cases) and intracranial hemorrhage (~0.4% incidence) are recognized NPSLE complications, the specific combination of concurrent subarachnoid hemorrhage (SAH), cranial subdural hemorrhage (SDH), spinal SDH, and myelitis occurring simultaneously is exceptionally uncommon. Case Presentation: This report details the case of a 30-year-old female, whose previously well-controlled SLE (Class II Lupus Nephritis) acutely worsened into a severe multi-system flare. Prodromal symptoms emerged roughly six weeks earlier, followed by an acute onset of severe headache, neck pain, and swiftly progressing quadriparesis approximately 18 days before she was admitted to our tertiary care facility in Kolkata, India. Upon admission (Day 0), her neurological examination revealed quadriparesis, affecting the lower limbs and left side more significantly. Imaging and investigations subsequently confirmed widespread SAH, bilateral cerebellar SDH, non-enhancing myelitis from C4-C6, and an anterior spinal SDH spanning C5-D3. Multifocal intracranial vasculitis was suggested by digital subtraction angiography. Laboratory findings indicated high SLE activity, including low complement levels (C3/C4) and positivity for specific antibodies (SS-A/Ro52/nRNP/Sm), although dsDNA antibody tests were negative at this time. An initial period of stabilization permitted transfer to a high-dependency unit (around Day 10). However, despite treatment with high-dose steroids, prior IVIG administration, and Rituximab infusion (Day 18), her clinical trajectory declined due to complications including severe sepsis, pancytopenia, possible myocarditis, and progressive multi-organ dysfunction. This necessitated readmission to the ICU (Day 25) for mechanical ventilation. Following discussions about the goals of care given her grave condition, she was discharged at the family's request on Day 28 for continued supportive care in her home country of Bhutan. Conclusions: This case underscores the potential for a devastating cluster of neurological emergencies in NPSLE, likely stemming from CNS vasculitis during a severe systemic SLE exacerbation. It highlights significant diagnostic hurdles, especially distinguishing the inflammatory flare from infection, and illustrates the complexities of managing critical illness alongside aggressive autoimmune disease.