A severe case of systemic lupus erythematosus with cerebral involvement

M Wiedmann1, W Seidel, L Mende

  • 1Department of Internal Medicine II (Gastroenterology), Center of Internal Medicine, University of Leipzig, Leipzig, Germany. wiedm@medizin.uni-leipzig.de

Insights

This case study details a severe systemic lupus erythematosus case in an 18-year-old female, complicated by bleeding and neurological issues. The patient experienced a challenging recovery despite intensive treatment for lupus and its complications.

Area of Science:

  • Neurology
  • Rheumatology
  • Neurosurgery

Background:

  • Systemic lupus erythematosus (SLE) can present with severe multi-organ involvement.
  • Neurological complications, including seizures and intracranial hemorrhage, are serious manifestations of SLE.

Observation:

  • An 18-year-old female with severe SLE developed intracerebral bleeding requiring neurosurgical intervention.
  • Postoperative complications included rebleeding, disseminated intravascular coagulation, platelet dysfunction, and hydrocephalus requiring a ventriculo-peritoneal (VP) shunt.
  • Catastrophic antiphospholipid syndrome was suspected but unconfirmed.

Findings:

  • Treatment involved plasmapheresis, cyclophosphamide, corticosteroids, and VP shunt placement.
  • The patient had a slow, incomplete recovery with persistent cachexia and amaurosis.
  • A complication of an intraperitoneal VP shunt cyst necessitated surgical revision.

Implications:

  • This case highlights the complex management challenges in severe SLE with neurological and bleeding complications.
  • Aggressive immunosuppressive therapy and neurosurgical interventions may be required.
  • Long-term sequelae and management of complications like VP shunt issues are critical considerations.

Related Concept Videos

Encephalitis l: Introduction01:19

Encephalitis l: Introduction

Encephalitis is inflammation of the brain parenchyma, most often due to infections or autoimmune processes. It presents with neuropsychiatric features such as fever, altered mental status, behavioral changes, cognitive dysfunction, seizures, focal deficits, and sometimes autonomic instability. In some cases, the meninges are also involved, resulting in meningoencephalitis.Infectious CausesInfectious encephalitis is most commonly viral but can also result from bacterial, fungal, or parasitic...
Multiple Sclerosis l: Introduction01:19

Multiple Sclerosis l: Introduction

Multiple sclerosis is a chronic autoimmune disease of the central nervous system (CNS) that affects the brain, spinal cord, and optic nerves. It is an inflammatory demyelinating disorder and a leading cause of neurological disability in young adults.EpidemiologyMS commonly begins between 20 and 40 years of age and is twice as common in women. Its exact cause remains unclear, but genetic susceptibility contributes, with higher risk in first-degree relatives and identical twins. A greater...
Brain Abscess l: Introduction01:26

Brain Abscess l: Introduction

A brain abscess is a focal, intracerebral infection characterized by a localized collection of pus within the brain parenchyma, resulting from microbial invasion and the body’s inflammatory response. It progresses through stages: early and late cerebritis, followed by early and late capsule formation, reflecting tissue destruction, immune response, and eventual encapsulation.Etiology and PathogenesisCausative organisms vary with source and host factors, often involving polymicrobial infections,...
Encephalitis ll: Pathophysiology01:26

Encephalitis ll: Pathophysiology

Encephalitis is inflammation of the brain parenchyma caused by direct viral invasion or immune-mediated mechanisms triggered by infections or tumors. Both processes lead to neuronal injury, disrupted neurotransmission, and diverse neurological symptoms, often with overlapping clinical and pathological features.Autoimmune EncephalitisIn autoimmune encephalitis, antibodies target neuronal antigens on cell surfaces, synapses, or within neurons. A key example is anti-NMDAR encephalitis, which can...
Cryptococcal Meningitis01:27

Cryptococcal Meningitis

Cryptococcal meningitis is a life-threatening opportunistic infection predominantly associated with HIV/AIDS, accounting for over 100,000 deaths annually worldwide. However, it also affects individuals with other forms of immunosuppression, including those undergoing immunosuppressive therapy, organ transplant recipients, patients with innate immunodeficiencies, and individuals with hematological disorders. The infection is caused mainly by Cryptococcus neoformans and Cryptococcus gattii,...
Cerebral Edema ll: Pathophysiology01:22

Cerebral Edema ll: Pathophysiology

Vasogenic edema is a major form of cerebral edema characterized by abnormal accumulation of fluid in the brain’s extracellular space due to disruption of the blood–brain barrier (BBB). The BBB is a specialized structure composed of endothelial cells connected by tight junctions, supported by astrocytic endfeet and a basement membrane. Under normal conditions, it tightly regulates the movement of ions, proteins, and solutes between the bloodstream and brain parenchyma. When this barrier loses...