From the Guidelines
Relationship Between Lupus and Encephalopathy
Lupus, or Systemic Lupus Erythematosus (SLE), is strongly associated with an increased risk of various neuropsychiatric manifestations, including encephalopathy.
- The relationship between lupus and encephalopathy is complex, with encephalopathy being one of the neuropsychiatric manifestations that can occur in SLE patients, although it is considered relatively uncommon with a cumulative incidence of less than 5% 1.
- Risk factors for neuropsychiatric manifestations in SLE include previous or concurrent severe neuropsychiatric SLE, antiphospholipid antibodies, and generalized SLE activity or damage 1.
- The diagnostic work-up for suspected neuropsychiatric SLE, including encephalopathy, should be similar to that in non-SLE patients presenting with the same manifestations and aims to exclude causes unrelated to SLE 1.
- Glucocorticoids and immunosuppressive therapy are indicated for neuropsychiatric manifestations felt to reflect an immune/inflammatory process, such as encephalopathy, following exclusions of non-SLE-related causes 1.
- Antiplatelet/anticoagulation therapy may be considered when manifestations are related to antiphospholipid antibodies, particularly in thrombotic cerebrovascular disease 1.
- Management of SLE-associated encephalopathy may also involve symptomatic therapies and the treatment of aggravating factors, such as infection, hypertension, and metabolic abnormalities 1.
From the Research
Relationship between Lupus and Encephalopathy
The relationship between lupus (Systemic Lupus Erythematosus) and encephalopathy is complex and has been studied in various research papers.
- Encephalopathy has been associated with SLE, particularly in cases of limbic encephalitis, which has been rarely reported in SLE patients 2.
- Posterior Reversible Encephalopathy Syndrome (PRES) is a neuropsychiatric manifestation of SLE, characterized by seizures, altered mental status, headache, and visual disturbances 3.
- PRES has been identified as a distinct neuropsychiatric syndrome associated with SLE, with a relatively high frequency and distinct clinical course 3.
Risk Factors for Encephalopathy in Lupus
Several risk factors have been identified for encephalopathy in lupus patients, including:
- Lupus nephritis with renal dysfunction 4, 5, 6
- Hypertension 4, 5, 6
- High-dose steroid and cyclophosphamide treatment 4
- Recent transfusion 4
- Younger age at onset 5
- History of eclampsia 5
- White blood cells >9 × 10^9/l 5
- Heart failure 5
Clinical Features and Outcomes
The clinical features of encephalopathy in lupus patients include:
- Seizures 4, 5, 6
- Headache 4, 5, 6
- Confusion 4, 5, 6
- Visual disturbances 3
- Memory deficits, bizarre behavior, psychosis, neuromyotonia, and movement disorders have been described in autoimmune central nervous system disorders and associated with specific antibodies 2 The outcomes of encephalopathy in lupus patients are generally good with early supportive treatment, with a mean hospital stay of 11.2 days and no neurological deficits 6. However, SLE patients with PRES have been found to have a higher mortality rate compared to controls 5.