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Florian Stierlin

on 21 April 2011

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Transcript of Neurolupus

Antiphospholipid syndrome


Description of the patient
How did we do?


CONCLUSION INTRODUCTION Systemic Lupus Erythematosus A multisystem autoimmune disease characterized by a vast array of auto-antibodies

Epidemiology = 6-150 / 100 000 persons, dependent on sex and ethnic origin

Etiology = Unknown cause

Pathogenesis = (Hypothesis-stage) Genetic predisposition leading to failure of self tolerance and continue formation of auto-antibodies

Diagnosis = SOAP BRAIN MD Neurolupus - Neurolupus = SLE with neuropsychiatric involvements

- EPIDEMIOLOGY = Up to 50% of patients with SLE


Central nervous system involvement (Aseptic meningitis, Headaches, Chorea, Anxiety and Mood disorder, Psychosis, Seizure disorder, Acute confusional state, Cognitive dysfunction)
Peripheral nervous system involvement (Myasthenia gravis...)

Prove SLE
Confirmation of the presence of some neurological manifestations

- Several investigations are recommended for the diagnosis
Blood tests and serology, CSF examination, Brain imaging...
But no "gold-standard" test Antiphospholipid Syndrome A systemic disorder characterized by:
Anti-phospholipids antibodies (aPL)
Arterial and venous thrombosis
Adverse outcomes in pregnancy (for mother and fetus)

40% of SLE patient are positive for aPL:
Higher risk of thrombosis
Higher risk of miscarriages
Higher risk of valve pathology ANTIPHOSPHOLIPID ANTIBODIES:

- Heterogeneous group of autoantibodies directed at plasma protein that bind to phospholipids

- Contradictory effect of some aPL
In vitro = anti-coagulant
In vivo = pro-coagulant

- aPL found in 1-5% of healthy people (risk = unknown) OBJECTIVES Lot of investigations to diagnose neurolupus
One of these = MRI imaging

Some hyperintensities are seen MATERIAL

- 57 years old woman (1998)

- SLE diagnosed at the age of 23
dsDNA antibodies positive
Lupus nephritis and skin involvement

- In 1983 (42 years), neuropsychiatric involvements started
Neurolupus diagnosed

- aPL syndrome diagnosed in 1990

- From 1983 to 1998 :
Progressive cerebral infarctions causing various symptoms like cognitive impairment, visual loss, balance problems, headaches and finally an acute confusional state and coma
Also multi-organ failure with myocardial infarction and pulmonary embolus
The cause of her death = not only cerebral infarctions

- She was under under immunosuppressive therapy for SLE and Antiphospholipid syndrome

- On MRI a lot of white matter hyperintensities are seen 7-Tesla MRI Paraffin block How? What do they represent pathologycally?
Can they help to diagnose neurolupus? Results Discussion and conclusion Brain into fomblin Immunochemistry (HE, C4d staining) Light microscopy Hematoxylin-Eosin:
Microinfarcts C4d Staining:
No staining in vessels
Some little round staining in tissue MRI Hyperintensities:
Present in a lot of different cases Majority of case = not relevant (no correlation with lesions established) In neurolupus patient:
Possible correlation between MRI hyperintensities and infarct zones - Need for more investigations

- If a correlation is found it can be useful for doctors and patients:
E.g.: Should a neurolupus patient with MRI hyperintensities be treated with anticoagulant drugs? C4d Staining: Autoantibodies that migrates from vessels to tissue?
Activation of complement in infarction zone in order to clean it? Complement cascade activated:
Regulatory mechanisms stop it before formation of C4d?
Zone of microinfarction is too evolved (e.g. scarring) and complement is no more present?

No complement:
Microinfarcts due to microthrombi formed in the heart (e.g. cardiac valve pathology in aPL syndrome)? C4d tissue staining No C4d in vessels Microinfarcts:
Swollen vessels lead to obstruction of the lumen
Damages to endothelium lead to endothelial activation and thrombosis Vasculopathy:
Auto-antibodies bind to endothelium and activate complement system leading to recruitment of inflammatory cells and damage to endothelium
IC deposition in vessels Main lesions Thank you!
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