1. Combination of RNA and proteins 2. Associated with photosensitivity 3. Factor of 50

CNS Lupus Flashcards by J Abow

What is history of Lupus divided into?

Classical
Neoclassical
Modern

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What is Lupus described as?

Erosive facial lesions that were reminiscent of a wolf’s bite

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What is the shape of Lupus?

Discoid lupus

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What is Systemic Lupus Erythematosus?

A clinical syndrome, cause known, characterised by inflammation and multisystem involvement

Mainly a disease of women aged 15-50 (reproductive ears)

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What is systemic Lupus Erythematosus a member of and give examples?

A member of the family of autoimmune rheumatic diseases:

Rheumatoid’s syndrome
Sherbone’s syndrome
Polymyositis
They coexist

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What do 5% of Lupus patients have?

Erosive kind of arthritis

called Rufus

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What do 15% of Lupus patients have?

Sherborne syndrome which cause profound dryness of the eyes and dryness of the mouth

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What do 4% of Lupus patients have?

Muscle disease or myelitis

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Where is Systemic Lupus Erythematosus common among?

Black (and probably Chinese) women than Caucasian women

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What is matched by serological diversity?

Clinical diversity of Systemic Lupus Erythematosus

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Why should SLE patients seen regularly?

This disease can affect any organ or system

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What is the active and inactive disease of Lupus disease?

Active disease: see every 1-3 months

2. Inactive disease: see 6-12 months

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What is an example of drug that should be monitored carefully and what is it retreated by?

Hydroxychloroquine [treatment of malaria]

Retreated with non-steroidal, anti-inflammatory drugs

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What does the drug Hydroxtchloroquine have?

Profound effect intracellularly

Affects the cellular PH

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What is given to every Lupus patients?

Hydroxychloroquine

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What is the consequence of taking too much of Hydroxychloroquine?

Take eye test as it causes balls-eye appearance at the back of ey e

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What is given to treat more aggressive forms of Lupus?

Immunosuppressive drug

Steroids

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How should Immunosuppressive drugs be monitored closely?

Check their blood count

2. Their kidney and liver test

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What is the number one test to do for the diagnosis of Lupus?

Anti-nuclear, antibody test (98%)

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What does the nucleus house?

Nearly 2-3000 antigens

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What is Ro/Lo?

Combination of RNA and proteins
Associated with photosensitivity
Factor of 50

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What can the antibodies of Ro/L0 do?

Cross the placenta and bind to the conducting tissue of heart
Heart beats in a disordered function [ heart block]
Heart block treated with a pacemaker

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What are the 2 problems that Antiphospholipids are associated with?

Clotting - Deep veins of the legs

2. Increasing Miscarriage

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What is Increase in dsDNA associated with?

Renal disease

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What are the co-morbidities for Lupus?

1. Atherosclerosis 2. Osteoporosis/ avascular necrosis 3. Infection 4. Cancer

What is strongly linked to the process of steroids?

Osteoporosis/avascular necrosis

What is Infection linked to?

Steroids and immunosuppressive drugs

What is decreased in Lupus patients?

Breast cancer Endometrial cancer Ovarian cancer

What is found in a 1/4 of the Lupus patients?

Hair loss

What are some examples of clinical characteristic in Lupus Clinic?

1. Rash - 61% 2. Photosensitivity - 35% 3. Alopecia - 23% 4. Oral ulcers - 26% 5. Arthritis - 92% 6. Serositis - 39% 7. Renal - 30% 8. CNS - 20%

In SLE, what are the common . Haematological abnormalities?

1. Haemolytic anaemia [Hb <8g/dl) 2. Leucopenia 3. Lymphopenia 4. Thrombocytopenia

What do a small significant of patients have?

Very low haemoglobin, it should be above 11 | White cell reductions are quite common

What are the key measures of Lupus?

Antibodies to DNA and C3

What happens if the patients are flaring?

1. DNA goes up 2. C3 goes down [complement are deposited in the tissue] 1. If the DNA are flat-lining and the C3 goes down

What is the normal amount for DNA antibodies?

50 units

What are more common in children?

Alopecia oral ulcers Thrombocytopaenia Haemolytic anemia

What are the main cause of death?

1. Infection 2. Vascular 3. Cancer 4. Active disease including renal

What was neurological involvement in SLE first mentioned by?

Kaposi in 1875

In 1904, what did Baum relate?

Active delirium, aphasia and hemiparesis to 'probable DLE'

What did Daly in 1945 correlate?

Clinical symptoms with abnormal CSF findings and with the presence of vasculitis

What did Dubois describe ?

Clinical neurological subset in 1953 among 62 patients

What did Lewis focus on?

First to focus on the importantce of EEG findings and psychometric testing in 1954

What are the two major complications of Lupus?

1. Psychosis | 2. Depression

Why is Depression in Lupus challenging to determine?

1. Is it due to reactive depression to the circumstance? 2. Is it due to fundamental problem within the brain? 3. Vasculitis 4. Thrombosis

What does vasculitis respond to?

Steroids and immunosuppressive drugs

When is warfarin required?

If it is clots

What is associated with an increased risk of clotting?

Phospholipid antibodies

What has a well established link with thrombosis and thromboemboli?

Anti-phospholipid antibodies

What do anti-P antibodies bind to?

Cell surface membrane protein on a neuroblastoma cell line

What do Intraventricular injection of IgG anti-P cause?

EEG and behavioural changes in rats | Bonfa et al

Where is true vasculitis present in?

Only ~10% of CNS lupus

What might the choroid plexus act as?

Non-specific trap for circulating immune complexes e.g. DNA/anti-DNA complexes

What are neurone reactive antibodies ?

Lymphoctyotoxic antibodies (LCA) cross reactive with neuronal antigens Directly targeted to neuronal antigens

What does evidence suggest about LCA?

Have a specific role in the pathogenesis of cerebral Lupus, particularly cognitive dysfunction and visuospatial deficit

What are potential antigenic targets in CNS-Lupus?

1. Phospholipids +/- B2 glycoprotein 1 2. Glycolipid 3. Neurofilaments 4. Ribosomal P 5. 50-52 kDa (lymphatic, synaptic) 6. 97 kDa (neuronal) 7. GMP-140 (Endothelial) 8. Limbic-system specigic antigen

What are the major target for anti-ribosomal protein antibodies?

Ribosomal P proteins - P0 - P1 - P2

Where are the ribosomal P proteins located?

On the larger 60 unit of eukaryotic ribsomes | At least 1 epitope is common to all 3 proteins

What does the majority of patients with anti-ribsomal P antibodies do not have?

Psychosis

What do a subset of anti-DNA antibodies react with?

A sequence within the glutamate/NMDA receptor subunit NR2

What do X reactive antibodies recognise?

A common pentapaptide | Asp.Glu-Trp-Asp/Glu-Tyr Ser Se/Gly

What are common in SLE?

Clinical anxiety | Depression

What did depression show some link to?

Anti-Sm antibodies

What is anxiety seem to b associated mainly with?

Appearance problems Reduction in social contacts and interactions Concern that mental/cognitive abilities are affected

What are depression linked to?

Physical disability and anxiety

What are common Neuropsychiatric events?

1. Headache 2. Anxiety 3. Mild depression 4. Mild cognitive impairment 5. Polyneuropathy lacking confirmation

What is Neuropsychiatriac events associated with?

Negative impact on HRQoL and increased mortality

What are cytokines implicated in pathogenesis of NP-SLE?

1. Interferon alpha 2. IL-6 3. IL-8 4. APRIL 5. BAFF 6. CCL2 7. RANTES

What are the sources of intracranial cytokine production?

1. Immune effector cell 2. Glial 3. Neuronal cells

What is the stimulus for enhanced cytokine production?

Autoantibody mediated cell activation

What are the treatment for CNS Lupus?

1. Symptomatic e.g. anti-convulsants where epilepsy is a problem 2. Immunosuppression 3. No biologic drugs are yet approved

Where is Lupus features more diverse?

CNS system 12 central 7 peripheral

What is the immunopathology of CNS Lupus?

Equally diverse

What does not help much in CNS disease?

The standard serological test for Lupus activity

What are the investigations in SLE patients with NP events?

1. Autoantibodies 2. CSF 3. Electophysiological assessment 4. Electropsychological assessment 5. Neuroimaging

What are the management of NPSLE principles?

1. Establish diagnosis of SLE 2. Decide attribution of NP event 3. Treat non-SLE factors 4. Symptomatic therapy 5. Focal NP-SLE 6. Diffuse NP-SLE

What are the potential outcome measures for CNS Lupus?

1. Biomarkers - Tissue damage - BBB permeability 2. Neuroimaging - Brain structure - Brain function 3. Clinical outcome - Individual NP events - Multiple NP events - Brain function

Key points - Treatment of CNS Lupus

``` Use immunosuppression (often steroids and azathioprine/iv cyclophosphamide/mycophenolate). Use an appropriate drug e.g. an antipsychotic for a patient with delusions/psychosis or an anti convulsant for those with seizures. Be patient! ```

CNS Lupus Flashcards

(77 cards)