Systemic Lupus Erythematosus (SLE)

Question 1

What is it

Answer 1

An inflammatory, multi-systemic autoimmune disorder
Autoimmune Rheumatic disease
(that usually affects females of childbearing age)

Question 2

Signs and symptoms

Answer 2

Fatigue
Myalgia
Rashes - malar (butterfly) rash, discoid rash
Raynaud's phenomenon
Arthritis
CNS disorders - Epilepsy, Headache
Haematological disorders - hameolytic anaemia
Immunological disorders
Nephritis
Oral ulcers
Photosensitivity
Pericarditis
Pleuritis

Question 3

Complications

Answer 3

Increased risk of:
-atherosclerosis
-stroke
-myocardial infarction
-other autoimmune conditions
Depression
Risk of lupus nephritis

Question 4

Investiagations

Answer 4

Bloods:
Antinuclear antibody (ANA)
Anti-Smith antibodies and antidouble-stranded DNA
FBC, U and Es, LFTs, TFTs, glucose
Increase ESR, normal CRP

Biopsies of kidney and skin - deposition of IgG and complement
GFR - assess renal function
Pulmonary function tests

Question 5

**Criteria for diagnosis of SLE:

Answer 5

At least 4 out of 11 is diagnostic:
I AM PORN HSD:
Immunological disorder

ANA positive
Malar rash

Photosensitivity
Orla ulcers
Renal disorder
Nonerosive arthritis/Neurological disorder

Haematological disorder
Serositis
Discoid rash

Question 6

Conservative treatment

Answer 6

Patient education
Lifestyle - Advise patient about sun protection/avoid sunlight* and encourage smoking cessation
Assess psychological impact of disease

Question 7

Medical treatment

Answer 7

Symptomatic treatment:
NSAIDs (for arthralgia, arthritis, serositis)
Reduce CV risk
Antidepressants (high rate mental health issues)
Bisphosphonates (high rate osteoporosis)

Immunosuppression:
Steroid therapy
Immunosuppressive therapy e.g. azathioprine, cyclophosphamide

Monoclonal antibodies e.g. rituximab

Question 8

Epidemiology

Answer 8

Young 20-40 years old (younger as autoimmune process)

Women 9xs more common

Question 9

Aetiology

Answer 9

Combination of genetic and environmental

Potential triggers: Drugs, UV light

Question 10

Example of drug that can cause SLE

Answer 10

Isoniazid

Question 11

Pathophysiology

Answer 11

Type 3 hypersensitivity (possibly also type 2)

Leads to deposition of immune complexes in tissues all over body, causing inflammation

Question 12

What does each hypersensitivity type refer to?

Answer 12

1 – allergy, IgE mediated, asthma, eczema, hayffever
2 – cytotoxic, antibody dependent – autoantibodies bind to antigens on the surface of cells and cause cellular destruction. EG Goodpastures
3 – Immune complexes – autoantibodies bnd to soluble antigens in the blood and cause them to become insoluble. Leads to deposition of many insoluble immune complexes in body tissues
4 – Delayed type hypersensitivity – T cell mediated - formation of granulomas in TB

Question 13

7 symptoms

Answer 13

Lethargy
Joint pain
Muscle pain
Photosensitivity
Chest pain (serositis)
SOB
‘Gritty’ eyes (related to sjogrens)

Question 14

6 signs

Answer 14

Pale mucous membranes (anaemia)
Oral ulcers
Murmur
Malar rash
Discoid rash
Ataxia

Question 15

Describe the blood tests expected in SLE

Answer 15

FBC - anaemia of chronic disease or autoimmune haemolytic
ESR - high
U and Es - urea and creatinine raised in advanced renal disease
Autoantibodies - ANA, anti-dsDNA, antiSm, Antiphospholipid antibodies (APLS highly associated with SLE)

Question 16

What is literal translation of Systemic Lupus Erythematosus

Answer 16

Systemic = affects multiple organs
Lupus = diseases affecting skin
Erythematosus = reddening of skin