SLE, APS, Raynauds

Question 1

What is the pathology of autoimmune connective tissue diseases? Which type of immune reaction is it?

Answer 1

body raises immune response to its own cells

Creates Antinuclear antibodies - IgG which forms complexes with the antigen (cells nucleus)

Deposition of these complexes into tissues attracts complements

Reaction with complements attracts neutrophils leading to inflammation

This inflammation is referred to as ‘immune complex mediated’

This inflammation leads to tissue damage

Type 3 hypersensitivity reaction (3 letters in SLE)

Question 2

What are the two components of SLE pathogenesis?

Answer 2

Inflammation leading to tissue damage

Thrombosis: phospholipid antibodies

Question 3

What is the disease course of SLE? What are leading causes of death?

Answer 3

relapsing - remitting

chronic inflammation means patients have reduced life expectancy cardiovascular disease and infection are leading causes of death

Question 4

What autoantibodies are important markers of SLE? Which can be used as a marker of disease activity?

Answer 4

1. Antinuclear Antibody - initial step (also present in autoimmune hepatitis)
2. Anti-double stranded DNA (anti-dsDNA) - specific to SLE
3. Anti-Smith (highly specific but not very sensitive)

Anti double stranded DNA titres can be used to monitor disease, not present in all patients however

Question 5

What markers are used to monitor disease in SLE?

Answer 5

1. ESR usually used. during active disease CRP may be normal, raised CRP may indicate underlying infection
2. C3, C4 - low during active disease (formation of complexes leads to consumption of complements)
3. anti-dsDNA titres - can be used for disease monitoring, not present in all patients

Question 6

Besides autoantibodies and markers to monitor disease activity, which other investigations can be done in SLE?

Answer 6

FBC - normocytic anaemia of chronic disease

Urine analysis and urine protein: creatinine ratio to assess for Lupus nephritis

Renal biopsy: Lupus nephritis

Question 7

Who does SLE tend to affect?

Answer 7

• females - Asian/ Afro-Caribbean
• onset 20-40 years

Question 8

general features of SLE ?

Answer 8

tiredness

fever

lymphadenopathy

mouth ulcer

Question 9

Skin features of SLE?

Answer 9

MALAR rash, sparing nasolabial folds

discoid rash - well demarcated rash in sun exposed areas

photosensitivity

Livedo-reticularis (mottled skin)

Raynaud’s phenomenon

non-scarring alopecia

Question 10

Cardiac SLE features

Answer 10

Pericarditis (most common)

Myocarditis

Question 11

Lung features of SLE

Answer 11

Pleurisy

Question 12

MSK features of SLE

Answer 12

arthralgia

myalgia

non-erosive arthritis

Question 13

Renal features of SLE

Answer 13

proteinurea

Glomerulonephritis (diffuse, proliferative glomerulonephritis most common)

Question 14

Neuropsychiatric

Answer 14

Anxiety and depression

Psychosis

Seizures

Question 15

How is SLE diagnosed?

Answer 15

SLICC and ACR criteria - ANA antibodies and a number of symptoms (at least 4)

Question 16

What are important complications of SLE?

Answer 16

1. CVS disease - chronic inflammation leading to htn CAD
2. infection - as part of disease and secondary to immunosuppressants
3. Anaemia of chronic disease, leucopenia, neutropenia, thrombocytopenia
4. Pericarditis, Pleuritis
5. Pulmonary fibroris (inflammation of lung tissue)
6. Lupus nephritis - can lead to end stage renal failure
7. Neuropsychiatric SLE - optic neuritis, transverse myelitis, psychosis
8. Recurrent miscarriage - IUGR, Pre-eclampsia, pre-term labour
9. Venous thromboembolism - anti-phospholipid syndrome

Question 17

What is the management of SLE? First line immunosuppressant for mild and another that may be used? How is SLE Renal disease managed?

Answer 17

NSAIDs Steroids (prednisolone)

Hydroxychloroquine (first line for mild SLE)

Suncream and sun avoidance for the photosensitive the malar rash

Other immunosuppressants - methotrexate, azathioprine etc -

manage htn - steroid - immunosuppressants - aza/cyclophosphamide

Question 18

Which autoantibodies can occur in 40% of patients with SLE? what is it associated with an increased risk of?

Answer 18

Anti-phospholipid antibodies

Associated with increased risk of venous thromboembolism

Question 19

What is the pathology of the Antiphospholipid syndrome?

Answer 19

autoantibodies which interfere with coagulation and lead to a hypercoaguable state

Question 20

What autoantibodies are associated with APS?

Answer 20

Lupus anticoagulant

Anticardiolipin antibodies

Anti-beta-2 glycoprotein I antibodies

Question 21

What is antiphospholipid syndrome associated with? 7

Answer 21

Venous thrombosis: DVT and PE

Arterial thrombosis: MI, Stroke, Renal thrombosis

Pregnancy complications: recurrent miscarriage, stillbirth, pre-eclampsia

Livedo reticularis

Libmann-Sacks Endocarditis - non bacterial vegetation on the valves (usually mitral) seen in SLE and APS Thrombocytopenia

Prolonged aPTT, low platelets

Question 22

How do you diagnosie APS

Answer 22

history of thrombosis persistent autoantibodies

Question 23

How do you manage APS?

Answer 23

Primary thromboprophylaxis - low dose aspirin

Secondary thromboprophylaxis - Warfarin INR target 2-3 (lifelong, 3-4 if recurrent)

Pregnant: LMWH + Aspirin

Question 24

What is raynaud’s ?

Answer 24

exaggerated vasoconstriction in digital arteries and cutaneous arterioles in response to cold or emotional stress

Question 25

What is the difference between Raynaud’s disease and Raynaud’s phenomenon?

Answer 25

Raynaud’s disease is primary

Raynaud’s phenomenom is secondary to another condition

Question 26

What are causes of Raynaud’s phenomenon?

Answer 26

Connective tissue disease: Scleroderma, Rhumatoid arthritis, SLE

Leukaemia

Use of Vibrating tools

Drugs (OCP, ergot)

Cervical rib

Question 27

How do you manage raynauds ?

Answer 27

all suspected cases should be referred to secondary care

1st line: CCB - nifedipine

2nd line: IV prostacyclin infusion - effect lasts weeks to months