Lupus and autoimmune connective tissue disease Flashcards

1
Q

What are the 4 dif autoimmune connective tissue disorders

A

SLE
Sjorgen’s syndrome
Systemic sclerosis (scleroderma)
Autoimmune inflammatory muscle disease

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2
Q

What is overlap syndrome

A

Patients who exhibit characteristics of multiple autoimmune connective tissue disorders
- mainly children

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3
Q

What is SLE?

A

Systemic Lupus Erythematosus:
chronic tissue inflammation from autoantibodies
Involves both innate and adaptive immune systems

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4
Q

What type of antibodies are formed in SLE and what does this cause

A

Autoantibodies against nuclear components
Causes multi-site inflammation esp. jts, skin, kidney

As basically all cells have a nucelus

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5
Q

Where does SLE present?

A

Multi-site but mostly skin (rash), joints (arthritis), kidney

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6
Q

What are the key symptoms of connective tissue disorders?

A

Arthralgia, Arthritis, Raynaud’s Phenomenon

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7
Q

What is the difference between arthralgia and arthritis?

A

Arthralgia - joint pain no swelling/clinical signs
Arthritis - joint pain with swelling

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8
Q

What is Raynaud’s phenomenon?

A

Vasospasm of digits with cold exposure

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9
Q

What are the triphasic colour changes seen in Raynaud’s

A

WHITE - contraction of blood vessels .: lack of blood to digit
BLUE - cyanosis due to deoxygenation of venous blood
RED - when circulation opens up again and blood returns (painful)

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10
Q

Consequences of severe Raynaud’s

A

tissue ischaemia
ulcers
necrosis

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11
Q

What is the rash associated with SLE?

A

Photosensitive malar rash - erythema on cheeks and nose sparing nasolabial fold

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12
Q

What are the systemic clinical manifestations of SLE?

A

Malar rash (butterfly)
Mouth ulcers
Alopecia
Raynaud’s
Serositis
Arthralgia/Arthritis
Glomerulonephritis
Haemolytic anaemia
Cerebral disease
Lymphadenopathy
fever w/o infection

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13
Q

What autoantibodies are present in SLE?

A

Anti-nuclear antibodies (ANA) e.g. (Anti-Ro), (Anti-La)
Anti-double stranded DNA antibodies (Anti-ds-DNA)
Anti-Phospholipid antibodies (APL)

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14
Q

Significance of anti-nuclear antibodies in SLE

A

Found in all patients w/ SLE
Non-specific for SLE

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15
Q

What are anti-phospholipid antibodies associated with

A

Thrombosis
- Arterial e.g. stroke
- Venous e.g. DVT, PE
Pregnancy loss, miscarriage

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16
Q

Significance of Anti-dsDNA in SLE

A

Specific for SLE
Serum levels correlate with disease activity

17
Q

Significance of Anti-Sm in SLE

A

Specific for SLE
No correlation with serum levels

18
Q

What are the key autoantibodies in rheumatoid arthritis?

A

Rheumatoid Factor,
Anti-cyclic citrullinated peptide antibody (Anti-CCP)

19
Q

SLE immunopathogenesis in innate and adaptive immunity

A

Innate immunity - Overactive Type 1 interferon pathway,
- Complement pathway abnormalities
Adaptive immunity - Autoreactive B/T cells

20
Q

What is the theorised pathogenesis of SLE?

A

Apoptosis translocates nuclear antigens to cell surface.
In SLE, impaired clearance of apoptotic cells leads to prolonged presentation of antigens to immune cells - B cell autoimmunity + complement activation, leading to tissue damage

21
Q

What will you see in SLE after blood investigating inflammation?

A

High ESR, Normal CRP (remember this! in rheumatoid this is high)
unless severe arthritis/serositis

22
Q

What are the haematological manifestations of SLE?

A

Haemolytic anaemia, Lymphopenia, Thrombocytopenia

23
Q

What immunological investigations can you do for SLE?

A

Antinuclear antibodies, Anti ds DNA antibodies, Low complement (C3 and C4)

24
Q

What renal tests do you do in SLE?

A

Urine Albumin:Creatinine Ratio (for glomerulonephritis in SLE)
Urinalysis
Creatinine via U&E
Kidney biopsy w/ persistent proteinuria

25
What is seen in active SLE
Low complement -> C3/4 High Anti-dsDNA
26
Aim of SLE management
Remission/low disease activity Prevention of flares
27
Immunosuppressant side effects in SLE
Preference to immunosuppressants due to: infection osteoporosis Avascular necrosis (AVN) affecting hips due to APL
28
What is the primary lupus treatment?
Hydroxycholoroquine (DMARDs)
29
What are other treatments that can be initiated in SLE?
Immunosuppressants: Methotrexate, Azathioprine, B cell monoclonal AB: Rituximab
30
Considerations of SLE and pregnancy
Antiphospholipid AB associated w/ miscarriage (red. risk with aspirin/heparin) pregnancy worsens renal function Anti-Ro can cause fetal heartblock Some drugs cause fetal malformation e.g. warfarin/methotrexate
31
What are the skin signs of Dermatomyositis?
Lilac heliotrope rash on eyelids or malar regions, gottron's papules (red raised lesions),