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
Q

What is seen in active SLE

A

Low complement -> C3/4
High Anti-dsDNA

26
Q

Aim of SLE management

A

Remission/low disease activity
Prevention of flares

27
Q

Immunosuppressant side effects in SLE

A

Preference to immunosuppressants due to:
infection
osteoporosis
Avascular necrosis (AVN) affecting hips due to APL

28
Q

What is the primary lupus treatment?

A

Hydroxycholoroquine (DMARDs)

29
Q

What are other treatments that can be initiated in SLE?

A

Immunosuppressants: Methotrexate, Azathioprine,
B cell monoclonal AB: Rituximab

30
Q

Considerations of SLE and pregnancy

A

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
Q

What are the skin signs of Dermatomyositis?

A

Lilac heliotrope rash on eyelids or malar regions,
gottron’s papules (red raised lesions),