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Y2 MSK > Lupus and related autoimmune connective tissue diseases > Flashcards

Lupus and related autoimmune connective tissue diseases Flashcards

1
Q

what is the term given to arthritis associated with gastrointestinal inflammation?

A

enteropathic synovitis)

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2
Q

what is systemic lupus erythematosus?

A

Chronic tissue inflammation in the presence of antibodies directed against self antigens

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3
Q

what does systemic lupus erythematosus cause?

A

Multi-site inflammation but particularly the joints, skin and kidney

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4
Q

what antibodies are associated with systemic lupus erythematosus?

A

antinuclear antibodies
anti-double stranded DNA antibodies
anti-phospholipid antibodies

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5
Q

arthralgia and arthritis is typically_____

A

non-erosive

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6
Q

what is Raynauds phenomenon?

A

Intermittent vasospasm of digits on exposure to cold
Typical colour changes – white to blue to red

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7
Q

what causes the colour change in the digits seen in Raynauds phenomenon?

A

Vasospasm leads to blanching of digit
Cyanosis as static venous blood deoxygenates
Reactive hyperaemia

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8
Q

is Raynauds phenomenon a benign or metastatic condition?

A

benign, most commonly isolated

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9
Q

what demographic has the most common diagnosis of SLE?

A

females aged 15-45

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10
Q

what are the clinical manifestations of SLE?

A

Malar rash – erythema that spares the nasolabial fold
Photosensitive rash
Mouth ulcers
Hair loss
Raynaud’s phenomenon
Arthralgia and sometimes arthritis
Serositis (pericarditis, pleuritis, less commonly peritonitis)
Renal disease – glomerulonephritis (‘lupus nephritis’)
Cerebral disease – ‘cerebral lupus’ e.g. psychosis

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11
Q

outline the pathogenesis of SLE?

A

incompletely understood:
1. apoptosis leads to translocation of nuclear antigens to membrane surface
2. impaired clearance of apoptotic cells results in enhanced presentation of nuclear antigens to immune cells.
3. b cell autoimmunity
4. tissue damage by antibody effector mechanisms e.g complement activation and Fc receptor engagement

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12
Q

are the anti-phospholipid antibodies associated with SLE only found in SLE?

A

may also occur in absence of SLE in what is termed the ‘primary anti-phospholipid antibody syndrome

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13
Q

what is the other term given to anti-phospholipid antibodies in SLE and what are they associated with?

A

also termed anti-cardiolipin antibodies and associated with risk of arterial and venous thrombosis in SLE

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14
Q

what are the key auto-antibodies associated with systemic vasculitis?

A

Anti-neutrophil cytoplasmic antibodies (ANCA)

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15
Q

If ANA is positive the clinical laboratory will perform further tests to determine which type of ANA it is – typically these include screening for:

A

Anti-Ro
Anti-La
Anti-centromere
Anti-Sm
Anti-RNP
Anti-ds-DNA antibodies
Anti-Scl-70

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16
Q

Cytoplasmic antinuclear antibodies include:

A

Anti-tRNA synthetase antibodies
Anti-ribosomal P antibodies

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17
Q

which autoantibody in SLE correlates with disease activity?

A

Anti-double stranded DNA antibodies (anti-dsDNA)

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18
Q

what autoantibody is associated with secondary Sjogrens syndrome?

A

Anti-Ro antibodies
antigen is ribonucleoprotein

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19
Q

which autoantibody is associated with neonatal lupus syndrome?

A

Anti-La antibodies
antigen is ribonucleoprotein

20
Q

what are the symptoms of neonatal lupus syndrome?

A

transient rash in neonate, permanent heart block)

21
Q

which autoantibodies are associated with cerebral lupus?

A

Anti-ribosomal P antibodies

22
Q

what is seen in the haematology of a patient with SLE?

A

inc. ESR but CRP normal unless infection or serositis/arthritis
Haemolytic anaemia, Lymphopenia, Thrombocytopenia

23
Q

what renal markers indicate SLE?

A

very important to measure urine protein (most commonly urine protein:creatinine ratio [uPCR])
look at albumin

24
Q

what happens to C3 and C4 levels in SLE?

A

decrease

25
Q

what is the treatment aim for SLE?

A

aims at remission or low disease activity and prevention of flares

26
Q

what medication is given for the treatment of SLE?

A

Hydroxychloroquine is recommended in all patients with lupus
Maintenance treatment glucocorticoids should be minimised and, when possible, withdrawn.
Appropriate initiation of immunomodulatory agents (methotrexate, azathioprine, mycophenolate) can expedite the tapering/discontinuation of glucocorticoids
In persistently active or severe disease we use cyclophosphamide and B cell targeted therapies (rituximab and belimumab)

27
Q

what is SJÖGREN’S SYNDROME

A

autoimmune exocrinopathy -lymphocytic infiltration of especially exocrine glands and sometimes of other organs (extra-glandular involvement)

28
Q

what does exocrine gland pathology in sjogrens result in?

A

Dry eyes (xerophthalmia)
Dry mouth (xerostomia)
Parotid gland enlargement

29
Q

what are the most common extra-glandular manifestations of sjogrens?

A

non-erosive arthritis and Raynaud’s phenomenon

30
Q

what is secondary sjogrens?

A

Termed ‘secondary’ Sjögren’s syndrome if occurs in context of another connective tissue disorder e.g. SLE

31
Q

in sjogrens, what will a salivary gland biopsy show?

A

Salivary gland biopsy will show lymphocytic infiltration predominantly CD4 helper T cells and to lesser extent B lymphocytes

32
Q

what is the schirmers test

A

– a test to assess tear production. Filter paper is placed under lower eyelid and extent of wetness measured after 5 minutes. Abnormal is <5mm after 5 minutes

33
Q

what is autoimmune inflammatory muscle disease?

A

Proximal muscle weakness due to autoimmune-mediated inflammation either with (dermatomyositis) or without (polymyositis) a rash

34
Q

what skin changes are seen in dermatomyositis?

A

Lilac-coloured (heliotrope) rash on eyelids, malar region and naso-labial folds
Red or purple flat or raised lesions on knuckles (Gottron’s papules)
Subcutaneous calcinosis
Mechanic’s hands (fissuring and cracking of skin over finger pads)

35
Q

how is autoimmune inflammatory muscle disease detected?

A

Elevated CPK, abnormal electromyography, abnormal muscle biopsy

36
Q

what are the key features of systemic sclerosis?

A

Thickened skin with Raynaud’s phenomenon
Dermal fibrosis, cutaneous calcinosis and telangiectasia
Skin changes may be limited or diffuse

37
Q

what are the features of diffuse systemic sclerosis?

A

Fibrotic skin proximal to elbows or knees (excluding face and neck)
Anti-topoisomerase-1 (anti-Scl-70) antibodies
Pulmonary fibrosis, renal (thrombotic microangiopathy) involvement
Short history of Raynaud’s phenomenon

38
Q

what are the feature of limited systemic sclerosis?

A

Fibrotic skin hands, forearms, feet, neck and face
Anti-centromere antibodies
Pulmonary hypertension
Long history of Raynaud’s phenomenon

39
Q

what is overlap syndrome?

A

When features of more than 1 connective tissue disorder are present e.g. SLE and inflammatory muscle disease we can use the term overlap syndrome

40
Q

what does the term undifferentiated connective tissue disease describe?

A

When incomplete features of a connective tissue disease are present we can use the term undifferentiated connective tissue disease

41
Q

what is mixed connective tissue disease??

A

In one instance a group of patients with features of seen in SLE, scleroderma, rheumatoid arthritis, and polymyositis were identified by the presence of an autoantibody:
Anti-U1-RNP antibody*

42
Q

what are the key autoantibodies associated with diffuse systemic sclerosis?

A

Anti-Scl-70 antibody
also termed antibodies to topoisomerase-1

43
Q

what are the key autoantibodies associated with limited systemic sclerosis?

A

Anti-centromere antibodies

44
Q

what are the key auto-antibodies associated with dermato/polymyositis?

A

Anti-tRNA transferase antibodies

45
Q

what are the key autoantibodies associated with sjogrens syndrome?

A

No unique antibodies but typically see
Antinuclear antibodies - Anti-Ro and anti-La antibodies
Rheumatoid factor

46
Q

what are the key autoantibodies associated with mixed connective tissue disease?

A

Anti-U1-RNP antibodies

Y2 MSK (5 decks)

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