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MSK > multi system autoimmune conditions > Flashcards

multi system autoimmune conditions Flashcards

1
Q

Who is lupus more common in

A

Females 9:1

Afro-carribean>asian>caucasion

People with a family history of Lupus

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2
Q

What is the common age that lupus occurs

A

15-50 years old

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3
Q

What are the risk factors for Lupus

A

Genetic factors - family history

Hormonal factors

Environmental factors e.g UV, drugs and infections

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4
Q

What happens in lupus

A

There is an immune response against endogenous nuclear antigens - break in immunological tolerance

This results in immune complex formation which activates the compliment - T cells activate B cells which produce autoantibodies and cytokines which causes tissue injury

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5
Q

What are the symptoms of lupus

A

Alopecia - hair loss

Nervous system issues

butterfly rash

photosensitivity

purpura and urticuaria

chest pain

abdominal pain

necrosis of the pain and artitis in small joints

myopathy

Cardiac issues

Anaemia, leukopenia and thrombocytopenia

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6
Q

What are the classifications for lupus

A

Patient requires at least 4 for lupus classification
Malar rash - butterfly rash
Discoid rash - alopecia included
photosensitivity
oral ulcers
arthritis
serositis - pleurisy or pericarditis
Renal issues
neurological - unexplained seizures or psychosis
Haematological - low WCC, platelets, lymphocytes and haemolytic anaemia
Immunological - anti ds-DNA, Sm, cardiolipin, lupus anticoagulant, low compliment
ANA - antinuclear antibodies

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7
Q

When should a diagnosis of lupus be considered

A

Women of childbearing age

Constitutional symptoms - fever, weight loss, malaise and fatigue

Skin rash / stomatitis

Arthritis

pleuritic chest pain

renal disease

cytopenia

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8
Q

What are the autoantibodies that are screened for in lupus

A

ANA - anti-nuclear antibodies - high sensitivity but low specificity

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9
Q

What other conditions have a positive ANA - anti-nuclear antibodies

A

normal people

rheumatoid arthritis

MS

infection

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10
Q

What are the autoantibodies that should be checked in lupus

A

ANA - anti-nuclear antibodies

Anti- ds DNA - anti- double stranded DNA - high specificity but is raised by a number of inflammatory conditions

Anti-Sm - highly specific for lupus but not that sensitive

Anti-Ro - risk of foetal congenital heart block

Antiphospholipid antibodies - anti-cardiolipin and lupus anticoagulant - associated with thrombosis and miscarriages

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11
Q

What is scleroderma

A

Fibrosis of the skin

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12
Q

What is limited cutaneous systemic sclerosis

A

The skin fibrosis is limited to distal to the elbows and knees

Skin fibrosis at head as well

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13
Q

What is diffuse cutaneous systemic sclerosis

A

Skin fibrosis - both trunk and proximal involvement

Skin fibrosis at head as well

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14
Q

What is localised scleroderma associated with

A

A good prognosis

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15
Q

Who does systemic sclerosis commonly occur in

A

30-50 years

Females more common then males

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16
Q

What can cause systemic sclerosis

A

Environmental:
silica dust
Industrial solvents
viral infection - e.g cytomegalovirus

Genetic predisposition

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17
Q

What are the 3 main mechanisms which happen in systemic sclerosis

A

vascular damage in the microcirculation
Immune system activation/ inflammation
Fibrosis

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18
Q

What is the common presentation of systemic scleroderma

A

Reynauds’s - vasospasm that causes decrease blood flow to the fingers – fingers become white, then blue, then red

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19
Q

What is the antibody found in limited systemic scleroderma

A

Anti centromere antibodies - specific for limited

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20
Q

What is the antibodies found in diffuse systemic scleroderma

A

Anti Scl70 antibodies - exclusive for diffuse

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21
Q

What are the main complications in limited systemic scleroderma

A

Pulmonary hypertension and GI issues such as oesophageal dysmotility

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22
Q

What are the complications of diffuse systemic scleroderma

A

pulmonary fibrosis
renal crisis
small bowel bacterial overgrowth

Patients appear very ill early on

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23
Q

What is sjogren’s syndrome

A

dry mucosal surfaces due to exocrine glands being affected due to rheumatoid arthritis or other connective tissue disorders

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24
Q

Who does sjpgrens syndrome more commonly occur in

A

40-50 year olds

Female 9:1

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25
Q

What is the presentation of Sjorgren’s syndrome

A

Severely dry eyes and mouth
Parotid gland enlargement
Some have systemic symptoms - fatigue, fever, myalgia, arthralgia and dry skin

26
Q

What antibodies are detected in Sjorgen’s syndrome

A

Anti RO and anti La (SSB_ antibodies

27
Q

how is Sjogren’s syndrome diagnosed

A

Salivary gland biopsy

28
Q

What are the complications of Sjorgen’s syndrome

A

lymphoma
neuropathy
cutaneous vasculitis
interstitial lung disease
renal tubular acidosis

29
Q

Who is autoimmune myositis more common in

A

females
50-60 years old

30
Q

What are the two types of autoimmune myositis

A

Polymyositis and dermatomyositis

31
Q

What is the difference between polymyositis and dermatomyositis

A

Dermatomyositis has some skin involvement while polymyositis is limited to the muscle

32
Q

Who is dermatomyositis more common in

A

Younger patients so can present in childhood

33
Q

Who does polymyositis more commonly affect

A

The older age groups

34
Q

What is the common complication of autoimmune - myositis

A

Increased risk of malignancy that should be screened

35
Q

how does myositis present

A

Symmetrical, proximal muscle weakness
raised CK level - creatine kinase
Interstitial lung disease

36
Q

What is the classical sign seen in dermatomyositis

A

Gottron’s papules - Erythematous rashes over the small hand joints

Heliotrope rash - peri-orbital oedema - rash on face but covers the nasolabial folds which separates the rash from the lupus butterfly rash

37
Q

What investigations are done for myositis

A

Electromyogram

MRI - shows muscle oedema in myositis

Muscle biopsy - gold standard diagnostic test

38
Q

What conditions are anti Jo1 antibodies found in and what does it increase the risk of

A

Myositis - increases risk of interstitial lung disease

39
Q

What is vasculitides

A

inflammation in the blood vessel wall

40
Q

What are the examples of large vessel vasculitides

A

Takayasu arteritis

Giant cell arteritis

41
Q

Who does takayasu arteritis commonly affect

A

Young women from far east asia - less than 50 usually

42
Q

What does takayasu arteritis present with

A

arteriole stenosis - carotid artery, subclavian, lower limbs

43
Q

Who does giant cell arteritis affect

A

Northwest europe
Older age group over 50 years old
Women

44
Q

What are thee two types of medium vessel vasculitides

A

polyarteritis nodosa

Kawasaki disease

45
Q

What is polyarteritis nodosa

A

Causes stenosis and small aneurysms with the risk of hameorrhage

Affects the renal and mesenteric arteries

46
Q

How does giant cell arteritis present

A

New sudden onset unilateral headache over the temporal area commonly

The pain is constant and only partially responds to analgesia

Jaw claudication - pain when chewing which reduces when you stop chewing

Scalp tenderness

47
Q

What is one of the major complications of giant cell arteritis

A

Irreparable visual loss

Other complications :
aortic aneurysms
arterial stenosis and limb ischaemia
stroke

48
Q

What is the treatment for giant cell arteritis

A

Immediate high does prednisolone : 40-60 mg per day which is then gradually tapered

PPI - gastric protection

bone protection - steroids can cause osteoporosis

steroid sparing medication

49
Q

What is the giant cell arteritis classification criteria and what score is required

A

3 of the following required :

Age of onset over 50

new headache

temporal artery tenderness or reduced pulsation

ESR equal or greater than 50 - inflammation marker

Abnormal temporal biopsy

50
Q

What is the issue of temp-oral artery biopsy in giant cell arteritis

A

The inflammation has skip lesions which means if you biopsy during one of the skips, you will get a false negative

51
Q

What gives diagnosis of giant cell arteritis

A

Ultrasound doppler of the temporal artery which shows oedema in the wall of the artery if there is giant cell arteritis

52
Q

What are common presentations in ANCA associated vasculitis

A

saddle nose
lung haemorrhage
skin vasculitis

53
Q

How is granulomatosis with polyangiitis (Wegner’s) characterised

A

Necrotising granulomatous inflammation

54
Q

What is the classical presentation of granulomatous with polyangiitis (Wegner’s)

A

Upper and lower respiratory tract affected

Hearing loss
sinusitis
haemoptysis
Necrotising glomerulonephritis is common

55
Q

What is normally checked for in granulomatosis with polyangiitis (Wegner’s)

A

cANCA and anti PR3 antibodies

56
Q

What is seen in microscopic polyangitis

A

Necrotising vasculitis affecting the small vessels

No granulomatous inflammation

Commonly renal and pulmonary involvement

Pulmonary haemorrage with renal failure is common presentation

57
Q

What is looked for in microscopic polyangitis

A

pANCA and anti MPO antibodies

58
Q

What is eosinophilic granulomatosis with polyangiitis characterised by

A

Eosinophil rich and necrotising granulomatous inflammation involving the respiratory tract

59
Q

What is eosinophilic granulomatosis polyangiitis associated with (Churg strauss)

A

Late onset asthma
nasal polyps
eosinophilia

60
Q

What is the common complications of eosinophilic granulomatosis polyangiitis

A

neurological involvement is most common

MSK (36 decks)

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