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Year 2- MSK > Multi-System Autoimmune Disease > Flashcards

Multi-System Autoimmune Disease Flashcards

1
Q

What is the gold-standard modality for diagnosis of a lot of autoimmune diseases?

A

Biopsies

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2
Q

Autoimmune diseases are diagnosed by exclusion.
What are some of the things that autoimmune conditions can mimic?

A

Malignancies
Infectons- endocarditis, Hep.B and C, HIV

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3
Q

Use of which drug can mimic symptoms of/cause autoimmune disease?

A

Cocaine
Minocycline

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4
Q

How can infection be eliminated from diagnosis?

A

Blood cultures

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5
Q

How can malignancy be excluded from diagnosis?

A

Screening/scanning

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6
Q

Who tends to get Lupus?

A

Female > male
Onset 15-15yrs
Ethnicity: Afro-Caribbean > Asian > Caucasian

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7
Q

Causes of lupus?

A

Genetics
Environmental; drugs, infections and UV light
Hormones

->patients with lupus may develop a flare of their lupus in response to UV light

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8
Q

Pathogenesis of lupus?

A

Immune response against endogenous nuclear antigens
Immune complex formation an complement activation leading to tissue injury

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9
Q

Clinical signs of lupus?

A

Butterfly rash
Fever
Depression
Alopecia
Myopathy
Abdominal pain
Heart; pericarditis, endocarditis, aortic heart lesions
NS; ataxia, hemiplegia
Raynaud’s
Arthritis in small joints

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10
Q

Classification criteria (not diagnostic) can point towards a diagnosis of Lupus.
A patient needs any four of the following:

A

Butterfly/malar rash
Discoid rash
Photosensitivity
Oral ulcers
Arthritis
Serositis
Renal (significant proteuria or cellular casts)
Neurological (unexplained seizures or psychosis)
Haematological (low WCC, platelets, haemolytic anaemia)
Immunological
ANA

->so 4//11, just read through for general idea

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11
Q

Kidney function really important if suspecting lupus.
What investigation needs to be done if suspcious?

A

Urine dip

->kidney involvement may be first sign of Lupus but can progress at any time during Lupus so need to keep an eye on kidney function

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12
Q

Just read through this general presentation of lupus x

A

Women of childbearing age
Fever, weight loss, malaise, severe fatigue
Skin rsh
Arthritis
Pleuritic chest pain
Renal disease
Cytopenia

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13
Q

Autoantibodies can be seen in 95% of patients but what is important to remember>?

A

Not specific for lupus
5-15% of healthy population has ANA too

->however, negative ANAs means <3% chance of Lupus

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14
Q

Which other autoanbodies should be considered in lupus?

A

Anti-ds DNA- highly specific
Anti-Sm- highly specific
Anti-Ro- neonatal lupus
Antiphospholipid antibodies

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15
Q

Scleraderma?

A

Chronic hardening and contraction of the skin and connective tissue

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16
Q

Two types of scleraderma?

A

Systemic
Localised

->localised scleroderma associated with good prognosis

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17
Q

What does sclerderma look like?

A

Google but similar to browny bruises kind of

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18
Q

Who tends to get systemic scelrosis?

A

Female > male (3:1)
Onset- 30-50yrs

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19
Q

Causes of systemic sclerosis?

A

Environmental; silica dust, solvents, viral infections
Genetic predisposition

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20
Q

Pathogenesis of systemic sclerosis?

A

Vascular damage
Immune system activation
Fibrosis

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21
Q

What tends to be the presenting symptom of a patient with systemic sclerosis?

A

Raynauds

->tends to occur in children and teenagers so not then but with a new presentation

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22
Q

What antibodies are positive in limited systemic sclerosis?

A

Anti-centromere antibodies

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23
Q

What antibodies are positive in diffuse systemic sclerosis?

A

Anti Scl17 antibodies

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24
Q

What are some of the features of limited systemic sclerosis?

A

Pulmonary hypertension
GI symptoms

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25
Q

What are some of the features of diffuse systemic sclerosis?

A

Pulmonary fibrosis
Renal crisis
Small bowel bacteria overgrowth

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26
Q

Who is more likely to get Sjogren’s syndrome?

A

Female > male
40-50 yrs

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27
Q

What is Sjorgren’s syndrome characterised by>

A

Lymphocitic infiltrate in exocrine glands e.g. tear and salivary glands so leads to occular dryness

28
Q

Symptoms of Sjogren’s syndrome?

A

Dry eyes and mouth, feeling of sand or grit in eyes

1 in 3 have systemic upset e.g. fever, malaise, myalgia, dry skin

Parotid gland enlargement

->common during menopause age but these are also symptoms of menopause so difficult

29
Q

Diagnosis of Sjogren’s syndrome?

A

Anti Ro antibodies
Anti La antibodies
OR (if negative)
Salivary gland biopsy

30
Q

Which antibodies are found in patient’s with Sjogren’s?

A

Anti Ro antibodies
Anti La antibodies

31
Q

Sjogren’s can cause multi-organ disease too as a complication.

Give some examples.

A

Lymphoma
Neuropathy
Cutaneous vasculitis
Interstitial lung disease
Renal tubular acidosis

32
Q

Who gets polymyelitis and dermatomyositis?

A

Females > males
Peak incidence of 50-60

33
Q

What are those with myositis at increased risk of?

A

Malignancy

->risk is highest at time of presentation

34
Q

How do myositis patients present?

A

Muscle weakness- symmetrical, often complain of difficulty with stairs or getting out of chairs

35
Q

Girl you’re distracted because you’re about to leave for convention….maybe look over this again

A

Hmm maybe idk

36
Q

Diagnosis of myositis?

A

Raised creatinine kinase in bloods
EMG
MRI
Muscle biopsy- gold standard

37
Q

Which antibodies can be seen in myositis and what do they suggest if found?

A

Anti Jo1 antibodies- suggest increased risk of interstitial lung disease

38
Q

Vasculitis?

A

Inflammation in the walls of blood vessels

39
Q

Give some examples of large vessel vasculitis.

A

Takayasu arteritis
Giant Cell arteritis

40
Q

Give some examples of medium vessel vasculitis.

A

Polyarteritis Nodosa
Kawasaki Disease

41
Q

How does giant cell arthritis present?

A

Unilateral new onset headache
Jaw claudication- pain on chewing/after chewing
Scalp tenderness
Fever, weight loss, fatigue
->jaw claudication is due to damage to masseter muscle

42
Q

Who tends to get GCA?

A

Older
Female > male

->most common form of arteritis in this part of the world

43
Q

What is a major complication of GCA if left untreated?

A

Blindness

44
Q

GCA classification criteria?

A

3 of following:

Age >50
New headache
Temporal artery tenderness/reduced pulsation
ESR > 50
Abnormal temporal biopsy

45
Q

What is another less-invasive way of diagnosing GCA?

A

Ultrasound doppler
CT angiograms
MR angiogram
FDG PET- for large vessel involvement, like GCA

46
Q

Complications of GCA?

A

Irreversible visual loss
Aortic aneurysms
Arterial stenosis and limb ischaemia
Stroke

47
Q

Treatment of GCA?

A

Urgent initiation of high dose Prednisolone
PPI
Bone protection (because of long-term steroid)

48
Q

Which vessels are affected by ANCA associated vasculitis?

A

Very small blood vessels

49
Q

Three types of ANCA associated vasculitis

A

Granulomatosis with polyangititis (GPA)
Microscopic polyangiitis
Eosioniphillic granulamatomis with polyangiitis

->all v rare

50
Q

What is GPA characterised by?

A

Necrotising granulamatous inflammation
Usually involving upper and lower resp tract

->be suspicious of patient with reoccuring ear infections

51
Q

Symptoms of GPA?

A

Hearing loss
Sinusitis
Hemoptysis

52
Q

Which antibodies may be positive for GPA?

A

cANCA
PR3 antibodies

53
Q

What is the difference between GPA and MPA?

A

No granulomas in MPA on biopsy

54
Q

Which other systems are commonly involved in MPA?

A

Renal and pulmonary involvement

->so might be coughing up blood and need a CXR

55
Q

Which antibodies may be positive in MPA?

A

pANCA
Anti MPO antibodies

56
Q

Eosinophilic Granulomatosis with Polyangiitis aka EGPA?

A

Eosinophil rich and necrotsizing granulomatous inflammation, often involving respiratory tract

57
Q

Symptoms of EGPA?

A

Late onset asthma
Nasal polyps
Eosinophilia

58
Q

What other system can be involved in EGPA?

A

Neuro
Cardiac, GI- poor prognosis

59
Q

Antibodies positive in EGPA?

A

Anti MPO antibodies in about half of patients

60
Q

Okayyyy time to round up
What is a very specific autoantibody for lupus?

A

Anti-Sm (anti-smith)

->also DsDNA, Anti-Ro

61
Q

Antibodies for Sjogren?

A

Anti-Ro
Anti-La

62
Q

Antibodies for limited scleroderma?

A

Centromere antibodies

63
Q

Antibodies for diffuse scleorderma?

A

Scl70

64
Q

Antibodies for myositis?

A

Jo1

65
Q

Antibodies for GPA?

A

PR3 (c-ANCA)

66
Q

Antibodies for MPA?

A

MPO (p-ANCA)

67
Q
A

Year 2- MSK (36 decks)

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