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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
What are some of the features of diffuse systemic sclerosis?
Pulmonary fibrosis Renal crisis Small bowel bacteria overgrowth
26
Who is more likely to get Sjogren's syndrome?
Female > male 40-50 yrs
27
What is Sjorgren's syndrome characterised by>
Lymphocitic infiltrate in exocrine glands e.g. tear and salivary glands so leads to occular dryness
28
Symptoms of Sjogren's syndrome?
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
Diagnosis of Sjogren's syndrome?
Anti Ro antibodies Anti La antibodies OR (if negative) Salivary gland biopsy
30
Which antibodies are found in patient's with Sjogren's?
Anti Ro antibodies Anti La antibodies
31
Sjogren's can cause multi-organ disease too as a complication. Give some examples.
Lymphoma Neuropathy Cutaneous vasculitis Interstitial lung disease Renal tubular acidosis
32
Who gets polymyelitis and dermatomyositis?
Females > males Peak incidence of 50-60
33
What are those with myositis at increased risk of?
Malignancy ->risk is highest at time of presentation
34
How do myositis patients present?
Muscle weakness- symmetrical, often complain of difficulty with stairs or getting out of chairs
35
Girl you're distracted because you're about to leave for convention....maybe look over this again
Hmm maybe idk
36
Diagnosis of myositis?
Raised creatinine kinase in bloods EMG MRI Muscle biopsy- gold standard
37
Which antibodies can be seen in myositis and what do they suggest if found?
Anti Jo1 antibodies- suggest increased risk of interstitial lung disease
38
Vasculitis?
Inflammation in the walls of blood vessels
39
Give some examples of large vessel vasculitis.
Takayasu arteritis Giant Cell arteritis
40
Give some examples of medium vessel vasculitis.
Polyarteritis Nodosa Kawasaki Disease
41
How does giant cell arthritis present?
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
Who tends to get GCA?
Older Female > male ->most common form of arteritis in this part of the world
43
What is a major complication of GCA if left untreated?
Blindness
44
GCA classification criteria?
3 of following: Age >50 New headache Temporal artery tenderness/reduced pulsation ESR > 50 Abnormal temporal biopsy
45
What is another less-invasive way of diagnosing GCA?
Ultrasound doppler CT angiograms MR angiogram FDG PET- for large vessel involvement, like GCA
46
Complications of GCA?
Irreversible visual loss Aortic aneurysms Arterial stenosis and limb ischaemia Stroke
47
Treatment of GCA?
Urgent initiation of high dose Prednisolone PPI Bone protection (because of long-term steroid)
48
Which vessels are affected by ANCA associated vasculitis?
Very small blood vessels
49
Three types of ANCA associated vasculitis
Granulomatosis with polyangititis (GPA) Microscopic polyangiitis Eosioniphillic granulamatomis with polyangiitis ->all v rare
50
What is GPA characterised by?
Necrotising granulamatous inflammation Usually involving upper and lower resp tract ->be suspicious of patient with reoccuring ear infections
51
Symptoms of GPA?
Hearing loss Sinusitis Hemoptysis
52
Which antibodies may be positive for GPA?
cANCA PR3 antibodies
53
What is the difference between GPA and MPA?
No granulomas in MPA on biopsy
54
Which other systems are commonly involved in MPA?
Renal and pulmonary involvement ->so might be coughing up blood and need a CXR
55
Which antibodies may be positive in MPA?
pANCA Anti MPO antibodies
56
Eosinophilic Granulomatosis with Polyangiitis aka EGPA?
Eosinophil rich and necrotsizing granulomatous inflammation, often involving respiratory tract
57
Symptoms of EGPA?
Late onset asthma Nasal polyps Eosinophilia
58
What other system can be involved in EGPA?
Neuro Cardiac, GI- poor prognosis
59
Antibodies positive in EGPA?
Anti MPO antibodies in about half of patients
60
Okayyyy time to round up What is a very specific autoantibody for lupus?
Anti-Sm (anti-smith) ->also DsDNA, Anti-Ro
61
Antibodies for Sjogren?
Anti-Ro Anti-La
62
Antibodies for limited scleroderma?
Centromere antibodies
63
Antibodies for diffuse scleorderma?
Scl70
64
Antibodies for myositis?
Jo1
65
Antibodies for GPA?
PR3 (c-ANCA)
66
Antibodies for MPA?
MPO (p-ANCA)
67

Year 2- MSK (36 decks)

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