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MSK > Autoimmune Disease > Flashcards

Autoimmune Disease Flashcards

1
Q

When should you consider autoimmune connective tissue

A

Any unwell patient with multi-organ involvement

Especially if no infection

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2
Q

What is ANA

A 
Tells something is wrong with immune system
General Ab screen 
Sensitive but NOT specific
Can occur with MS / infection
30+ of people in health have
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3
Q

When do you test for ANCA

A

Vasculitis

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4
Q

what is cANCA +Ve in and what is target

A

Known known as proteinase 3 / PR3 Ab (or target)
Macroscopic vasculitis
Granulomatosis with polyangiitis

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5
Q

What is pANCA +ve in and target

A

Known known as MPO - myeloperoxidase (or target)

Microscopic vasculitis
Churg Strauss
Microscopic polyangiitis 
PSC
GN
SLE
Sjogren
RA
IBD
Autoimmune hepatitis
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6
Q

Is suspecting autoimmune connective tissue disease what is important to do

A

Specific nuclear antigens / ENA

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7
Q

Specific antigens for SLE

A 
ANA 
Anti-dsDNA
Anti-Ro
Anti-SM
Low complement
Lupus anti-coagulant
Anti-phospholipid can be +Ve
RF can be +Ve
\+Ve DAT
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8
Q

Specific antigens for Sjogren

A

Anti-Ro
Anti-La
Can be RF +Ve / ANA

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9
Q

Specific antigens for Sclerosis

A

Anti-centromere in limited
Anti-Scl-70 in diffuse
Ant-RNA pol 1,2,3
Can be ANA / RF +ve

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10
Q

Specific antigens for polymyositis

A

Anti-Jo1

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11
Q

What antigen if presenting with mixed connective tissue disease

A

Anti-RNP

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12
Q

Specific antigens for anti-phospholipid

A 
Technically not ENA as not part of nucleus 
Anti-carpolipin
Anti-phospholipid 
Anti-binding globulin
Lupus anti-coagulant
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13
Q

What is important to remember

A

Ab often overlap
Conditions overlap too
Clinical Hx then support from immunology is important for Dx

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14
Q

How do you investigate autoimmune connective tissue disease

A 
Hx 
Immunological bloods if specific - ANA + specific 
ANCA if suspect vasculitis 
Biopsy = best way to Dx
PET may show inflammaiton
USS may show vasculitis
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15
Q

CRP doesn’t tend to rise in flares so what does a raised CRP suggest

A

Sepsis
Synovitis
Serositis (pleurisy / peritonitis)

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16
Q

What are maintenance Rx

A

NSAID + hydroxychoroquine for joint and skin = most mild
DMARD - Azathioprine / methotrexate = steroid sparing
Biologics - Monoclonal Ab

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17
Q

What is used if mild flare

A

Mild medications
DMARD - hydroxychloroquine
Low dose steroid

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18
Q

What is used if moderate flare with organ involvement

A

Add immunosuppression e.g. MMF

+ DMARD

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19
Q

What may be needed in severe flare

A

High dose steroids
Cyclophosphamide
Rituximab

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20
Q

What do you do if on long term steroid

A

DEXA
Bone protection with biphosphonates
Increased risk of infection but sometimes patient requires

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21
Q

What is 5 year damage

A 
Nerve damage
Malignancy
Intersitial lung disease
End stage renal
CVS disease 
Osteoporosis
Hearing loss
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22
Q

What is severe complications

A

Haemolytic anaemia
Nephritis
Severe pericarditis or CNS

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23
Q

What annual monitoring is needed to look for complications

A 
Clinic
ECHO for pulmonary hypertension
PFT for ILD
BP 
Urine dip - look for protein or blood (nephritis or nephrotic) 
Bloods - FBC, U+E, LFT, CRP, autoimmune
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24
Q

What is SLE (Lupus) and what type of hypersensitive

A

Autoimmune disease
Ab targeted at double stranded DNA
Type 3 hypersensitivity reaction

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25
Q

Who does it tend to affect / what is associated

A 
YOUNG FEMALE  
Rare post-menopausal
Sjogren
Autoimmune thyroid disease
Alopecia
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26
Q

What is typical presentation / mnemonic

A

Relapsing remitting
Joints / Skin / Kidney
Need 4+ to Dx

SOAPBRAIN MD

  • Serositis - pleuritic / peri
  • Oral painful ulcers
  • Arthritis + stiffness
  • Photosensitivity
  • Blood disorder
  • Renal immune complex
  • ANA+ve
  • Immune- anti-dsDNA
  • Neuro
  • Malar / butterfly rash
  • Discoid rash
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27
Q

How can skin be affected

A 
Malar / butterfly rash 
Discoid rash
Photosensitivity 
Raynaud's
Livedo reticularis - non-blanching pink / blue mottling 
Alopecia 
Oral ulcers
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28
Q

How can joints be affected

A

Joints tend to be less affected than other arthritis
Arthritis symptoms in 2 joints - no X-ray change
Arthralgia
Myalgia

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29
Q

Kidney affected

A

Lupus nephritis
Proteinuria
Haematuria
GN

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30
Q

What are CVS / resp signs

A

CVS disease = leading causes of death due to inflammation causing hypertension
Interstitial lung disease
Pulmonary fibrosis
Pleural effusion
Pericarditis / myocarditis / endocarditis
Stroke

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31
Q

What are neuro / psych signs

A 
Stroke 
CN palsy
Optic neuritis 
Transverse myelitis 
Neuropathy
Seizures
Psychosis 
Depression
Anxiety
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32
Q

What are general features

A 
Fatigue
Weight loss
PUO
Lymphadenopathy 
Oral ulcers 
Anaemia - normocytic
Thrombocytopenia
Leucopenia
Infections due to immunosuppression drugs
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33
Q

How do you Dx

A 
History 
Immunological test
Bloods 
Urinanalysis 
Biopsy - may need renal biopsy or CXR
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34
Q

What is seen on bloods

A 
Low WCC
Low platelet
Low complement 
Low Hb - anaemia of chronic disease 
\+Ve DAT
Raised ESR / CRP
Raise Ig due to activation of B cells in inflammation
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35
Q

What should always be done

A

Urine dip

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36
Q

How do you monitor disease

A 
Anti-dsDNA titre
Complement level
ESR
BP 
Urine - protein / casts
Bloods - FBC, U+E, LFT, CRP (usually normal)
37
Q

What is a malar rash

A

Butterfly rash

Spares nasolabial folds

38
Q

What is a discoid rash

A 
Scaly erythematous well demarcated rash 
Photosensitive so worse with sunlight 
Sun-exposed area
Can be pigmented / hyperkeratotic
Associated with scarring alopecia
39
Q

What is general Rx if Dx

A

High factor sun block
Hydroxychloroquine unless CI - reduced disease activity
Topical steroids if skin flares

40
Q

What is maintenance Rx

A

NSAID (unless renal) + hydroxychloquine - good for joints and skin
Can add azathioprine / methotrexate (steroid sparing)
Biologics if severe

41
Q

If develop lupus nephritis what may be needed

A

Intense immunosuppression with steroid

RRT

42
Q

What drugs can cause a drug induced lupus

A 
Hydralazine = common
Procainamide
Isoniazid
Chloropramazine
Phenytoin
A nti-TNF
43
Q

How does it commonly present

A 
Arthralgia
Myalgia
Skin malaria rash
Pleurisy
Renal / nervous involvement = uncommon
44
Q

What Ab

A

Anti-histone

45
Q

How do you Rx

A

Stop drug

46
Q

What are the three types of systemic sclerosis

A

Linear
Limited
Diffuse

47
Q

What is linear

A

Small patches of tightening of skin (scleroderma)

Can’t pinch

48
Q

What is limited

A

Limited skin thickening to face, feet and arms
CREST Syndrome
Anti-centromere

49
Q

What is CREST

A

C - calcinosis
R - Raynaud’s (can be 1st sign)
E - oesophageal dysmotility (reflux / dysphagia)
S - sclerodactyl (localised thickening of fingers and toe)
T - telangiectasia

50
Q

What is diffuse disease

A 
Whole body affected
Trunk + proximal limbs 
Face
- Beak nose
- Microstomia
- Radial furrowing
- Jaw and teeth deformity 
Scl-70 Ab
Anti-RNA
51
Q

Who is affected

A

Middle aged female

52
Q

How do you investigate

A 
History 
Bloods - FBC, U+E
Immunological test
Urine dip and PCR
Ba swallow
ECG / ECHO
CT
53
Q

How do you monitor

A

Annual ECHO to look for pulmonary hypertension
PFT annual
BP + urine

54
Q

What are complications of limited

A

Pulmonary hypertension

55
Q

How do you Rx

A

Sildenafil - phosphoridertase inhibitor

56
Q

What are complications of diffuse

A 
Pulmonary fibrosis / ILD = common cause of death
Pulmonary hypertension
Small bowel overgrowth = diarrhoea
Renal crisis
Hypertension due to renal fibrosis
Arrhythmia 
Dysphagia 
Aspiration
57
Q

What is Sjogren’s

A

Immune attack on lacrimal and salary glands

Commonly middle aged female

58
Q

How does Sjogren present

A 
Sicca Sx - dry eyes (keratoconjunctivitis) / mouth
Dry mouth = halitosis / dysphagia 
Bilateral parotid gland enlargement 
Vaginal dryness 
Systemic Sx - fatigue / fever 
Arthralgia
Raynaud's 
Polyneuropathy
Purpura
Can present with dipsogenic DI as constantly drink water
59
Q

Other Sx (less common)

A 
Polyarthritis
Pulmonary fibrosis
Vasculitis
Recurrent parotiditis 
Renal tubular acidosis
60
Q

How do you Dx and Rx

A 
Dx = Schimmer test = <5mm tears 
Artificial saliva and tears 
Pilocarpine to stimulate saliva
Vaginal lubricants
Hydroxycholorqquine to halt progression
61
Q

What is seen on histology

A

Lymphocytic infiltration
Hyper Ig
Low C4

62
Q

What are complications

A 
Eye infections 
B cell lymphoma / NHL
Neuropathy
Vasculitis 
Purpura
ILD
Renal tubular acidosis
63
Q

What conditions can cause an inflammatory myositis

A

Polymyositis

Dermatomyositis

64
Q

What are the Sx

A 
Muscle weakness
Symmetrical 
Often proximal so difficulty stairs 
NOT stiff (full ROM) 
Myalgia / arthralgia 
No pain
65
Q

What are extra-articular features for both

A 
Fever
Raynaud's
ILD
Resp muscle weakness 
Dysphagia and dysphonia 
Myocarditis
Arrhythmia
66
Q

Who is affected

A

Young

M=F

67
Q

How do you investigate

A 
Muscle enzymes to look for damage - ALT / AST / LDH 
CK = most important 
Ab - Anti-Jo1
EMG to look for nerve damage
Muscle biopsy = confirms
68
Q

How do you Rx

A

Steroids
Immunosuppression if resistant
Hydroxychloroquine if skin involvement

69
Q

How does polymyositis tend to present

A

Dysphagia
Dysphonia
Resp weakness
NO SKIN

70
Q

What can dermatomyositis be caused by

A

Autoimmune connective tissue

Underlying malignancy - lung / pancreatic/. ovarian / bowel so screen for underlying malignancy e.g. CT

71
Q

What are skin features

A

Macular rash
Photosensitivty
Helitrope - peri-orbital violet discolouration
Gottron’s papules - rough red over extensor
Nail fold capillary dilatation
Telangiectasia - peri-ungal
Shawl sign - over shoulder photosensitive rash

72
Q

Who is affected dermatomyositis

A

Elderly female

73
Q

What is anti-phospholipid syndrome

A 
Venous thrombosis - DVT / PE 
Arterial thrombosis - stroke / MI 
Recurrent fetal loss
Thrombocytopenia
Levido reticularis
PET 
Pulmonary hypertension
74
Q

What can cause

A

Primary

2 to SLE

75
Q

How do you Dx

A

2 episodes of clots or fatal loss + Ab
Anti-phospholipid Ab
Lupus anti-coagulant
Anti-cardolipin

76
Q

What else in seen in bloods

A

Rise APTT
Normal PT
Low platelet

77
Q

How do you Rx

A

VTE
Warfarin 6 months target INR 2-3
If recurrent event = life long with target 3-4

If arterial
Life long warfarin target 2-3

78
Q

What do you give if pregnant

A

LMWH + aspirin

79
Q

How do you remember complications

A 
CLOTS
Coag defect
Levido reticularis
Obstetric - miscarriage / PET 
Thrombocytopenia
80
Q

What is Raynaud’s

A

Discolouration of fingers in response to cold
Then go blue / red and painful
Due to constriction of vessels
Usually young women and bilateral

Can lead to digital ischaemia (white fingers) / digital ulcers

81
Q

What suggests underlying connective tissue / autoimmune

A 
>40
Unilateral 
Rash
Presence of Ab
Digital ulcer
Calcinosis
Chillblains
82
Q

What are secondary causes

A 
Scleroderma
RA
SLE
Leukaemia
OCP
Cervical rib
83
Q

How do you Rx

A

Reassure
Stop smoking
CCB to dilate blood vessels
Other agents if refractory - isoprost / sidenafil

84
Q

What is Eshler Danlos

A

AD connective tissue disorder of type III collagen

85
Q

What are the symptoms

A

Hypermobility
Recurrent dislocation
Fragile elastic skin
Easily bruising

86
Q

What are complications

A 
Aortic regurgitation
Aortic dissection
MVP 
SAH 
Retinal streaks
87
Q

What is Langherhan cell histiocytosis

A

Rare cancer causing abnormal proliferation of Langherhan cells / histiocytes
Derived from bone marrow and capable of migrating from skin to LN

88
Q

What are the Sx

A

Bone pain - skull / femur
Cutaneous nodules
Recurrent otitis media / mastoidits

89
Q

How do you Dx

A

X-ray = osteolytic lesions

Electron microscopy

MSK (21 decks)

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