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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
Who does it tend to affect / what is associated
``` YOUNG FEMALE Rare post-menopausal Sjogren Autoimmune thyroid disease Alopecia ```
26
What is typical presentation / mnemonic
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
27
How can skin be affected
``` Malar / butterfly rash Discoid rash Photosensitivity Raynaud's Livedo reticularis - non-blanching pink / blue mottling Alopecia Oral ulcers ```
28
How can joints be affected
Joints tend to be less affected than other arthritis Arthritis symptoms in 2 joints - no X-ray change Arthralgia Myalgia
29
Kidney affected
Lupus nephritis Proteinuria Haematuria GN
30
What are CVS / resp signs
CVS disease = leading causes of death due to inflammation causing hypertension Interstitial lung disease Pulmonary fibrosis Pleural effusion Pericarditis / myocarditis / endocarditis Stroke
31
What are neuro / psych signs
``` Stroke CN palsy Optic neuritis Transverse myelitis Neuropathy Seizures Psychosis Depression Anxiety ```
32
What are general features
``` Fatigue Weight loss PUO Lymphadenopathy Oral ulcers Anaemia - normocytic Thrombocytopenia Leucopenia Infections due to immunosuppression drugs ```
33
How do you Dx
``` History Immunological test Bloods Urinanalysis Biopsy - may need renal biopsy or CXR ```
34
What is seen on bloods
``` 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 ```
35
What should always be done
Urine dip
36
How do you monitor disease
``` Anti-dsDNA titre Complement level ESR BP Urine - protein / casts Bloods - FBC, U+E, LFT, CRP (usually normal) ```
37
What is a malar rash
Butterfly rash | Spares nasolabial folds
38
What is a discoid rash
``` Scaly erythematous well demarcated rash Photosensitive so worse with sunlight Sun-exposed area Can be pigmented / hyperkeratotic Associated with scarring alopecia ```
39
What is general Rx if Dx
High factor sun block Hydroxychloroquine unless CI - reduced disease activity Topical steroids if skin flares
40
What is maintenance Rx
NSAID (unless renal) + hydroxychloquine - good for joints and skin Can add azathioprine / methotrexate (steroid sparing) Biologics if severe
41
If develop lupus nephritis what may be needed
Intense immunosuppression with steroid | RRT
42
What drugs can cause a drug induced lupus
``` Hydralazine = common Procainamide Isoniazid Chloropramazine Phenytoin A nti-TNF ```
43
How does it commonly present
``` Arthralgia Myalgia Skin malaria rash Pleurisy Renal / nervous involvement = uncommon ```
44
What Ab
Anti-histone
45
How do you Rx
Stop drug
46
What are the three types of systemic sclerosis
Linear Limited Diffuse
47
What is linear
Small patches of tightening of skin (scleroderma) | Can't pinch
48
What is limited
Limited skin thickening to face, feet and arms CREST Syndrome Anti-centromere
49
What is CREST
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
What is diffuse disease
``` Whole body affected Trunk + proximal limbs Face - Beak nose - Microstomia - Radial furrowing - Jaw and teeth deformity Scl-70 Ab Anti-RNA ```
51
Who is affected
Middle aged female
52
How do you investigate
``` History Bloods - FBC, U+E Immunological test Urine dip and PCR Ba swallow ECG / ECHO CT ```
53
How do you monitor
Annual ECHO to look for pulmonary hypertension PFT annual BP + urine
54
What are complications of limited
Pulmonary hypertension
55
How do you Rx
Sildenafil - phosphoridertase inhibitor
56
What are complications of diffuse
``` Pulmonary fibrosis / ILD = common cause of death Pulmonary hypertension Small bowel overgrowth = diarrhoea Renal crisis Hypertension due to renal fibrosis Arrhythmia Dysphagia Aspiration ```
57
What is Sjogren's
Immune attack on lacrimal and salary glands | Commonly middle aged female
58
How does Sjogren present
``` 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
Other Sx (less common)
``` Polyarthritis Pulmonary fibrosis Vasculitis Recurrent parotiditis Renal tubular acidosis ```
60
How do you Dx and Rx
``` Dx = Schimmer test = <5mm tears Artificial saliva and tears Pilocarpine to stimulate saliva Vaginal lubricants Hydroxycholorqquine to halt progression ```
61
What is seen on histology
Lymphocytic infiltration Hyper Ig Low C4
62
What are complications
``` Eye infections B cell lymphoma / NHL Neuropathy Vasculitis Purpura ILD Renal tubular acidosis ```
63
What conditions can cause an inflammatory myositis
Polymyositis | Dermatomyositis
64
What are the Sx
``` Muscle weakness Symmetrical Often proximal so difficulty stairs NOT stiff (full ROM) Myalgia / arthralgia No pain ```
65
What are extra-articular features for both
``` Fever Raynaud's ILD Resp muscle weakness Dysphagia and dysphonia Myocarditis Arrhythmia ```
66
Who is affected
Young | M=F
67
How do you investigate
``` 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
How do you Rx
Steroids Immunosuppression if resistant Hydroxychloroquine if skin involvement
69
How does polymyositis tend to present
Dysphagia Dysphonia Resp weakness NO SKIN
70
What can dermatomyositis be caused by
Autoimmune connective tissue | Underlying malignancy - lung / pancreatic/. ovarian / bowel so screen for underlying malignancy e.g. CT
71
What are skin features
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
Who is affected dermatomyositis
Elderly female
73
What is anti-phospholipid syndrome
``` Venous thrombosis - DVT / PE Arterial thrombosis - stroke / MI Recurrent fetal loss Thrombocytopenia Levido reticularis PET Pulmonary hypertension ```
74
What can cause
Primary | 2 to SLE
75
How do you Dx
2 episodes of clots or fatal loss + Ab Anti-phospholipid Ab Lupus anti-coagulant Anti-cardolipin
76
What else in seen in bloods
Rise APTT Normal PT Low platelet
77
How do you Rx
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
What do you give if pregnant
LMWH + aspirin
79
How do you remember complications
``` CLOTS Coag defect Levido reticularis Obstetric - miscarriage / PET Thrombocytopenia ```
80
What is Raynaud's
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
What suggests underlying connective tissue / autoimmune
``` >40 Unilateral Rash Presence of Ab Digital ulcer Calcinosis Chillblains ```
82
What are secondary causes
``` Scleroderma RA SLE Leukaemia OCP Cervical rib ```
83
How do you Rx
Reassure Stop smoking CCB to dilate blood vessels Other agents if refractory - isoprost / sidenafil
84
What is Eshler Danlos
AD connective tissue disorder of type III collagen
85
What are the symptoms
Hypermobility Recurrent dislocation Fragile elastic skin Easily bruising
86
What are complications
``` Aortic regurgitation Aortic dissection MVP SAH Retinal streaks ```
87
What is Langherhan cell histiocytosis
Rare cancer causing abnormal proliferation of Langherhan cells / histiocytes Derived from bone marrow and capable of migrating from skin to LN
88
What are the Sx
Bone pain - skull / femur Cutaneous nodules Recurrent otitis media / mastoidits
89
How do you Dx
X-ray = osteolytic lesions | Electron microscopy

MSK (21 decks)

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