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Connective Tissue Diseases Flashcards

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1
Q

List some common connective tissue diseases

A 
SLE
Sjogren’s syndrome
Systemic sclerosis
Dermatomyositis
Polymyositis
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2
Q

What are connective tissue diseases

A

NOT diseases of connective tissue
Spontaneous over activity of the immune system involving specific auto-antibodies
Can change with time and occasionally cause organ failure and death

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3
Q

Which parts of the body can SLE affect

A

Any part of the body

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4
Q

Which factors contribute to the aetiology of SLE

A

Genetic - can occur amongst relatives
Hormonal - linked to higher oestrogen exposure
Environmental
Immunological

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5
Q

Describe how autoimmunity arises in SLE

A 
Loss of immune regulation 
Increased and defective apoptosis 
Necrotic cells release nuclear material which act as auto-antigens 
This stimulates T and B cells 
Autoantibodies are produced
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6
Q

How does SLE lead to renal disease

A

Immune complexes of nuclear antigens and antibodies are deposited
This activates compliment system which attracts leucocytes
Leads to release of cytokines which cause inflammation, necrosis and scarring
Leads to glomerulonephritis

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7
Q

List some constitutional symptoms of SLE

A 
Fever
Malaise
Poor appetite
Weight loss
Fatigue
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8
Q

List the mucocutaneous features of SLE

A 
Photosensitivity
Malar rash - butterfly shape on face, sparing nasolabial folds
Discoid lupus erythematosus
Subacute cutaneous lupus
Mouth ulcers
Alopecia - patchy
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9
Q

What effect can SLE have on the heart and lungs

A

Pericarditis
Pericardial effusion

Pleurisy
Pleural effusion
Pulmonary hypertension - do echo
Interstitial lung disease - do CXR and pulmonary functions

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10
Q

List the neurological features of SLE

A 
Depression/psychosis
Migrainous headache
Seizures
Cranial or peripheral neuropathy
Mononeuritis multiplex
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11
Q

List the haematological features of SLE

A 
Lymphadenopathy 
Leucopenia
Lymphopenia
Haemolytic anaemia
Thrombocytopenia
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12
Q

What is anti-phospholipid syndrome

A

Syndrome associated with SLE and other autoimmune conditions
Causes unprovoked venous and arterial thrombosis, pre-eclampsia, recurrent miscarriage and thrombocytopenia

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13
Q

When should a positive ANA test be taken seriously

A

When the other anti-nuclear antibodies are also positive
AND
the patient is presenting with symptoms of a connective tissue disease

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14
Q

Which antibody test is the most specific for SLE

A

Anti-double stranded DNA antibody

However, not very sensitive - only 40-50% SLE patient have it

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15
Q

List the 3 main anti-phospholipid antibodies

A

Anti-cardiolipin antibody
Lupus anticoagulant
Anti-beta 2 glycoprotein

Can have just one or all 3 (all 3 increases risk of thrombosis)

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16
Q

Aside from antibody tests, what investigations may you do in an SLE patient

A

Depends on symptoms
Chest: CXR, pulmonary function tests or CT
Urine protein tests
Renal biopsy - if urine positive for protein or red cell casts
Echo - look for pulmonary hypertension
Nerve conduction study
MRI of brain

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17
Q

Describe the general (non drug) management of SLE

A

Counselling
Regular monitoring
Avoiding excessive sun-exposure

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18
Q

List some immunosuppressives used in the treatment of SLE

A

Methotrexate - good for synovitis
Azathioprine
Cyclophosphamide
Rituximab

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19
Q

What are some risks of immunosuppressive treatment

A

Can cause bone marrow suppression
Increased susceptibility to infection
Potentially teratogenic

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20
Q

Which treatments are used for mild SLE

A

Hydroxychloroquine - all patients start on this
Topical steroids
NSAID’s - to treat joint pain

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21
Q

Which treatments are used in moderate SLE

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A

Oral steroids
Azathioprine
Methotrexate

22
Q

Which treatments are used in severe SLE

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A

Steroid - IV, high dose or prolonged courses
Cyclophosphamide
Biologics - Rituximab, Belimumab

23
Q

In general what causes an autoimmune disease

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A

The immune system attacks self antigens as a result of breakdown of tolerance to autoreactive immune cells
Involves the adaptive immune system
Often has genetic, environmental and/or an infectious trigger

24
Q

Which group are more likely to have autoimmune disease

25
Which core clinical features would make you suspect an autoimmune disease
``` Arthralgia Muscle pain or weakness Photosensitivity Raynaud's Mucosal ulcers Alopecia Unexplained thrombosis SOB Neurological symptoms Recurrent pregnancy issues/ miscarriages ```
26
What is the general screening test for all autoimmune diseases
Screen for ANA If positive you then look for specific ones for certain conditions Do a ENA extractable nuclear antigens test - connective tissue screen Includes anti-ro, la, sm, RNP, SCl70, centromere etc.
27
Who gets primary Raynaud's
Teenagers No underlying autoimmune disease Benign condition typically seen when it is cold
28
Who gets secondary Raynaud's
Older age group Have underlying autoimmune disease - systemic sclerosis More likely to get associated ulcers or gangrene Seen all year round
29
How do you treat Raynaud's
Keep digits warm | Vasodilators - e.g. calcium channel blockers
30
What are the 2 subtypes of systemic sclerosis
Diffuse cutaneous - has skin involvement above and below the elbows and knees (everywhere basically) Limited cutaneous - skin involvement only on face and below knees and elbows Previously called CREST syndrome
31
What is calcinosis
calcium deposits in the skin
32
what is sclerodactyly
thickening and tightening of the skin on fingers and hands
33
What is telangiectasia
Dilation of capillaries causing red marks on the surface of the skin
34
How is systemic sclerosis managed
Yearly ECHO and pulmonary function tests Treat symptomatically: - treat Raynaud's - treat reflux with PPI etc - immunosuppressive for pulmonary fibrosis and skin involvement - tight control of blood pressure
35
What are some symptoms of Sjogren's syndrome
``` Dry, gritty eyes Dry mouth - drinking lots, struggling to swallow Dry cough Tooth decay Vaginal dryness ```
36
How do you diagnose Sjogren's syndrome
Antibody tests - ANA positive, look for Anti-Ro and Anti-La (La is the most specific) May also have RF - not specific If antibodies negative do salivary gland ultrasound and biopsy. Normally have raised ESR, plasma viscosity and IgG
37
How do you treat Sjogren's syndrome
Eye drops, salivary supplements and vaginal lubricants Good dental hygiene Hydroxychloroquine - treats fatigue/arthralgia Immunosuppressants if there is major organ involvement
38
What criteria must be met to diagnose anti-phospholipid syndrome
Presence of antiphospholipid antibodies on 2 occasions 12 weeks apart-
39
How do you treat anti-phospholipid syndrome
Lifelong anticoagulation if there is thrombosis - warfarin Pre-pregnancy LMWH and aspirin (or when pregnancy confirmed)which continue throughout pregnancy until 6 weeks post-partum
40
Why should you do an urinalysis on someone with SLE
Risk of glomerulonephritis - presents with haematuria (red cell casts in particular) and proteinuria Urine often changes before bloods do Do a dip and if more than a trace of protein you do a protein creatinine ratio and if blood send to micro to look for casts
41
How can SLE affect pregnancy
Active disease is associated with poor maternal and fetal outcomes - advised not to conceive until disease is stable for at least 6 months Some of the immunosuppressants are teratogenic - must modify drugs so safe (azithio or low dose hydroxy) Anti-Ro antibodies can congenital heartblock in 2- 5% of foetuses so montior baby if present Associated with anti-phospholipid which has high miscarriage risk
42
Which antibodies may be positive in SLE
``` ANA - 98% of patients Anti- double stranded DNA Anti-Ro Anti-smith - high titres only are suggestive RNP ```
43
Anaemia and cytopenia's are common in autoimmune diseases - true or false
True
44
Which autoimmune conditions can cause a raised IgG
Autoimmune liver diseases | Sjogren's
45
Photosensitivity is a sign of which autoimmune disease
SLE - will develop a rash days after exposure and it will last for weeks Different to prickly heat
46
What is sicca
Another name for Sjorgren's!! | Symptoms of waking up with dry mouth, eyes feeling gritty, difficulty swallowing dry food without water, vaginal dryness
47
What are the symptoms of limited systemic sclerosis
``` Calcification Raynaud's Oesophageal dysmotility - reflux Sclerodactyly Telangiectasia - including abnormal nailfold capillaries ```
48
Raynauds + reflux makes you think of which condition
limited systemic sclerosis
49
Mucosal ulceration is a sign of which autoimmune disease
SLE
50
Alopecia is a sign of which autoimmune disease
SLE | Can get male pattern baldness and patchy alopecia

MSK (21 decks)

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