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MSK > Connective Tissue Diseases > Flashcards

Connective Tissue Diseases Flashcards

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

A

Oral steroids
Azathioprine
Methotrexate

22
Q

Which treatments are used in severe SLE

A

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

23
Q

In general what causes an autoimmune disease

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

A

Women

25
Q

Which core clinical features would make you suspect an autoimmune disease

A 
Arthralgia 
Muscle pain or weakness 
Photosensitivity
Raynaud's 
Mucosal ulcers
Alopecia 
Unexplained thrombosis
SOB 
Neurological symptoms 
Recurrent pregnancy issues/ miscarriages
26
Q

What is the general screening test for all autoimmune diseases

A

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
Q

Who gets primary Raynaud’s

A

Teenagers
No underlying autoimmune disease
Benign condition typically seen when it is cold

28
Q

Who gets secondary Raynaud’s

A

Older age group
Have underlying autoimmune disease - systemic sclerosis
More likely to get associated ulcers or gangrene
Seen all year round

29
Q

How do you treat Raynaud’s

A

Keep digits warm

Vasodilators - e.g. calcium channel blockers

30
Q

What are the 2 subtypes of systemic sclerosis

A

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
Q

What is calcinosis

A

calcium deposits in the skin

32
Q

what is sclerodactyly

A

thickening and tightening of the skin on fingers and hands

33
Q

What is telangiectasia

A

Dilation of capillaries causing red marks on the surface of the skin

34
Q

How is systemic sclerosis managed

A

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
Q

What are some symptoms of Sjogren’s syndrome

A 
Dry, gritty eyes 
Dry mouth - drinking lots, struggling to swallow 
Dry cough 
Tooth decay 
Vaginal dryness
36
Q

How do you diagnose Sjogren’s syndrome

A

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
Q

How do you treat Sjogren’s syndrome

A

Eye drops, salivary supplements and vaginal lubricants
Good dental hygiene
Hydroxychloroquine - treats fatigue/arthralgia
Immunosuppressants if there is major organ involvement

38
Q

What criteria must be met to diagnose anti-phospholipid syndrome

A

Presence of antiphospholipid antibodies on 2 occasions 12 weeks apart-

39
Q

How do you treat anti-phospholipid syndrome

A

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
Q

Why should you do an urinalysis on someone with SLE

A

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
Q

How can SLE affect pregnancy

A

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
Q

Which antibodies may be positive in SLE

A 
ANA - 98% of patients 
Anti- double stranded DNA 
Anti-Ro
Anti-smith - high titres only are suggestive
RNP
43
Q

Anaemia and cytopenia’s are common in autoimmune diseases - true or false

A

True

44
Q

Which autoimmune conditions can cause a raised IgG

A

Autoimmune liver diseases

Sjogren’s

45
Q

Photosensitivity is a sign of which autoimmune disease

A

SLE - will develop a rash days after exposure and it will last for weeks
Different to prickly heat

46
Q

What is sicca

A

Another name for Sjorgren’s!!

Symptoms of waking up with dry mouth, eyes feeling gritty, difficulty swallowing dry food without water, vaginal dryness

47
Q

What are the symptoms of limited systemic sclerosis

A 
Calcification 
Raynaud's
Oesophageal dysmotility - reflux 
Sclerodactyly 
Telangiectasia - including abnormal nailfold capillaries
48
Q

Raynauds + reflux makes you think of which condition

A

limited systemic sclerosis

49
Q

Mucosal ulceration is a sign of which autoimmune disease

A

SLE

50
Q

Alopecia is a sign of which autoimmune disease

A

SLE

Can get male pattern baldness and patchy alopecia

MSK (21 decks)

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