Connective tissue conditions

Question 1

What type of hypersensitivity is SLE?

Answer 1

Type III hypersensitivity

Question 2

What are the two most likely patient groups to have SLE?

Answer 2

Chinese women at childbearing age

People of African-American heriatage

Question 3

What are the main symptoms of SLE?

Answer 3

Malar rash
Photosensitivity
Non-scarring alopecia
Livedo reticularis
Arthritis, arthralgia, myalgia

Question 4

What blood results are expected in SLE?

Answer 4

Decreased WBC and platelets
Decreased FBC

Question 5

What are the three antibodies associated with SLE?

Answer 5

ANA
Anti-Smith
Anti-dsDNA

Question 6

How is mild-moderate SLE managed?

Answer 6

Hydroxychloroquine
NSAIDs for symptoms

Question 7

How is moderate-severe SLE managed?

Answer 7

Hydroxychloroquine
Immunosuppressants and steroids (methotrexate or azathioprine)

Question 8

How is severe SLE managed?

Answer 8

Immunosuppressants
Cyclophosphamide
Mycophenolate
Rituximab
IV steroids

Question 9

What is Sjogren’s?

Answer 9

An autoimmune inflammation affecting exocrine glands characterised by lymphocytic infiltrates

Question 10

What is the key symptom of Sjogren’s?

Answer 10

Dry mucous membranes - eyes, mouth, throat, vagina etc.

Question 11

What test can be done to confirm Sjogren’s?

Answer 11

Schirmers test

Question 12

How is Sjogren’s managed?

Answer 12

Tear + salivary replacement
Analgesia

Hydroxychloroquine may be given for arthralgia and fatigue

Question 13

What is antiphospholipid syndrome?

Answer 13

Antiphospholipid antibodies react against proteins that bind to anionic phospholipids on plasma membranes

Question 14

What are the symptoms of antiphospholipid syndrome?

Think CLOT

Answer 14

Clots
Livedo reticularis
Obstetric loss
Thrombocytopenia

Question 15

What investigations should be done in antiphospholipid syndrome?

Answer 15

Lupus anticoagulant
Anticardiolipin
Anti-beta-2-glycoprotein

Question 16

How is antiphospholipid syndrome managed?

Answer 16

Lifelong warfarin if thrombotic event occurs

Aspirin if no thrombotic event

LMWH in pregnancy if recurrent pregnancy loss

Question 17

What distribution is seen in diffuse systemic sclerosis?

Answer 17

Above elbows and knees, includes face and trunk

Question 18

What distribution is seen in limited systemic sclerosis?

Answer 18

Distal to elbows and knees and face, does not include trunk

Question 19

What are the common symptoms of diffuse systemic sclerosis?

Answer 19

Raynaud’s
Thickened skin
Renal crisis
Interstitial lung disease

Question 20

What are the common symptoms of limited systemic sclerosis?

Think CREST

Answer 20

Calcinosis
Raynaud’s
Enteric dysmotility
Sclerodactyly
Telangiectasia

Question 21

What are the facial symptoms of systemic sclerosis?

Answer 21

Small mouth with puckered lips
‘Beaked’ nose
Lack of wrinkles

Question 22

What is the management of systemic sclerosis?

Answer 22

Avoid smoking
Avoid cold
Emollients
Analgesia
Nifedipine for Raynaud’s

Question 23

What is myositis characterised by?

Answer 23

Muscle weakness

Question 24

What is polymyostis?

Answer 24

An idiopathic inflammatory myopathy that causes symmetrical, proximal muscle weakness

Question 25

What is dermatomyositis?

Answer 25

An idiopathic inflammatory myopathy that causes symmetrical, proximal muscle weakness and affects the skin

Question 26

What causes polymyositis and dermatomyositis?

Answer 26

T cells attacking muscle antigen

Question 27

What are the symptoms of polymyositis?

Answer 27

Proximal muscle weakness - trouble brushing hair Systemic symptoms

Question 28

What are the symptoms of dermatomyositis?

Answer 28

Proximal muscle weakness - trouble brushing hair Systemic symptoms Gottron's sign (purple DIP/PIP etc) Helitrope rash Shawl sign

Question 29

What antibodies are seen in dermatomyositis?

Answer 29

Anti-Jo-1 AntiSRP

Question 30

What is the definitive diagnostic investigation for polymyositis or dermatomyositis?

Answer 30

Muscle biopsy

Question 31

How is polymyositis and dermatomyositis managed?

Answer 31

Steroids initially Immunosuppression - methotrexate or azathioprine IV Igs and rituximab if not responsive to above

Question 32

What is fibromyalgia characterised by?

Answer 32

Muscle pain and generalised fatigue

Question 33

What are some common symptoms of fibromyalgia?

Answer 33

Fatigue Headache Anxiety / depression Brain fog 'Tender when hugged'

Question 34

How is fibromyalgia managed?

Answer 34

Patient education Physiotherapy Occupational therapy

Question 35

What is polymyalgia rheumatica characterised by?

Answer 35

Muscle pain and stiffness

Question 36

What is strongly associated with polymyalgia rheumatica?

Answer 36

Giant cell arteritis

Question 37

What are the common symptoms of polymyalgia rheumatica?

Answer 37

Proximal muscle pain No weakness Morning stiffness for > 1 hour Pitting oedema Systemically unwell Reduced movement of shoulders, neck and hips

Question 38

What is the management for polymyalgia rheumatica?

Answer 38

15mg daily prednisolone