Connective Tissue Disease Flashcards

1
Q

What are connective tissue diseases

A

Autoimmune conditions assoc with spontaneous over activity of the immune system
Doesnt actually affect the CT

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2
Q

What does all CT diseases have in common

A

Specific auto antibodies

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3
Q

Name some CT disease

A
Systemic lupus erythematous 
Sjögren’s syndrome 
Systemic sclerosis 
Dermatomyositis / polymyositis 
Anti - phospholipid syndrome
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4
Q

What are spondyloathropathies

A

Family inflammatory arthritis characterised by involvement of both spine and joints
HLA B27 assoc

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5
Q

What are the 4 main spondyloarthropathies

A

Ankylosing spondylitis
Enteropathic arthritis
Reactive arthritis
Psoriatic arthritis

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6
Q

How does mechanical back pain differ from inflammatory back pain

A

Mechanical - worse by activity, at end of day , better with rest
Inflammatory - worse with rest, better with activity, early morning stiffness >30 mins

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7
Q

What are the other features present in most spondlyoarthropathies

A
Enthesitis 
Inflammatory arthritis 
Dactylitis 
Ocular inflammation 
Mucocutaneous lesions 
Rare aortic incompetence or heart block
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8
Q

What is enthesitis

A

Inflammation at insertion of tendons into bones

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9
Q

What is dactylitis

A

Sausage digits - inflammation of entire digits

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10
Q

What is the treatment of ankylosing spondylitis

A

1st line : NSAIDs
DMARDS
Anti - TNF e.g infliximab

Obv physio

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11
Q

What is inflammatory myopathies characterised

A

Weakness of the muscle

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12
Q

How is polymyalgia rheumatica characterised

A

Pain and stiffness

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13
Q

What is fibromyalgia characterised

A

Pain and fatigue

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14
Q

What is myopathy

A

Disorders of muscles in which muscle fibres don’t function properly

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15
Q

What is vasculitis

A

Inflammation of the blood vessels

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16
Q

What are the classification of vasculitis

A

Large vessel
Medium vessel
Small vessel

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17
Q

What are the large vessel vascualitis

A

Takayasu arteritus, giant cell arteriti

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18
Q

What are the medium vessel vasculitis

A

Poly arteritis nodosa

Kawasaki disease

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19
Q

What age does giant cell affect

A

> 50

20
Q

Features of giant cell

A
Causes temporal arteritis 
Acute, unilateral temporal headache 
Jaw claudication 
Scalp tenderness
Visual disturbances
21
Q

Ix of giant cell

A

ESR
Plasma viscosity
GOLD standard: temporal artery biopsy

22
Q

Management of giant cell

A

40mg prednisolone if no visional effect

60 if there is

23
Q

What are the features of takayasus arteritis

A

< 40

Females

24
Q

What is the main symptoms of takaysus arteritis

A

Claudication
Blood pressure difference ie cant tell pulse on one arm
Bruit ( most commonly being carotid)

25
Q

Ix of takayasus arteritis

A

Angiogram

26
Q

What are the two categories of small vessel vasculitis

A

ANCA - assoc small vessel vasculitis

Non - ANCA assoc small vessel vasulitis

27
Q

What are the ANCA associated small vessel vasculitis

A

Wegener’s granulomatosis
Microscopic polyangitis
Churg Strauss syndrome

28
Q

What are the ANCA non assoc small vessel vasculitis

A

Henoch - schnlein purpura

Serum cryoglobulin

29
Q

Is there granuloma present in microscopic polyangitis

A

No

30
Q

Is there granuloma in churg Strauss syndrome and wegener’s granulomatosis

A

Yes

31
Q

Difference in churg Strauss syndrome

A

Asthma and eosinophilia is present in churgs NOT wegener’s

32
Q

What vasculitis is IgA Dominant immune deposit

A

Henoch schnlein purpura

33
Q

No IgA dominant immune deposit, there is serum cryoglobulin

A

Cryoglobulinemia

34
Q

Old name for wegener’s graulomatosis

A

Granulomatosis with polyangitis ( GPA )

35
Q

Old na,e for Churg - Strauss syndrome

A

Eosinophilic granulomatosis with polyangitis ( EGPA)

36
Q

Old name for microscopic polyangitiis

A

Microscopic polyangitis ( MPA)

37
Q

Who gets wegener’s

A

Males

33-55

38
Q

What is henoch schonlein purpura mediated by

A

Acute immunoglobulin A mediated disorder

= ALWAYS A TRIGGER.. maybe a UTI

39
Q

Management of HSP

A

Self limiting
Symptoms will resolve within 8 weeks
Release may occur
Check urinalysis for renal involvement

40
Q

What is the general ix for vasculitis

A

Urine dipstick ( as kidney involvement is asymptomatic bit life threatening)
Gold standard: tissue biopsy
ANCA, CT, complement level ( will be depleted)

41
Q

Treatment for localised / early systemic ANCA associated vasculitis ( AVV)

A

Methotrexate + steroids

42
Q

Treatment for generalised / systemic

A

1st line: Cyclophosphamide + steroids
Rituximab + steroids ( alternative)
Plasma exchange if creatinine > 500

Followed by azathioprine with alternatives being methotrexate

43
Q

What is localised / early systemic

A

Upper / lower resp tract disease without any other systemic involvements
Without organ involvement or life threatening

44
Q

What is generalised / systemic

A

Renal ( creatinine < 500) or other vital organ failure

45
Q

What is refractory

A

Progressive disease unresponsive to steroids + cyclo

46
Q

Treatment of refractory

A

IV immunoglobulins

Rituximab