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CP3 MDD > Multisystem connective tissue disease > Flashcards

Multisystem connective tissue disease Flashcards

1
Q

Who is affected by SLE?

A

Young/middle aged Asian F

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2
Q

Cause SLE?

A

Unknown

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3
Q

Skin manifestations SLE (4)

A

Chronic discoid LE
Malar rash
Photosensitivity rash
Alopecia

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4
Q

Oral manifestations SLE (2)

A

Oral mucosal lesions

Ulcers

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5
Q

CNS manifestations SLE (3)

A

Depression/anxiety
Grand mal seizures
Vascular occlusion/infarction

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6
Q

Renal manifestations SLE (2)

A

Glomerular disease

Interstitial nephritis

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7
Q

Haematological manifestations SLE (3)

A

Thrombocytopaenia
Leukopaenia
Anaemia

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8
Q

MSK manifestations SLE (2)

A

Arthritis - fingers, wrists, knees

Myalgia

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9
Q

Lung manifestations SLE (2)

A

Pleural effusion

Pulmonary fibrosis

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10
Q

Cardiac manifestations SLE (3)

A

Pericarditis
Aortic valve
Cardiomyopathy

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11
Q

Ix SLE (8)

A 
FBC
ESR/CRP
U+E
Serum ANA 
Anti-dsDNA
RNA antibodies 
Se complement levels (C3/4) 
Urine dip
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12
Q

Which marker in SLE is specific but not sensitive?

A

anti-dsDNA

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13
Q

Mx mild SLE (4)

A

NSAIDS
Hydroxychloroquine
Monitor signs infection
Avoid XS sunlight + CV RF

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14
Q

Mx severe SLE (5)

A 
Prednisolone 
Azathioprine 
Hx/Exam 
Monitor for signs active disease 
FBC,U+E,C3/4 + urine dip
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15
Q

What is APLS

A

Presence of autoantibodies towards phospholipids

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16
Q

What other autoimmune syndrome does APLS relate to?

A

SLE

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17
Q

PS APLS (7)

A 
PE 
Aa thrombosis 
Miscarriage - 2nd/3rd trimester 
Livedo reticularis 
Thrombocytopenia 
Migraine 
Epilepsy
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18
Q

Ix APLS (5)

A 
ESR - 
ANA -ve
APPT incr
Coombs +ve
Anticardiolipin
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19
Q

WHich is the diagnostic test for APLS

A

Anticardiolipin (12 w apart)

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20
Q

Mx APLS - w/ Hx severe thrombosis

A

Warfarin

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21
Q

Mx APLS - w/o Hx severe thrombosis

A

Low dose aspirin

Lifestyle advice

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22
Q

LT prognosis APLS

A

1/3 have organ damage within 10 y

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23
Q

What are the 2 main types of sclerosis

A

Limited cutaneous scleroderma

Diffuse cutaneous scleroderma

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24
Q

What other condition do pt w/ Limited cutaneous scleroderma have a LONG Hx of

A

Raynaud’s (+ skin tightening)

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25
Q

Early Sx Limited cutaneous scleroderma (3)

A

Fatigue
GORD
Ulcers on digits

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26
Q

Later Sx Limited cutaneous scleroderma (2)

A

Oseoph strictures

Pulmonary fibrosis

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27
Q

CREST syndrome

A 
Calcinosis 
Raynaud's 
Oesophageal dysmotility 
Sclerodactyly
Tenangiectasia
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28
Q

Mx Limited cutaneous scleroderma

A

Digital vasodilators
Removal of calcinosis
Tx oesoph problems

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29
Q

What do people w/ Diffuse Cutaneous Scleroderma have a SHORT hx of

A

Raynauds

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30
Q

Sx Diffuse Cutaneous Scleroderma (3)

A

Lethargy
Decr W
Anorexia

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31
Q

What complications will people w Diffuse Cutaneous Scleroderma have within 3years? (3)

A

Myocardial fibrosis
Pulmonary fibrosis
Renal fibrosis

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32
Q

Mx Diffuse Cutaneous Scleroderma (2)

A

Immunosuppression

Vasodilators

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33
Q

Ix limited cutaneous scleroderma

A

Anti-centromere antibody +ve

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34
Q

Ix diffuse cutaneous scleroderma

A

Anti-scl-70 antibodies

RNA polymerase antibodies

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35
Q

Which condition has the highest mortality rate of any autoimmune rheumatic disease?

A

systemic sclerosis

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36
Q

1’ Sjogrens

A

Dry eyes

Dry mouth

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37
Q

2’ Sjogrens

A

Sicca Sx

+ autoimmune disease

38
Q

Which autoimmune diseases are associated w/ 2’ SJOGRENS (4)

A

RA
SLE
Scleroderma
Polymyositis

39
Q

Test Sjogrens

A

Schirmer

<1cm 5 mins indicates defective tear prod

40
Q

Blood results Sjogrens (4)

A

RF incr
ANA incr
Anti-RO +ve
Anti-LA +ve

41
Q

Mx Sjogrens

A

Artificial tears + saliva replacement

42
Q

Genetic marker polymyositis

A

HLA-B8/DR3

43
Q

What is polymyositis

A

Inflammation of striated muscle –> proximal mm weakness

44
Q

Sx polymyositis (5)

A 
Weakness in absence pain 
Assoc mm wasting 
Insidious/acute onset 
Assoc malaise, wt loss + fever
Difficulty squatting or climbing stairs
45
Q

What can happen if polymyositis is left untreated?

A

Resp mm involvement –> resp failure

46
Q

Antibodies Dermatomyositis

A

HLA B8/DR3

47
Q

What is dermatomyositis

A

Polymyositis + skin involvement

48
Q

What 2 rashes are classical in dermatomyositis

A

Heliotropic rash of eyes

Gottrons papules over knuckles

49
Q

Which conditions are dermato + myositis assoc w/ (4)

A

SLE
RA
Sclerosis
Malig

50
Q

Ix dermato/myositis (8(

A 
Serum CK
ESR
ANA +ve 
RF 
HLAB8/DR3
EMG
MRI - abnorm mm 
Needle biospy - fibre necrosis
51
Q

What % patients w/ dermato/myositis = RF + ve

A

50%

52
Q

Mx dermato/myositis

A

Prednisolone

DMARDS

53
Q

Who is affected by inclusion body myositis?

A

White males >50

54
Q

Sx inclusion body myositis

A

Insidious onset - progressive proximal + distal mm wasting

55
Q

Ix inclusion body myositis (3)

A

ANA can be +ve
Myositis antigens (HLA-B8/DR3) -ve
Mm biopsy - inflam infiltrate + vacuoles + B-amyloid

56
Q

Inclusion body myositis - response to pred/DMARDS

A

Poor

57
Q

What % of pt w/ Polymyalgia rheumatic go on to develop GCA?

A

13-30%

58
Q

What % of pt w/ GCA go on to develop PMR?

A

50-70%

59
Q

What is polymyalgia rheumatica

A

Mild non-erosive synovial + periarticular inflammation of proximal joints

60
Q

Age of Who gets PMR?

A

> 50y/o

61
Q

Sx PMR (7)

A 
Sudden onset severe pain + stiffness 
In neck/shoulders/hips/spine
Worse in AM 
Lasts >30 mins 
\+ Systemic - tiredness, fever, W loss
62
Q

O/E PMR

A

Mm are tender to palpate

63
Q

Diagnosis PMR made by…

A

Bilat shoulder/pelvic girdle pain
Acute phase response
>2W
+ assess for GCA

64
Q

Ix PMR (4)

A

Raised ESR/CRP
Normochromic anaemia
Platelets - incr
RF/ANA/Anti-CCP -ve

65
Q

Mx PMR (3)

A

15mg prednisolone 2w
Then reduce 5-7/day maintainence 6m
Consider specialist Rx

66
Q

Giant Cell Arteritis

A

Inflammation infiltrates accumulate in medium + large aa –> granulomatous vasculitis –> ischaemia

67
Q

Sx GCA (5)

A 
Severe abrupt onset headaches 
Scalp/temporal tenderness 
Jaw claudication 
SLOV painless 
O/E - tenderness/swelling temple
68
Q

Ix GCA (6)

A 
Anaemia on FBC
Raised ESR/CRP
Raised ALP/GGT
RF/ANA/Anti-CCP -ve
CK -ve 
TEMPORAL AA BIOPSY for defintive diagnosis
69
Q

Mx GCA (4)

A

PO pred (60mg if visual Sx, 40mg if not)
Should respond to steroids rapidly
5 years
Monitor for large vessel involvement

70
Q

General Sx vasculitis (5)

A 
Malaise
Weight loss 
Fever
Myalgia 
Arthalgia
71
Q

Skin Sx vasculitis (2)

A

Purpura

Ulcers

72
Q

GI Sx vasculitis (3)

A

Mouth ulcers
Abdo pain
Diarrhoea

73
Q

Resp Sx vascultis (2)

A

Haemoptysis

SOB

74
Q

ENT Sx vasculitis (2)

A

Epistaxis

Crusting

75
Q

Cardiac Sx vasculitis (1)

A

Chest pain

76
Q

Vasculitis of the large aa (2)

A

GCA

Takayasu

77
Q

What is Takayasu

A

Young adults w/ upper limb claudication + stroke

78
Q

Vasculitis of the medium aa (2)

A

Polyarteritis nodosa

Kawasaki

79
Q

What is polyarteritis nodosa

A

Multisystemic Sx + microaneurysms

80
Q

Vascultis of med-small aa (3)

A

All ANCA +
Wengers granulomatosis
Churg-strauss
Microscopic polyangitis

81
Q

Which organs does Wenger’s grandulomatosis effect? (3)

A

Lung
Kidney
ENT

82
Q

PS Churg-Strauss

A

Late onset asthma

+ Cardiopulmonary involvement

83
Q

PS Microscopic polyangitis

A

Pulmonary renal syndrome

Haemoptysis + haematuria

84
Q

Vasculitis of the small aa (2)

A

HSP

Cryoglobinaemia

85
Q

What is cryoglobinaemia

A

Hep C
Rash
Arthralgia

86
Q

Ix vasculitis (5)

A 
FBC
LFT
inflammatory markers 
Immunology 
Tissue biopsy
87
Q

C-ANCA

A

Wegners

88
Q

P-AANCA (2)

A

Churg Strauss

Microscopic polyangitis

89
Q

Mx vasculitis

A

CCS = mainstay
If ANCA +VE - cyclophosphamide
IVIG (KAwasaki)

90
Q

Mx life threatening vasculitis

A

Plasma exchange

CP3 MDD (13 decks)

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