Multisystem connective tissue disease Flashcards
(90 cards)
1
Q
Who is affected by SLE?
A
Young/middle aged Asian F
2
Q
Cause SLE?
A
Unknown
3
Q
Skin manifestations SLE (4)
A
Chronic discoid LE
Malar rash
Photosensitivity rash
Alopecia
4
Q
Oral manifestations SLE (2)
A
Oral mucosal lesions
Ulcers
5
Q
CNS manifestations SLE (3)
A
Depression/anxiety
Grand mal seizures
Vascular occlusion/infarction
6
Q
Renal manifestations SLE (2)
A
Glomerular disease
Interstitial nephritis
7
Q
Haematological manifestations SLE (3)
A
Thrombocytopaenia
Leukopaenia
Anaemia
8
Q
MSK manifestations SLE (2)
A
Arthritis - fingers, wrists, knees
Myalgia
9
Q
Lung manifestations SLE (2)
A
Pleural effusion
Pulmonary fibrosis
10
Q
Cardiac manifestations SLE (3)
A
Pericarditis
Aortic valve
Cardiomyopathy
11
Q
Ix SLE (8)
A
FBC ESR/CRP U+E Serum ANA Anti-dsDNA RNA antibodies Se complement levels (C3/4) Urine dip
12
Q
Which marker in SLE is specific but not sensitive?
A
anti-dsDNA
13
Q
Mx mild SLE (4)
A
NSAIDS
Hydroxychloroquine
Monitor signs infection
Avoid XS sunlight + CV RF
14
Q
Mx severe SLE (5)
A
Prednisolone Azathioprine Hx/Exam Monitor for signs active disease FBC,U+E,C3/4 + urine dip
15
Q
What is APLS
A
Presence of autoantibodies towards phospholipids
16
Q
What other autoimmune syndrome does APLS relate to?
A
SLE
17
Q
PS APLS (7)
A
PE Aa thrombosis Miscarriage - 2nd/3rd trimester Livedo reticularis Thrombocytopenia Migraine Epilepsy
18
Q
Ix APLS (5)
A
ESR - ANA -ve APPT incr Coombs +ve Anticardiolipin
19
Q
WHich is the diagnostic test for APLS
A
Anticardiolipin (12 w apart)
20
Q
Mx APLS - w/ Hx severe thrombosis
A
Warfarin
21
Q
Mx APLS - w/o Hx severe thrombosis
A
Low dose aspirin
Lifestyle advice
22
Q
LT prognosis APLS
A
1/3 have organ damage within 10 y
23
Q
What are the 2 main types of sclerosis
A
Limited cutaneous scleroderma
Diffuse cutaneous scleroderma
24
Q
What other condition do pt w/ Limited cutaneous scleroderma have a LONG Hx of
A
Raynaud’s (+ skin tightening)
25
Early Sx Limited cutaneous scleroderma (3)
Fatigue
GORD
Ulcers on digits
26
Later Sx Limited cutaneous scleroderma (2)
Oseoph strictures
| Pulmonary fibrosis
27
CREST syndrome
```
Calcinosis
Raynaud's
Oesophageal dysmotility
Sclerodactyly
Tenangiectasia
```
28
Mx Limited cutaneous scleroderma
Digital vasodilators
Removal of calcinosis
Tx oesoph problems
29
What do people w/ Diffuse Cutaneous Scleroderma have a SHORT hx of
Raynauds
30
Sx Diffuse Cutaneous Scleroderma (3)
Lethargy
Decr W
Anorexia
31
What complications will people w Diffuse Cutaneous Scleroderma have within 3years? (3)
Myocardial fibrosis
Pulmonary fibrosis
Renal fibrosis
32
Mx Diffuse Cutaneous Scleroderma (2)
Immunosuppression
| Vasodilators
33
Ix limited cutaneous scleroderma
Anti-centromere antibody +ve
34
Ix diffuse cutaneous scleroderma
Anti-scl-70 antibodies
| RNA polymerase antibodies
35
Which condition has the highest mortality rate of any autoimmune rheumatic disease?
systemic sclerosis
36
1' Sjogrens
Dry eyes
| Dry mouth
37
2' Sjogrens
Sicca Sx
| + autoimmune disease
38
Which autoimmune diseases are associated w/ 2' SJOGRENS (4)
RA
SLE
Scleroderma
Polymyositis
39
Test Sjogrens
Schirmer
| <1cm 5 mins indicates defective tear prod
40
Blood results Sjogrens (4)
RF incr
ANA incr
Anti-RO +ve
Anti-LA +ve
41
Mx Sjogrens
Artificial tears + saliva replacement
42
Genetic marker polymyositis
HLA-B8/DR3
43
What is polymyositis
Inflammation of striated muscle --> proximal mm weakness
44
Sx polymyositis (5)
```
Weakness in absence pain
Assoc mm wasting
Insidious/acute onset
Assoc malaise, wt loss + fever
Difficulty squatting or climbing stairs
```
45
What can happen if polymyositis is left untreated?
Resp mm involvement --> resp failure
46
Antibodies Dermatomyositis
HLA B8/DR3
47
What is dermatomyositis
Polymyositis + skin involvement
48
What 2 rashes are classical in dermatomyositis
Heliotropic rash of eyes
| Gottrons papules over knuckles
49
Which conditions are dermato + myositis assoc w/ (4)
SLE
RA
Sclerosis
Malig
50
Ix dermato/myositis (8(
```
Serum CK
ESR
ANA +ve
RF
HLAB8/DR3
EMG
MRI - abnorm mm
Needle biospy - fibre necrosis
```
51
What % patients w/ dermato/myositis = RF + ve
50%
52
Mx dermato/myositis
Prednisolone
| DMARDS
53
Who is affected by inclusion body myositis?
White males >50
54
Sx inclusion body myositis
Insidious onset - progressive proximal + distal mm wasting
55
Ix inclusion body myositis (3)
ANA can be +ve
Myositis antigens (HLA-B8/DR3) -ve
Mm biopsy - inflam infiltrate + vacuoles + B-amyloid
56
Inclusion body myositis - response to pred/DMARDS
Poor
57
What % of pt w/ Polymyalgia rheumatic go on to develop GCA?
13-30%
58
What % of pt w/ GCA go on to develop PMR?
50-70%
59
What is polymyalgia rheumatica
Mild non-erosive synovial + periarticular inflammation of proximal joints
60
Age of Who gets PMR?
>50y/o
61
Sx PMR (7)
```
Sudden onset severe pain + stiffness
In neck/shoulders/hips/spine
Worse in AM
Lasts >30 mins
+ Systemic - tiredness, fever, W loss
```
62
O/E PMR
Mm are tender to palpate
63
Diagnosis PMR made by...
Bilat shoulder/pelvic girdle pain
Acute phase response
>2W
+ assess for GCA
64
Ix PMR (4)
Raised ESR/CRP
Normochromic anaemia
Platelets - incr
RF/ANA/Anti-CCP -ve
65
Mx PMR (3)
15mg prednisolone 2w
Then reduce 5-7/day maintainence 6m
Consider specialist Rx
66
Giant Cell Arteritis
Inflammation infiltrates accumulate in medium + large aa --> granulomatous vasculitis --> ischaemia
67
Sx GCA (5)
```
Severe abrupt onset headaches
Scalp/temporal tenderness
Jaw claudication
SLOV painless
O/E - tenderness/swelling temple
```
68
Ix GCA (6)
```
Anaemia on FBC
Raised ESR/CRP
Raised ALP/GGT
RF/ANA/Anti-CCP -ve
CK -ve
TEMPORAL AA BIOPSY for defintive diagnosis
```
69
Mx GCA (4)
PO pred (60mg if visual Sx, 40mg if not)
Should respond to steroids rapidly
5 years
Monitor for large vessel involvement
70
General Sx vasculitis (5)
```
Malaise
Weight loss
Fever
Myalgia
Arthalgia
```
71
Skin Sx vasculitis (2)
Purpura
| Ulcers
72
GI Sx vasculitis (3)
Mouth ulcers
Abdo pain
Diarrhoea
73
Resp Sx vascultis (2)
Haemoptysis
| SOB
74
ENT Sx vasculitis (2)
Epistaxis
| Crusting
75
Cardiac Sx vasculitis (1)
Chest pain
76
Vasculitis of the large aa (2)
GCA
| Takayasu
77
What is Takayasu
Young adults w/ upper limb claudication + stroke
78
Vasculitis of the medium aa (2)
Polyarteritis nodosa
| Kawasaki
79
What is polyarteritis nodosa
Multisystemic Sx + microaneurysms
80
Vascultis of med-small aa (3)
All ANCA +
Wengers granulomatosis
Churg-strauss
Microscopic polyangitis
81
Which organs does Wenger's grandulomatosis effect? (3)
Lung
Kidney
ENT
82
PS Churg-Strauss
Late onset asthma
| + Cardiopulmonary involvement
83
PS Microscopic polyangitis
Pulmonary renal syndrome
| Haemoptysis + haematuria
84
Vasculitis of the small aa (2)
HSP
| Cryoglobinaemia
85
What is cryoglobinaemia
Hep C
Rash
Arthralgia
86
Ix vasculitis (5)
```
FBC
LFT
inflammatory markers
Immunology
Tissue biopsy
```
87
C-ANCA
Wegners
88
P-AANCA (2)
Churg Strauss
| Microscopic polyangitis
89
Mx vasculitis
CCS = mainstay
If ANCA +VE - cyclophosphamide
IVIG (KAwasaki)
90
Mx life threatening vasculitis
Plasma exchange
