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Flashcards in CSIM multi-system disease Deck (94):
1

vit D in sarcoidosis?

raised

2

deficiency in what vit leads to aches and pain?

D

3

where is SLE rash typically sparing?

in the nasal-labial folds

4

diagnostic criteria for SLE

SOAP(clinical) BRAIN(bloods) MD(rash) - 4 of these where at least 1 is clinical

serositis
oral ulcers
arthralgia
photosensitivity

blood disorders
renal disease
a=ANA
immunilogical
neuro - seizures / psychosis

malar
discoid

5

what are the anti-bodies in SLE that are in the diagnostic criteria

ANA

then these are all one criteria
ds DNA
SM
complement
anti-phospholipid

6

what are the anti-bodies in SLE that are in the diagnostic criteria

ANA

then these are all one criteria
ds DNA
SM
complement
anti-phospholipid

7

how can you use APTT in investigating SLE?

if APTT is prolonged it will normally correct if you add plasma to it
in SLE there is lupus anti-coagulant so even when you add plasma the APTT will NOT correct

8

what is the test for lupus anti-coagulant?

dilute russells viper venom time

9

clinically how is SLE different to APLS

APLS is associated with thrombotic events and misscarriage

10

what is livido reticularis and what is it associated with?

wavy mottled rash on legs

SLE
APLS
RA

11

most common CT disease?

SLE

12

pathophys of SLE?

immune response to self antigen

immune complexes form leading to
inflammation
complement activation

13

what are the 6 symptoms of CTD that should always be asked about?

from top to bottom:

1. hair loss
2. dry eyes
3. mouth ulcers
4. rashes (photosensitive)
5. Raynauds
6. joint pain

14

what is Jaccoud's arthropathy?

seen in the hands:

normal at rest
swan necking on flexion

15

test for dry eyes?

Schirers tear test

16

most significant complication in congenital lupus?

heart block

17

what is APLS?

anti-phospholipid syndrome:

acquired autoimmune disorder that manifests clinically as recurrent venous or arterial thrombosis and/or foetal loss.

18

diagnostic criteria for ALPS?

one from lab and one clinical

CLINICAL
pregnancy:
- late term (>10wks) misscarriage
- severely prem (<34)wks delivery due to severe pre eclampsia
- 3 consecutive, unexplained miscarriages before 10 weeks

Vascular thrombosis:
- one or more episodes of arterial, venous or small vessel thrombosis.

LAB : on 2 occasions at least 12 weeks apart
- lupus anti-coag.
- aCL
- anti-Beta2-glycoprotein I

19

what pattern of rashes do we see in CTD?

lichenoid
vasculopathic

20

where does thrombocytopenic purpura present/ why?

in the legs

thats where pressure is highest

21

iatrogenic cause of purpura?

steroids

22

why blistering in vasculitis?

proteins that hold the epidermis to the dermis let go when it dies

23

how does the rash in vasculitis change as it progresses?

ulceration

24

what does livedo mean in latin?

net (thats why its a wavy shaped rash)

like when you get cold

25

difference between vasculitic and thrombotic rashes?

in thrombosis there is no time for it to rise so it's FLAT

26

which anti body is associated with necrotizing vasculitis?

ANCA, pANCA or cANCA

systemic

27

two important Ix in systemic GPA?

bronchoscopy
renal biopsy

28

what is Henoch-Schönlein purpura (HSP)?

Immune complex deposition disease
DEFINITION OF leucocytoclastic vasculitis - small vessel, neutrophilis with extruded nuclei

29

epidemiology of GCA?

Commonest vasculitis (UK)
Age > 50 (usually in 70’s)
Female 2:1 Male

30

two types of vasculitis that effect the large vessels

Takayasu Arteritis (rare in the UK, common in Japan)
Giant Cell Arteritis

31

two types of vasculitis that effect the medium vessels?

polyartritis nodosa
Kawasaki disease

32

what are the most common types of vasculitis in adults and children?

adults - GCA
children - Henoch- Schönlein (IgA)

33

Takayasu vasc effects who?

WOMEN
children up to 40
Japanese

34

effect of cushings on the skin?

STEROIDS

thins the skin so get striae and can see vessels through skin

acne

35

effect of Addisons on the skin? what about in primary adrenal failure?

ACTH stimulates melanocytes so get PIGMMENTATION

classically in the palmar creases and buccal muscosa

opposite if prmary as ACTH is LOW

36

thyroid disease skin changes

HYPO
proptosis -fat around eyes
loss of outer third of eye brows

HYPER
pre-tibial myxedema
sweat -> sweat rash

association with alopecia and vitiligo

37

how does liver disease show in the skin?

porphyria- blisters on the back of the hands with scaring

38

how do lipid abnormalities present?

xanthelasma- macrophages eat the lipids

39

types of xantholasma and what they indicate?

Xanthelasma
-Normal/several dyslipidaemias
Tendon xanthomas
-Familial hypercholesterolaemia
Eruptive xanthomas
-Hypertriglyceridaemia

40

skin changes in T1DM

Acanthosis nigricans
Also occurs in obesity
Necrobiosis lipoidica - granulomatous skin on tibia
Infections eg candida

41

skin changes in gout?

Tophi
periarticular urate - looks like RA

42

what does a blue edged ulcer make you think?

haem malignancies -> Pyoderma Gangrenosum

also seen in IBD

43

how can lymphoma present on the skin?

Cutaneous T cell lymphoma - often confused with psoriasis or eczema

44

what are koplic spots and what causes them?

blueish spots on buccal mucosa
measles

45

umbilicated blisters all the same size think what?

zoster

46

skin changes in syphilis?

Primary - Chancre
Secondary - 6 weeks later
palms, soles, generalised
Snail-track ulcers
Gumma- ulcers in bone, skin

47

rash in Lyme disease?

called erythema migrans - red ring rash ripples out over weeks

48

what is erythema nodosum?

inflammation of fat - seen in IBD, strep. , TB, sarcoidosis

49

skin in IBD?

pyoderma gangrenosum

erytherma nodosum

mouth ulcers

50

reiters syndrome triad? and other symptom

conjunc
urethritis
arthritis

psoriasis

51

what is pathergy? when is it seen?

when you get pyoderma due t puncturing the skin
seen in Behcet's syndrome

52

skin changes in systemic sclerosis?

thickening - hands tends to claws, mouth is tight, beaking nose

53

another name for systemic sclerosis and the symptoms?

CREST syndrome

Calcification
Raynauds
Eosophagitis
Sclerodactyly
Telangiectasia

54

two paraneoplastic skin changes?

Acanthosis nigricans
Dermatomyositis

55

best marker in the blood for dermatomyositis?

CK elevation

56

causes of raised CK

Inflammatory muscle disease
Polymyositis
dermatomyositis
muscular dystrophies
rhabdomyolysis
Motor neurone disease
Iatrogenic – statin induced myositis

57

examination findings in GCA

absent pulses or bruits
tender temporal arteries

58

Ix for diagnosis of GCA?

biopsy

59

clinical corse of Takayasus?

inflammatory phase - may progress
ischemic phase - claudication

60

how does medium vessel vasculitis present differently to large?

orchitis
acute abdo
GI bleeding / ichaemia

61

who does Kawasaki occur in?

young children

62

symptoms of Kawasaki?

fever >5 days
♣ Rash
♣ Swollen glands in the neck
♣ Dry, cracked lips
♣ Red fingers or toes
♣ Red eyes

63

Ix to screen for complication of Kawasaki?

echo for aortic aneurysm

64

4 types of small vessel vasculitis?

Granulomatosis with Polyangiitis (GPA) – Wegener’s Granulomatosis
Eosnophilic granulomatosis with polyangiitis (EGPA) – Churg Strauss Syndrome
Microscopic polyangiitis (MPA)
Henoch-Scholein purpura (HSP)

65

systemic presentation of small vessel vasc?

• Alveolar haemorrhage
• Glomerulonephritis
• Scleritis
• Mononeuritis multiplex

66

what facial deformity is seen in small vessel vasc?

saddle nose deformity

67

which antibodies are most closely associate with CTD?

ANA - bind to the nucleus

very sensitive but not very specific

68

developing Raynauds when youre older is more or less associate with CTD?

more associated

having it when youre young is better

69

2 types of scleroderma and how they present?

localised - in skin and dermis bands of skin that are thickened

systemic - skin and organs
- digital ulceration
- renal vessel involvement - > htp
- pulmonary involvement -> pul. hpt

eventually skin becomes very tight

70

how does limited scleroderma present?

CREST syndrome

calcification
raynauds
esophageal (GI in general)
sclerodactly
telangectasia

71

what antibody associated with limited scleroderma?

anti-centromere

72

Ix to monitor the progession of scleroderma?

renal
PFT
weigh
ECHO

73

what is the main risk in Primary Sjogrens syndrome

lymphoma

74

presentation of PSS?

FEMALES in 60s:
- fatigue
- dry eyes
- dry mouth
- dry vagina
- parotid gland swelling

75

inflam. factors in PSS?

LOW CRP
high ESR

76

association with myalgia with muscle enzymes?

NONE

77

what is raised in myositis?

AST, ALT, LDH, CK

78

skin changes in dermatomyositis?

Gottrans papules - red papules on the knuckles
flagelate - lines

79

what anti-body is mixed connective tissue disease associated with?

RNP

80

Ix in sero-neg disease?

HLA-B27 +
Rheumatoid factor negative
ESR/CRP
X-rays
Hands and feet
Sacro-iliac joints (SIJ)
USS small joints
MRI whole spine and SIJ

81

what is sarcoidosis?

multi system disease characterised by epitheliod non-caseating granuloma in involved organs

82

where is sarcoidosis mostly seen?

lungs 95%
also skin, lymph nodes and everywhere else

83

signs and symptoms of sarcoidosis in the lungs?

signs - normally none (wheeze and fine crackles rarely)

symptoms - dyspnoea, cough, chest pain

84

characteristics of acute arthritis in sarcoidosis

Oligoarthritis
Large & small joint
Lower limb > upper limb

85

what is Lofgrens syndrome and what is it's triad?

acute and benign form of sarcoid

Erythema Nodosum
Arthralgia/ arthritis (Bilateral ankle)
Bilateral Hilar Adenopathy

86

what is erythema nodosum?

acute, nodular, erythematous eruption that usually is limited to the extensor aspects of the lower legs

87

what annual investigations in sarcoidosis?

ECG and ECHO due to heart block risk

88

other that lofgrens syndrome, what is the classical way sarcoidosis presents?

uveoparodit fever:

uveitis
parotitis
fever
CNVII palsy

89

what are the nodes like in lympoma?

large no. of FIRM lymph nodes

90

what CTD is associated with lymphoma?

Sjogrens

91

what are the lymph nodes like in reactive lymphadenopathy ?

soft, mobile, only a couple

92

abnormal bloods in myositis?

raised CK
raised AST
raised LDH

93

most diagnostic Ix for GCA?

biopsy

however this can give false neg as GCA is patchy stuff

94

presentation of HSP?

rash on legs / buttocks - palpable purpura (IgA deposited in places

swelling in wrists, knees, ankles

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