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4TH YEAR > CSIM multi-system disease > Flashcards

CSIM multi-system disease Flashcards

1
Q

vit D in sarcoidosis?

A

raised

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2
Q

deficiency in what vit leads to aches and pain?

A

D

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3
Q

where is SLE rash typically sparing?

A

in the nasal-labial folds

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4
Q

diagnostic criteria for SLE

A

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

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5
Q

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

A

ANA

then these are all one criteria
ds DNA
SM
complement
anti-phospholipid
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6
Q

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

A

ANA

then these are all one criteria
ds DNA
SM
complement
anti-phospholipid
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7
Q

how can you use APTT in investigating SLE?

A

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

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8
Q

what is the test for lupus anti-coagulant?

A

dilute russells viper venom time

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9
Q

clinically how is SLE different to APLS

A

APLS is associated with thrombotic events and misscarriage

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10
Q

what is livido reticularis and what is it associated with?

A

wavy mottled rash on legs

SLE
APLS
RA

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11
Q

most common CT disease?

A

SLE

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12
Q

pathophys of SLE?

A

immune response to self antigen

immune complexes form leading to
inflammation
complement activation

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13
Q

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

A

from top to bottom:

  1. hair loss
  2. dry eyes
  3. mouth ulcers
  4. rashes (photosensitive)
  5. Raynauds
  6. joint pain
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14
Q

what is Jaccoud’s arthropathy?

A

seen in the hands:

normal at rest
swan necking on flexion

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15
Q

test for dry eyes?

A

Schirers tear test

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16
Q

most significant complication in congenital lupus?

A

heart block

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17
Q

what is APLS?

A

anti-phospholipid syndrome:

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

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18
Q

diagnostic criteria for ALPS?

A

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
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19
Q

what pattern of rashes do we see in CTD?

A

lichenoid

vasculopathic

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20
Q

where does thrombocytopenic purpura present/ why?

A

in the legs

thats where pressure is highest

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21
Q

iatrogenic cause of purpura?

A

steroids

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22
Q

why blistering in vasculitis?

A

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

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23
Q

how does the rash in vasculitis change as it progresses?

A

ulceration

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24
Q

what does livedo mean in latin?

A

net (thats why its a wavy shaped rash)

like when you get cold

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25
Q

difference between vasculitic and thrombotic rashes?

A

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

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26
Q

which anti body is associated with necrotizing vasculitis?

A

ANCA, pANCA or cANCA

systemic

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27
Q

two important Ix in systemic GPA?

A

bronchoscopy

renal biopsy

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28
Q

what is Henoch-Schönlein purpura (HSP)?

A

Immune complex deposition disease

DEFINITION OF leucocytoclastic vasculitis - small vessel, neutrophilis with extruded nuclei

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29
Q

epidemiology of GCA?

A

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

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30
Q

two types of vasculitis that effect the large vessels

A 
Takayasu Arteritis (rare in the UK, common in Japan)
Giant Cell Arteritis
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31
Q

two types of vasculitis that effect the medium vessels?

A

polyartritis nodosa

Kawasaki disease

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32
Q

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

A

adults - GCA

children - Henoch- Schönlein (IgA)

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33
Q

Takayasu vasc effects who?

A

WOMEN
children up to 40
Japanese

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34
Q

effect of cushings on the skin?

A

STEROIDS

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

acne

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35
Q

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

A

ACTH stimulates melanocytes so get PIGMMENTATION

classically in the palmar creases and buccal muscosa

opposite if prmary as ACTH is LOW

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36
Q

thyroid disease skin changes

A

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

HYPER
pre-tibial myxedema
sweat -> sweat rash

association with alopecia and vitiligo

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37
Q

how does liver disease show in the skin?

A

porphyria- blisters on the back of the hands with scaring

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38
Q

how do lipid abnormalities present?

A

xanthelasma- macrophages eat the lipids

39
Q

types of xantholasma and what they indicate?

A 
Xanthelasma
   -Normal/several dyslipidaemias
Tendon xanthomas
   -Familial hypercholesterolaemia
Eruptive xanthomas
   -Hypertriglyceridaemia
40
Q

skin changes in T1DM

A

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

41
Q

skin changes in gout?

A

Tophi

periarticular urate - looks like RA

42
Q

what does a blue edged ulcer make you think?

A

haem malignancies -> Pyoderma Gangrenosum

also seen in IBD

43
Q

how can lymphoma present on the skin?

A

Cutaneous T cell lymphoma - often confused with psoriasis or eczema

44
Q

what are koplic spots and what causes them?

A

blueish spots on buccal mucosa

measles

45
Q

umbilicated blisters all the same size think what?

A

zoster

46
Q

skin changes in syphilis?

A 
Primary - Chancre
Secondary - 6 weeks later
  palms, soles, generalised
  Snail-track ulcers
Gumma- ulcers in bone, skin
47
Q

rash in Lyme disease?

A

called erythema migrans - red ring rash ripples out over weeks

48
Q

what is erythema nodosum?

A

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

49
Q

skin in IBD?

A

pyoderma gangrenosum

erytherma nodosum

mouth ulcers

50
Q

reiters syndrome triad? and other symptom

A

conjunc
urethritis
arthritis

psoriasis

51
Q

what is pathergy? when is it seen?

A

when you get pyoderma due t puncturing the skin

seen in Behcet’s syndrome

52
Q

skin changes in systemic sclerosis?

A

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

53
Q

another name for systemic sclerosis and the symptoms?

A

CREST syndrome

Calcification
Raynauds
Eosophagitis 
Sclerodactyly
Telangiectasia
54
Q

two paraneoplastic skin changes?

A

Acanthosis nigricans

Dermatomyositis

55
Q

best marker in the blood for dermatomyositis?

A

CK elevation

56
Q

causes of raised CK

A 
Inflammatory muscle disease
  Polymyositis
  dermatomyositis
muscular dystrophies
rhabdomyolysis
Motor neurone disease
Iatrogenic – statin induced myositis
57
Q

examination findings in GCA

A

absent pulses or bruits

tender temporal arteries

58
Q

Ix for diagnosis of GCA?

A

biopsy

59
Q

clinical corse of Takayasus?

A

inflammatory phase - may progress

ischemic phase - claudication

60
Q

how does medium vessel vasculitis present differently to large?

A

orchitis
acute abdo
GI bleeding / ichaemia

61
Q

who does Kawasaki occur in?

A

young children

62
Q

symptoms of Kawasaki?

A 
fever >5 days
♣	Rash
♣	Swollen glands in the neck
♣	Dry, cracked lips
♣	Red fingers or toes
♣	Red eyes
63
Q

Ix to screen for complication of Kawasaki?

A

echo for aortic aneurysm

64
Q

4 types of small vessel vasculitis?

A

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

65
Q

systemic presentation of small vessel vasc?

A
  • Alveolar haemorrhage
  • Glomerulonephritis
  • Scleritis
  • Mononeuritis multiplex
66
Q

what facial deformity is seen in small vessel vasc?

A

saddle nose deformity

67
Q

which antibodies are most closely associate with CTD?

A

ANA - bind to the nucleus

very sensitive but not very specific

68
Q

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

A

more associated

having it when youre young is better

69
Q

2 types of scleroderma and how they present?

A

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
Q

how does limited scleroderma present?

A

CREST syndrome

calcification
raynauds
esophageal (GI in general)
sclerodactly
telangectasia
71
Q

what antibody associated with limited scleroderma?

A

anti-centromere

72
Q

Ix to monitor the progession of scleroderma?

A

renal
PFT
weigh
ECHO

73
Q

what is the main risk in Primary Sjogrens syndrome

A

lymphoma

74
Q

presentation of PSS?

A

FEMALES in 60s:

  • fatigue
  • dry eyes
  • dry mouth
  • dry vagina
  • parotid gland swelling
75
Q

inflam. factors in PSS?

A

LOW CRP

high ESR

76
Q

association with myalgia with muscle enzymes?

A

NONE

77
Q

what is raised in myositis?

A

AST, ALT, LDH, CK

78
Q

skin changes in dermatomyositis?

A

Gottrans papules - red papules on the knuckles

flagelate - lines

79
Q

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

A

RNP

80
Q

Ix in sero-neg disease?

A 
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
Q

what is sarcoidosis?

A

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

82
Q

where is sarcoidosis mostly seen?

A

lungs 95%

also skin, lymph nodes and everywhere else

83
Q

signs and symptoms of sarcoidosis in the lungs?

A

signs - normally none (wheeze and fine crackles rarely)

symptoms - dyspnoea, cough, chest pain

84
Q

characteristics of acute arthritis in sarcoidosis

A

Oligoarthritis
Large & small joint
Lower limb > upper limb

85
Q

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

A

acute and benign form of sarcoid

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

86
Q

what is erythema nodosum?

A

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

87
Q

what annual investigations in sarcoidosis?

A

ECG and ECHO due to heart block risk

88
Q

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

A

uveoparodit fever:

uveitis
parotitis
fever
CNVII palsy

89
Q

what are the nodes like in lympoma?

A

large no. of FIRM lymph nodes

90
Q

what CTD is associated with lymphoma?

A

Sjogrens

91
Q

what are the lymph nodes like in reactive lymphadenopathy ?

A

soft, mobile, only a couple

92
Q

abnormal bloods in myositis?

A

raised CK
raised AST
raised LDH

93
Q

most diagnostic Ix for GCA?

A

biopsy

however this can give false neg as GCA is patchy stuff

94
Q

presentation of HSP?

A

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

swelling in wrists, knees, ankles

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4TH YEAR (12 decks)

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