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