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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
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 COMMONEST CHILDHOOD VASCULITIS

4TH YEAR (12 decks)

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