CSIM multi-system disease Flashcards
vit D in sarcoidosis?
raised
deficiency in what vit leads to aches and pain?
D
where is SLE rash typically sparing?
in the nasal-labial folds
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
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
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
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
what is the test for lupus anti-coagulant?
dilute russells viper venom time
clinically how is SLE different to APLS
APLS is associated with thrombotic events and misscarriage
what is livido reticularis and what is it associated with?
wavy mottled rash on legs
SLE
APLS
RA
most common CT disease?
SLE
pathophys of SLE?
immune response to self antigen
immune complexes form leading to
inflammation
complement activation
what are the 6 symptoms of CTD that should always be asked about?
from top to bottom:
- hair loss
- dry eyes
- mouth ulcers
- rashes (photosensitive)
- Raynauds
- joint pain
what is Jaccoud’s arthropathy?
seen in the hands:
normal at rest
swan necking on flexion
test for dry eyes?
Schirers tear test
most significant complication in congenital lupus?
heart block
what is APLS?
anti-phospholipid syndrome:
acquired autoimmune disorder that manifests clinically as recurrent venous or arterial thrombosis and/or foetal loss.
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
what pattern of rashes do we see in CTD?
lichenoid
vasculopathic
where does thrombocytopenic purpura present/ why?
in the legs
thats where pressure is highest
iatrogenic cause of purpura?
steroids
why blistering in vasculitis?
proteins that hold the epidermis to the dermis let go when it dies
how does the rash in vasculitis change as it progresses?
ulceration
what does livedo mean in latin?
net (thats why its a wavy shaped rash)
like when you get cold
difference between vasculitic and thrombotic rashes?
in thrombosis there is no time for it to rise so it’s FLAT
which anti body is associated with necrotizing vasculitis?
ANCA, pANCA or cANCA
systemic
two important Ix in systemic GPA?
bronchoscopy
renal biopsy
what is Henoch-Schönlein purpura (HSP)?
Immune complex deposition disease
DEFINITION OF leucocytoclastic vasculitis - small vessel, neutrophilis with extruded nuclei
epidemiology of GCA?
Commonest vasculitis (UK)
Age > 50 (usually in 70’s)
Female 2:1 Male
two types of vasculitis that effect the large vessels
Takayasu Arteritis (rare in the UK, common in Japan) Giant Cell Arteritis
two types of vasculitis that effect the medium vessels?
polyartritis nodosa
Kawasaki disease
what are the most common types of vasculitis in adults and children?
adults - GCA
children - Henoch- Schönlein (IgA)
Takayasu vasc effects who?
WOMEN
children up to 40
Japanese
effect of cushings on the skin?
STEROIDS
thins the skin so get striae and can see vessels through skin
acne
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
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
how does liver disease show in the skin?
porphyria- blisters on the back of the hands with scaring
how do lipid abnormalities present?
xanthelasma- macrophages eat the lipids
types of xantholasma and what they indicate?
Xanthelasma -Normal/several dyslipidaemias Tendon xanthomas -Familial hypercholesterolaemia Eruptive xanthomas -Hypertriglyceridaemia
skin changes in T1DM
Acanthosis nigricans
Also occurs in obesity
Necrobiosis lipoidica - granulomatous skin on tibia
Infections eg candida
skin changes in gout?
Tophi
periarticular urate - looks like RA
what does a blue edged ulcer make you think?
haem malignancies -> Pyoderma Gangrenosum
also seen in IBD
how can lymphoma present on the skin?
Cutaneous T cell lymphoma - often confused with psoriasis or eczema
what are koplic spots and what causes them?
blueish spots on buccal mucosa
measles
umbilicated blisters all the same size think what?
zoster
skin changes in syphilis?
Primary - Chancre Secondary - 6 weeks later palms, soles, generalised Snail-track ulcers Gumma- ulcers in bone, skin
rash in Lyme disease?
called erythema migrans - red ring rash ripples out over weeks
what is erythema nodosum?
inflammation of fat - seen in IBD, strep. , TB, sarcoidosis
skin in IBD?
pyoderma gangrenosum
erytherma nodosum
mouth ulcers
reiters syndrome triad? and other symptom
conjunc
urethritis
arthritis
psoriasis
what is pathergy? when is it seen?
when you get pyoderma due t puncturing the skin
seen in Behcet’s syndrome
skin changes in systemic sclerosis?
thickening - hands tends to claws, mouth is tight, beaking nose
another name for systemic sclerosis and the symptoms?
CREST syndrome
Calcification Raynauds Eosophagitis Sclerodactyly Telangiectasia
two paraneoplastic skin changes?
Acanthosis nigricans
Dermatomyositis
best marker in the blood for dermatomyositis?
CK elevation
causes of raised CK
Inflammatory muscle disease Polymyositis dermatomyositis muscular dystrophies rhabdomyolysis Motor neurone disease Iatrogenic – statin induced myositis
examination findings in GCA
absent pulses or bruits
tender temporal arteries
Ix for diagnosis of GCA?
biopsy
clinical corse of Takayasus?
inflammatory phase - may progress
ischemic phase - claudication
how does medium vessel vasculitis present differently to large?
orchitis
acute abdo
GI bleeding / ichaemia
who does Kawasaki occur in?
young children
symptoms of Kawasaki?
fever >5 days ♣ Rash ♣ Swollen glands in the neck ♣ Dry, cracked lips ♣ Red fingers or toes ♣ Red eyes
Ix to screen for complication of Kawasaki?
echo for aortic aneurysm
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)
systemic presentation of small vessel vasc?
- Alveolar haemorrhage
- Glomerulonephritis
- Scleritis
- Mononeuritis multiplex
what facial deformity is seen in small vessel vasc?
saddle nose deformity
which antibodies are most closely associate with CTD?
ANA - bind to the nucleus
very sensitive but not very specific
developing Raynauds when youre older is more or less associate with CTD?
more associated
having it when youre young is better
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
how does limited scleroderma present?
CREST syndrome
calcification raynauds esophageal (GI in general) sclerodactly telangectasia
what antibody associated with limited scleroderma?
anti-centromere
Ix to monitor the progession of scleroderma?
renal
PFT
weigh
ECHO
what is the main risk in Primary Sjogrens syndrome
lymphoma
presentation of PSS?
FEMALES in 60s:
- fatigue
- dry eyes
- dry mouth
- dry vagina
- parotid gland swelling
inflam. factors in PSS?
LOW CRP
high ESR
association with myalgia with muscle enzymes?
NONE
what is raised in myositis?
AST, ALT, LDH, CK
skin changes in dermatomyositis?
Gottrans papules - red papules on the knuckles
flagelate - lines
what anti-body is mixed connective tissue disease associated with?
RNP
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
what is sarcoidosis?
multi system disease characterised by epitheliod non-caseating granuloma in involved organs
where is sarcoidosis mostly seen?
lungs 95%
also skin, lymph nodes and everywhere else
signs and symptoms of sarcoidosis in the lungs?
signs - normally none (wheeze and fine crackles rarely)
symptoms - dyspnoea, cough, chest pain
characteristics of acute arthritis in sarcoidosis
Oligoarthritis
Large & small joint
Lower limb > upper limb
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
what is erythema nodosum?
acute, nodular, erythematous eruption that usually is limited to the extensor aspects of the lower legs
what annual investigations in sarcoidosis?
ECG and ECHO due to heart block risk
other that lofgrens syndrome, what is the classical way sarcoidosis presents?
uveoparodit fever:
uveitis
parotitis
fever
CNVII palsy
what are the nodes like in lympoma?
large no. of FIRM lymph nodes
what CTD is associated with lymphoma?
Sjogrens
what are the lymph nodes like in reactive lymphadenopathy ?
soft, mobile, only a couple
abnormal bloods in myositis?
raised CK
raised AST
raised LDH
most diagnostic Ix for GCA?
biopsy
however this can give false neg as GCA is patchy stuff
presentation of HSP?
rash on legs / buttocks - palpable purpura (IgA deposited in places
swelling in wrists, knees, ankles
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