Connective Tissue Diseases Flashcards

1
Q

name the algorithm used to name the different types of CT diseases

A
Samii
And
Sarah
Patted
My
Sausage
Dog
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2
Q

name the types of CT disease

A
sjogrens syndrome
anti-phospholipid syndrome
SLE
polymyositis
mixed CT disease
systemic sclerosis
dermatomyositis
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3
Q

are CT diseases diseases of connective tissue

A

no

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4
Q

what characterises CT diseases

A

all involve the presence of spontaneous over activity of the immune system usually by auto antibodies

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5
Q

which gender is lupus more common in

A

women

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6
Q

which ethnicity is lupus most common in

A

asians
afro-americans
afro-caribbeans
hispanic americans

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7
Q

is there a genetic element in lupus?

A

yes, common in identical twins

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8
Q

are there hormonal factors in lupus?

A

yes, increased oestrogen eg HRT/contraception increases risk

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9
Q

pathogenesis of lupus?

A

delayed clearance of dead cells

T cells provoke B cells to make ab’s via cytokines to them (thinks its an ag)

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10
Q

why should you worry about renal disease in lupus in particular?

A

shows no symptoms

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11
Q

why are the kidneys and skin infected in lupus?

A

antibody and antigen form an immune complex that deposits in kidney and skin; activate complement and get inflammatory reaction

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12
Q

clinical criteria in the classification of lupus?

A
cutaenous lupus
ulcers
arthritis
serositis
haemolytic anaemia 
leukopaenia
thrombocytopaenia
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13
Q

immunologic criteria in the classification of lupus?

A
presence of:
ANA
Anti-DNA
Antiphospholipid Ab
low complement
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14
Q

general symptoms of lupus

A
fever
malaise
poor appetite
weight loss
fatigue
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15
Q

mucocutaneous features of lupus

A

photosensitivity
malar rash
mouth ulcers
alopecia

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16
Q

MSK features of lupus

A

polyarthritis/arthralgia
myopathy
jaccouds arthritis

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17
Q

4 P’s of serositis

A

pericarditis
pleurisy
pleural effusion
pericardial effusion

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18
Q

what do blood and protein in urine indicate?

A

glomerulonephritis

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19
Q

neurological features of lupus

A

depression
seizures
confusion
cranial/peripheral neuropathy

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20
Q

haematological features of lupus

A
lymphademopathy
leucopenia
lymphopaenia
haemolytic anaemia
thombocytopaenia
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21
Q

female patient with recurrent miscarriages and recent DVT?

A

anti-phospholipid syndrome

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22
Q

main clinical features of APS

A

venous and arterial thrombosis
livido reticularis
miscarriage
thrombocytopaenia

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23
Q

what titre is most often positive in ANA testing of patients

A

1:160

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24
Q

what conditions cause a raised ANA

A

RA
HIV
hep c
CT disease

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25
Q

when should be the only time you take anti ANA into account with CT disease

A

if other autoantibodies are positive and the patient has CTD features

26
Q

most specific autoantibody test for lupus

A

anti double stranded antibody

27
Q

4 forms of anti ENA antibody

A

anti-ro
anti-la
anti-sm
anti-rnp

28
Q

which 2 neonatal issues are common when the mother is anti-ro +ve

A

neonatal lupus

foetal heart block

29
Q

name the 3 anti phospholipid antibodies

A

anti-cardiolipin antibody
lupus anticoagulant
anti-beta 2 glycoprotein

30
Q

what process ensures anti phospholipid antibody tests are accurate

A

repeat 3 months later to see if its still positive

31
Q

first investigation in suspected lupus?

A

urinalysis

32
Q

treatment for lupus?

A

NSAIDs
analgesia
hydroxychloroquine
steroids on a short course

33
Q

best drug for lupus

A

hydroxychloroquine

34
Q

what immunosuppressives can be used in lupus

A

azathioprine

methotrexate

35
Q

common symptoms that span all autoimmune diseases?

A
arthralgia
arthritis
raynauds
sicca (dryness)
myalgia
alopecia
ulcers
thrombosis
miscarriage
36
Q

when would you suspect APS?

A

young patient with unexplained venous and arterial events or thrombocytopaenia

37
Q

treatment for APS?

A

lifelong anticoags
aspirin
hydroxychlorine

38
Q

why does APS cause miscarriages?

A

clotsblood vessels to placenta so baby doesnt get nutrients or blood

39
Q

most common sex and age group for sjogrens

A

females in 50s and 60s

40
Q

clinical presentation of sjogrens

A

dry eyes, mouth due to diminished lacrimal and salivary gland function

gland enlargement
joint pain
fatigue

41
Q

treatment for sjogrens

A
tear supplements
eye drops
saliva supplements
hydrocychloroquine
pilocarpine
methotrexate
42
Q

what would you use to anticoagulate a pregnant woman?

A

aspirin

fragmin

43
Q

why are sjogrens patients more likely to get lymphoma

A

rapidly multiplying B cells

44
Q

2 main effects of systemic sclerosis

A

vascular endothelial changes

fibrosis

45
Q

most common secondary disease affecting lungs in diffuse cutaneous systemic sclerosis

A

pulmonary fibrosis

46
Q

which parts of the skin are involved in diffuse cutaneous systemic sclerosis

A

torso

proximal to forearms

47
Q

areas of skin affected in systemic sclerosis

A

distal to elbows only

48
Q

name the CREST symptoms that arise in systemic sclerosis

A
calcinosis
raynauds
esophageal dysmotility
sclerodactyly
telangiectasia
49
Q

what is sclerodactyly

A

localized thickening and tightness of the skin of the fingers or toes

50
Q

what is calcinosis

A

deposition of calcium in the skin, subcutaneous tissue, muscles and visceral organs

51
Q

common lung complication in systemic sclerosis

A

pulmonary hypertension

52
Q

what would treat the raynauds element of SS?

A

ca channel blockers

phosphodiesterase inhibitors

53
Q

what would treat the lung complications of SS?

A

immunosuppression

54
Q

what would treat the pulmonary hypertension complication of SS?

A

calcium channel blocker
endothelin receptor antagonists
oxygen

55
Q

what would treat the reflux complication of SS

A

PPIs

H2 receptor antagonists

56
Q

which auto antibody does mixed CT disease test positive to?

A

anti RNP

57
Q

what 3 diseases does mixed CT disease encompass

A

systemic sclerosis
lupus
polymyositis

58
Q

autoantibodies present in lupus

A

ana
anti-dna binding
anti-sm
anti-ro

59
Q

autoantibodies in systemic sclerosis

A

anti-centromere

anti-scl-70

60
Q

autoantibodies in polymyositis?

A

anti-jo-1