Connective Tissue Diseases

Question 1

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

Answer 1

Samii
And
Sarah
Patted
My
Sausage
Dog

Question 2

name the types of CT disease

Answer 2

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

Question 3

are CT diseases diseases of connective tissue

Answer 3

no

Question 4

what characterises CT diseases

Answer 4

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

Question 5

which gender is lupus more common in

Answer 5

women

Question 6

which ethnicity is lupus most common in

Answer 6

asians
afro-americans
afro-caribbeans
hispanic americans

Question 7

is there a genetic element in lupus?

Answer 7

yes, common in identical twins

Question 8

are there hormonal factors in lupus?

Answer 8

yes, increased oestrogen eg HRT/contraception increases risk

Question 9

pathogenesis of lupus?

Answer 9

delayed clearance of dead cells

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

Question 10

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

Answer 10

shows no symptoms

Question 11

why are the kidneys and skin infected in lupus?

Answer 11

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

Question 12

clinical criteria in the classification of lupus?

Answer 12

cutaenous lupus
ulcers
arthritis
serositis
haemolytic anaemia 
leukopaenia
thrombocytopaenia

Question 13

immunologic criteria in the classification of lupus?

Answer 13

presence of:
ANA
Anti-DNA
Antiphospholipid Ab
low complement

Question 14

general symptoms of lupus

Answer 14

fever
malaise
poor appetite
weight loss
fatigue

Question 15

mucocutaneous features of lupus

Answer 15

photosensitivity
malar rash
mouth ulcers
alopecia

Question 16

MSK features of lupus

Answer 16

polyarthritis/arthralgia
myopathy
jaccouds arthritis

Question 17

4 P’s of serositis

Answer 17

pericarditis
pleurisy
pleural effusion
pericardial effusion

Question 18

what do blood and protein in urine indicate?

Answer 18

glomerulonephritis

Question 19

neurological features of lupus

Answer 19

depression
seizures
confusion
cranial/peripheral neuropathy

Question 20

haematological features of lupus

Answer 20

lymphademopathy
leucopenia
lymphopaenia
haemolytic anaemia
thombocytopaenia

Question 21

female patient with recurrent miscarriages and recent DVT?

Answer 21

anti-phospholipid syndrome

Question 22

main clinical features of APS

Answer 22

venous and arterial thrombosis
livido reticularis
miscarriage
thrombocytopaenia

Question 23

what titre is most often positive in ANA testing of patients

Answer 23

1:160

Question 24

what conditions cause a raised ANA

Answer 24

RA
HIV
hep c
CT disease

Question 25

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

Answer 25

if other autoantibodies are positive and the patient has CTD features

Question 26

most specific autoantibody test for lupus

Answer 26

anti double stranded antibody

Question 27

4 forms of anti ENA antibody

Answer 27

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

Question 28

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

Answer 28

neonatal lupus | foetal heart block

Question 29

name the 3 anti phospholipid antibodies

Answer 29

anti-cardiolipin antibody lupus anticoagulant anti-beta 2 glycoprotein

Question 30

what process ensures anti phospholipid antibody tests are accurate

Answer 30

repeat 3 months later to see if its still positive

Question 31

first investigation in suspected lupus?

Answer 31

urinalysis

Question 32

treatment for lupus?

Answer 32

NSAIDs analgesia hydroxychloroquine steroids on a short course

Question 33

best drug for lupus

Answer 33

hydroxychloroquine

Question 34

what immunosuppressives can be used in lupus

Answer 34

azathioprine | methotrexate

Question 35

common symptoms that span all autoimmune diseases?

Answer 35

``` arthralgia arthritis raynauds sicca (dryness) myalgia alopecia ulcers thrombosis miscarriage ```

Question 36

when would you suspect APS?

Answer 36

young patient with unexplained venous and arterial events or thrombocytopaenia

Question 37

treatment for APS?

Answer 37

lifelong anticoags aspirin hydroxychlorine

Question 38

why does APS cause miscarriages?

Answer 38

clotsblood vessels to placenta so baby doesnt get nutrients or blood

Question 39

most common sex and age group for sjogrens

Answer 39

females in 50s and 60s

Question 40

clinical presentation of sjogrens

Answer 40

dry eyes, mouth due to diminished lacrimal and salivary gland function gland enlargement joint pain fatigue

Question 41

treatment for sjogrens

Answer 41

``` tear supplements eye drops saliva supplements hydrocychloroquine pilocarpine methotrexate ```

Question 42

what would you use to anticoagulate a pregnant woman?

Answer 42

aspirin | fragmin

Question 43

why are sjogrens patients more likely to get lymphoma

Answer 43

rapidly multiplying B cells

Question 44

2 main effects of systemic sclerosis

Answer 44

vascular endothelial changes | fibrosis

Question 45

most common secondary disease affecting lungs in diffuse cutaneous systemic sclerosis

Answer 45

pulmonary fibrosis

Question 46

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

Answer 46

torso | proximal to forearms

Question 47

areas of skin affected in systemic sclerosis

Answer 47

distal to elbows only

Question 48

name the CREST symptoms that arise in systemic sclerosis

Answer 48

``` calcinosis raynauds esophageal dysmotility sclerodactyly telangiectasia ```

Question 49

what is sclerodactyly

Answer 49

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

Question 50

what is calcinosis

Answer 50

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

Question 51

common lung complication in systemic sclerosis

Answer 51

pulmonary hypertension

Question 52

what would treat the raynauds element of SS?

Answer 52

ca channel blockers | phosphodiesterase inhibitors

Question 53

what would treat the lung complications of SS?

Answer 53

immunosuppression

Question 54

what would treat the pulmonary hypertension complication of SS?

Answer 54

calcium channel blocker endothelin receptor antagonists oxygen

Question 55

what would treat the reflux complication of SS

Answer 55

PPIs | H2 receptor antagonists

Question 56

which auto antibody does mixed CT disease test positive to?

Answer 56

anti RNP

Question 57

what 3 diseases does mixed CT disease encompass

Answer 57

systemic sclerosis lupus polymyositis

Question 58

autoantibodies present in lupus

Answer 58

ana anti-dna binding anti-sm anti-ro

Question 59

autoantibodies in systemic sclerosis

Answer 59

anti-centromere | anti-scl-70

Question 60

autoantibodies in polymyositis?

Answer 60

anti-jo-1