connective tissue disease

Question 1

what type of hypersensitivity reaction is systemic lupus erythematous?

Answer 1

type 3 HS

Question 2

how many symptoms do you need for a diagnosis of SLE?

Answer 2

at least 4

Question 3

what are the constitutional symptoms of SLE?

Answer 3

fatigue
weight loss
malaise 
fever
poor appetite

Question 4

what are the skin symptoms of SLE?

Answer 4

malar rasht hat spares the nasolabial folds 
(butterfly rash) 
photosenstivity 
alopecia
scaly scarring discoid rash

Question 5

what symptom of the mouth is common is SLE?

Answer 5

mouth ulcers

Question 6

what sort of joint pain is common in SLE?

Answer 6

non erosive small joint arthritis similar to RA

Jaccouds arthritis - non erosive arthritis associated with ulnar deviation

Question 7

what chest symptoms are in SLE?

Answer 7

pericarditis

pleural effusion/pleuritic chest pain/SOB

Question 8

what affect can SLE have on your kindeys?

Answer 8

glomerulonephritis?

Question 9

what haematological conditions can be caused by SLE?

Answer 9

lymphadenopathy
leukopenia
anaemia
thrombocytopaenia

Question 10

what is Libman Sack in SLE?

Answer 10

a type of non bacterial sterile endocarditis

Question 11

what is a symptom of the hands associated with SLE?

Answer 11

raynauds

Question 12

what is the 1st screening test done for SLE? what should follow if positive?

Answer 12

urine analysis

renal biopsy

Question 13

what immunology is associated with SLE?

Answer 13

anti - ANA (non specific)

anti - DsDNA

Question 14

what is anti-DsDNA associated with?

Answer 14

it increases with disease activity

Question 15

what would you expect C3/4 to do with active disease?

Answer 15

decrease

complement used up

Question 16

is CRP used in SLE?

Answer 16

no

does not increase with flare ups (only raised during infection/co-exciting disease e.g. RA)

Question 17

what is mild SLE?

Answer 17

skin disease and arthralgia

Question 18

what is moderate SLE?

Answer 18

inflammatory arthritis and organ involvement

Question 19

what is severe SLE?

Answer 19

organ disease

Question 20

what is the treatment for mild SLE?

Answer 20

hydroxycloroquine
NSAIDs
topical steroids

Question 21

what is the treatment for moderate SLE?

Answer 21

methotrexate
azathioprine
oral steroids

Question 22

what is the treatment for severe SLE?

Answer 22

IV steroids
cyclophosphamide
rituximab

Question 23

what else should be done to manage SLE?

Answer 23

avoid sun exposure (photosensitivity)

Question 24

how does drug induced SLE present?

Answer 24

feature of lupus without renal or neurological involvement

- arthralgia, myalgia, malaria skin rash, ANA +ve

Question 25

what drug commonly causes SLE?

Answer 25

hydralazine | stops on stopping the drug

Question 26

what can cause anti-phospholipid syndrome?

Answer 26

primary | secondary to lupus

Question 27

what symptoms are associated with anti-phospholipid syndrome?

Answer 27

venous/arterial thrombosis (DVT) recurrent miscarriage thrombocytopenia

Question 28

what skin symptom is seen in APS?

Answer 28

livedo reticularis | mottled skin covered with red/purple patched in a web pattern

Question 29

how is a diagnosis of APS made?

Answer 29

1 lab and 1 clinical finding

Question 30

what are the clinical findings needed to make a diagnosis of APS?

Answer 30

aterial/venous thrombosis pregnancy loss with no other explanation from 10 weeks onwards 3 pregnancy losses before 10 weeks pregnancy loss due to pre-eclampsia/placental insufficiency

Question 31

what are the lab findings needed to make a diagnosis of APS?

Answer 31

``` 2 positive findings, 12 weeks apart anti-cardiolipin antibody lupus anti-coagulation test anti-b2 glycoprotein causes low platelets and prolonged APTT ```

Question 32

what is the treatment for APS after a primary thrombosis?

Answer 32

low dose aspirin

Question 33

what is the treatment for APS after a secondary thrombosis?

Answer 33

after first VTW, life long warfarin INR 2-3 if it happens on warfarin then low dose aspirin too

Question 34

what is the treatment for APS if pregnant?

Answer 34

aspirin between pregnancies | aspirin and heparin during pregnancy (warfarin teratogenic)

Question 35

what does sjogrens increase your risk of?

Answer 35

lymphoma | increased B cell activity

Question 36

what are the symptoms of Sjogrens syndrome?

Answer 36

``` DRYNESS kertoconjunctivitis sicca (dry eyes) wake up at night needing water, difficulty swallowing bread xerostomia (dry mouth) parotid swelling vaginal dryness fatigue, raynauds tooth decay ```

Question 37

what test can be done for Sjogrens?

Answer 37

Schrimers test filter paper on eye, access how wet it is <5mm in 5 mins = +ve dry eyes

Question 38

what immunology is positive of Sjogrens

Answer 38

anti-ro anti-la ANA +ve (non specific)

Question 39

what is the treatment for Sjogrens?

Answer 39

1st line hydrocholoriquine (for fatigue and arthralgia) | pilocarpine - stimulate salivary glands

Question 40

who should not use pilocarpine?

Answer 40

asthmatics

Question 41

what should be monitored for in Sjogrens?

Answer 41

non Hodgkins B cell lymphoma

Question 42

what is scleroderma?

Answer 42

term meaning skin thickening

Question 43

what does 90% of sclerosis patients present with?

Answer 43

raynauds

Question 44

what is localised scleroderma?

Answer 44

dermal fibrosis and inflammation without any organ involvement like to be seen with raynauds

Question 45

what is systemic sclerosis (CREST)?

Answer 45

skin fibrosis associated with sclerosis of viscera and proceeded by raynauds

Question 46

what it the presentation of sclerosis?

Answer 46

``` lung fibrosis pulmonary hypertension hypertension dysphagia renal hypertensive crisis ```

Question 47

what is the classification for slerosis?

Answer 47

diffuse (30%) | limited (70%)

Question 48

what immunology and onset is seen in diffuse sclerosis?

Answer 48

anti-SCL 70 | onset of wisespread skin slecrosis affecting trunk and entire limbs 1 year after raynauds

Question 49

what immunology and onset is seen in limited sclerosis?

Answer 49

anti-centromere | onset of skin fibrosis limited to distal limbs year after raynauds

Question 50

what are the classic features of limited sclerosis?

Answer 50

``` CREST + P calcinosis - calcium deposits in fingers raynauds esophageal dysfunction (acid reflux) sclerodactyly (thinking of skin) talagiectasia pulmonary hypertension ```

Question 51

how is sclerosis managed?

Answer 51

annual ECHO and PFT (due to pulmonary hypertension) control BP - ACE inhibitor immunosuppression - cyclophosphamide (steroids may cause hypertensive crisis)

Question 52

what is mixed connective tissue disease?

Answer 52

features of SLE, systemic sclerosis and polymyositis

Question 53

what immunology is seen with mixed connective tissue disease?

Answer 53

anti-RNP

Question 54

what is 1st line drug treatment for raynauds?

Answer 54

calcium channel antagonist - nifedipine