connective tissue disorders

Question 1

systemic lupus erythematous

• m:f
• onset

Answer 1

90% females

20-80

Question 2

clinical features of LUPUS 12

Answer 2

1. fever
2. weight loss
3. mild lymphadenopathy
   4.arthritis-arthralgia
   5.raynaud’s
4. skin
   -photosensitive facial rash
   -erythematous
   -malar rash
   or discoid rash
   -livedo reticularis (antiphos syndrome)

6.kidney
-proliferative glomerulonephritis
7.cardio
-pericarditis
-myocarditis
-endocarditis
-arthersclerosis
8.lung
-pleuritic pain
-pneumonitis
-breathlessness
dvt risk
8. headaches

9. haematological
   - neutropenia
   - lymphopenia
   - thrombocytopenia
10. gi
    - mouth ulcers
    - peritoneal serositis
    - mesenteric vasculitis

Renal proliferative glomerular nephritis

Question 3

which of the above predicts severity

Answer 3

renal involvement

haematuria, proteinuria and cast

Question 4

what is anti-phospholipid syndrome and what to give

Answer 4

more miscarriages
more risk thrombosis and stroke
heart valve disease
low platelet count
give warfarin
Livedo reticularis also seen

Question 5

11 criteria of lupus

Answer 5

malar rask 
discoid rash
photosensitive skin rash
oral ulcers
arthritis: 2 or more joints
serositis: pericarditis, pleuritis
renal disorder >0.5g in 24 hrs
neuro disorder: seizure psychosis
haem disorder
4/11

Question 6

blood investigation results for SLE 5

Answer 6

Anti ds DNA 
ENA antibodies or Ana 
- anti la
-anti ro
-anti sm 
low c3 or c4 
raised esr

but normal crp

Question 7

management of SLE

Answer 7

analgesic and nsaid
hydroxychloroquine
cyclophosphamide 
steroids
dmards
biologicals

Question 8

sle with severe renal management

Answer 8

mycophenylate and steroids

Question 9

main bio for sle

Answer 9

belimumab

Question 10

advise for sle

Answer 10

stop smoking

control bp and lipids

Question 11

management antiphos syndrome

Answer 11

give warfarin

Question 12

management osteop

Answer 12

adcal

Question 13

what is systemic sclerosis

Answer 13

autoimmune disorder of connective tissue causing fibrosis

Question 14

symptoms of systemic sclerosis

Answer 14

crest
calcinosis: calcium depositis in skin
r: Raynaud's
e: oesophageal dysmotility
s: sclerodactyly: thickening of toes and fingers
t: telangiectasia
-oedema
-capillary loss
-arthralgia due to skin 
-gi involvement 
stomach and oesophagus
-pulmonary: ILD
-renal
hypertensive renal crisis

Question 15

f:m for systemic s

Answer 15

4:1

Question 16

onset of ss

Answer 16

4th and 5th decade

Question 17

poorer prognosis for ss

Answer 17

diffuse only 5 year survival
older age
higher esr
proteinuria
low gas transfer factor for Carbon monoxide
pulmonary hypertension

Question 18

gene assoc. to connective tissue disease

Answer 18

HLA locus

Question 19

inx of ss

Answer 19

-ANA antibodies positive or anti ENA
Which is anti scl 70

• dss antibodies to topoisomerase 1
• ISS anticentromere antibodies
• cxr for ILD
• barium swallow

Question 20

management of ss

Answer 20

no treatment

• calcium channel blockers: losartan, fluoxetine
• prostacyclin IV for severe disease
• bosentran: digital ulcers
• ppi for oesophagus

Question 21

mixed connective tissue disease includes

Answer 21

ss
myositis
sle

Question 22

symptoms of mixed connective tissue disease

Answer 22

puffy fingers
dactylitis 
sclerodactyly
Raynaud
myalgia

Question 23

antibody in mixed connective

Answer 23

anti RNP one of the anti ENA/ana

Question 24

primary sjorgen syndrome onset

Answer 24

40-50

Question 25

female to male ratio sjorgen

Answer 25

9:1

Question 26

gene for sjorgen

Answer 26

HLA-B8/DR3

Question 27

clinical symptoms of sjorgen 3 main

Answer 27

dry eyes 
-conjunctivitis
-blepharitits
dry mouth 
-dental carie
arthritis
fatigue

Question 28

word for dry mouth

Answer 28

xerostomia

Question 29

other clinical features of sjorgen

Answer 29

lymphoma
fever
anaemia
thrombocytopenia
lymphadenopathy 
ILD

Question 30

antibodies for sjorgen

Answer 30

RF
ANA
ssa anti ro
ssb anti la
gastric parietal cell
thyroid

Question 31

investigations of sjorgen

Answer 31

schrimer tear test

• 5 minute test
• esr
• lymphocyte
• antibodies

Question 32

management of sjorgen

Answer 32

• lacrimal substitutes: hypromellose
• saliva tablets
• oral hygiene
• pilocarpine for gland function
• hydroxychloroquine for skin and msk

Question 33

what is sox syndrome

Answer 33

sialadentitis (inflamed saliva glands)
oa
xerostomia

Question 34

Onset sle

Answer 34

15 to 40