Connective tissue disease

Question 1

What are the features of Sjorgen’s syndrome?

Answer 1

chronic inflammatory connective tissue disease
dysfunction of lacrimal and salivary glands
dry eyes and dry mouth

Question 2

Which sex is more commonly affected in sjorgen’s?

Answer 2

female 9:1

Question 3

Which autoantibodies are present in Sjorgen’s?

Answer 3

anti-ro/SSA
anti-la/PSS

Question 4

Aside from dryness, what are extraexocrine features/systems involved in sjorgen’s?

Answer 4

arhtritis
intersitial lung disease
GI symptoms
kidney disease
peripheral nervous system
CNS- meningitis, optic neuritis

Question 5

What is a serious long term complication of Sjorgen’s?

Answer 5

lymphoma!!

Question 6

List one feature of sjorgen’s that are risk factors for lymphoma?

Answer 6

parotid enlargement
lymphadenopathy
palpable purpura

Question 7

WHat are the key features of lupus?

Answer 7

vasculopathy
vasculitis
inflammation

Question 8

Three differentials for SLE?

Answer 8

TB
HIV/AIDS
Drug induced lupus
Sjorgen’s
Antiphospholipid syndrome
Vasculitis
Rheumatoid arthritis
Dermatomyositis

Question 9

List three investigations for SLE

Answer 9

FBC
ESR
CRP
Serum albumin
Serum creatinine/eGFR
Urinalysis and urine protein/creatinine ratio
renal biopsy
XRay hand and feet

Question 10

List two autoantibodies that are positive in SLE

Answer 10

ANA
dsDNA
Anti-Ro
Anti-La

Question 11

Which autoantibody in specific for SLE?

Answer 11

dsDNA

Question 12

List three criteria to diagnose SLE?

Answer 12

Malar rash
ridcoid rash
photosensitivity
oral ulcers
arthritis
renal disorder
neurologic disorder
haematologic sidorder
immunological disorder
ANA

Question 13

Which immune marker is positive in >95% of SLE?

Answer 13

ANA

Question 14

What is the treatment of SLE?

Answer 14

NSAIDs
smoking cessation
UV protection
exercise
corticosteroids
cDMARDs- methotrexate, hydroxychloroquine

Question 15

What is the treatment for severe SLE?

Answer 15

cyclophophamide IV

Question 16

Masklike face refers to which connective tissue disorder?

Answer 16

scleroderma

Question 17

What is CREST syndrome?

Answer 17

=limited cutaneous sclerosis
Calcinosis- calcium skin deposits
Raynaud’s
Esophageal involvement
Sclerodactly
Telangiectasia

Question 18

Which antibodies are positive in scleroderma?

Answer 18

ANA
Anti-Scl 70 antibody
Anticentromere antibody

Question 19

Which antibody is most commonly positive in limited scleroderma/CREST

Answer 19

anticentromere

Question 20

Which rash is characteristic of dermatomyositis

Answer 20

heliotrope rash
gottron’s papules

Question 21

What is the characteristic biochemical feature of polymyositis?

Answer 21

raised CK

Question 22

What are the features of polymyositis

Answer 22

symmetrical proximal muscle weakness
dysphagia
arrythmias
interstitial lung disease
acute tubular necrosis
arthralgia
raynaud’s

Question 23

List three lab tests that are positive in polymyositis

Answer 23

CK
ESR
ANA

Question 24

What are Gottron’s papules?

Answer 24

flat red papules scaly overlying knuckles of the fingers most commonly (and elbows and knees)

Question 25

Which condition are SLE pregnant patients at most high risk of?

Answer 25

pre eclampsia

Question 26

Anti-dsDNA is used to diagnose and monitor which disease?

Answer 26

SLE

Question 27

Anti-Ro?

Answer 27

Sjorgen’s

Question 28

Anti-La?

Answer 28

Ajorgen’s

Question 29

Anti-Sm?

Answer 29

SLE

Question 30

Ati- Jo 1?

Answer 30

myositis and lung disease

Question 31

Anti-Scl 70?

Answer 31

scleroderma

Question 32

Anti-centromere antibody?

Answer 32

scleroderma