Connective Tissue Diseases

Question 1

what are autoantibodies

Answer 1

antibodies generated by the immune system against the bodys own proteins

Question 2

what is another name for conditions with auto-antibody production

Answer 2

seropositive conditions

Question 3

condition associated with anti-CCP antibody

Answer 3

rheumatoid arthritis

Question 4

conditions associated with anti-nuclear antibody (5)

Answer 4

SLE
sjogrens
systemic sclerosis
MCTD
autoimmune liver disease

Question 5

condition associated with anti-double stranded DNA antibody

Answer 5

SLE

Question 6

condition associated with anti-Sm antibody

Answer 6

SLE

Question 7

conditions associated with anti-Ro antibody

Answer 7

SLE, sjogrens

Question 8

condition associated with anti-La antibody

Answer 8

sjogrens

Question 9

condition associated with anti-centromere antibody

Answer 9

systemic sclerosis (limited)

Question 10

condition associated with anti-Scl-70 antibody

Answer 10

systemic sclerosis (diffuse)

Question 11

condition associated with anti-neutrophil cytoplasmic antibody

Answer 11

small vessel vasculitis

Question 12

who is SLE more common in

Answer 12

Women
Asian, African, Caribbean and Hispanic ethnicity
Young to middle-aged adults

Question 13

pathophysiology of SLE

Answer 13

anti-nuclear antibodies generate a chronic inflammatory response

Question 14

name some non-specific symptoms of SLE

Answer 14

malar rash - worsened by sunlight
joint pain
muscle pain
non-scarring alopecia
raynauds
jaccouds arthropathy

Question 15

describe the arthritis seen in SLE

Answer 15

synovitis or tenderness of at least 2 joints with >30 mins of early morning stiffness

Question 16

what is jaccouds arthropathy

Answer 16

non-erosive reversible joint disoder

Question 17

name a renal complication of SLE

Answer 17

nephritis

Question 18

what is the first line inevstigation for SLE

Answer 18

antinuclear antibody titre

Question 19

name some antibodies that may be tested for if ANA positive in suspected SLE

Answer 19

anti-dsDNA
anti-Ro
anti-smith

Question 20

blood results in a patient with SLE

Answer 20

leukopenia, thrombocytopenia, haemolytic anaemia
low complement (C3+4)

Question 21

proteinuria in suspected SLE

Answer 21

indicated lupus nephritis

Question 22

investigation for lupus nephritis following positive urine dipstick

Answer 22

biopsy

Question 23

general management of SLE

Answer 23

sun protection
minimise steroid use
monitoring !!!

Question 24

management of mild-moderate SLE

Answer 24

hydroxychloroquine

Question 25

management of acute flairups of SLE

Answer 25

immunosuppressants, short stints of oral steroids

Question 26

management of severe organ damage associated with SLE

Answer 26

IV steroids and cyclophosphamide

Question 27

how do we monitor SLE

Answer 27

anti-dsDNA and complement levels, urinalysis for blood or protein

Question 28

complication of SLE

Answer 28

avascular necrosis, usually of the femoral head

Question 29

what is sjogrens syndrome

Answer 29

autoimmune condition that affects the exocrine glands

Question 30

who is more likely to present with sjogrens syndrome

Answer 30

middle aged women

Question 31

symptoms of sjogrens syndrome

Answer 31

dry eyes, mouth, throat vaginal dryness in pre-menopausal women joint pain fatigue

Question 32

name 2 clinical signs of sjogrens syndrome

Answer 32

bilateral parotid gland enlargement unexplained increase in dental carries

Question 33

investigations for sjogrens syndrome

Answer 33

bloods: anti-ro and anti-la antibodies

Question 34

management of sjogrens syndrome

Answer 34

symptom control: tear and salivary replacement, analgesia and exercise for pain and fatigue

Question 35

complications of sjogrens

Answer 35

increased risk of lymphoma peripheral neuropathy ILD

Question 36

what are the 2 main types of systemic sclerosis

Answer 36

limited and diffuse

Question 37

who usually presents with systemic sclerosis

Answer 37

women aged 30-50

Question 38

skin involvement in limited systemic sclerosis

Answer 38

face, hands, forearms and feet

Question 39

raynauds preceeding systemic sclerosis

Answer 39

limited

Question 40

clinical presentation of systemic sclerosis

Answer 40

CREST calcinosis, Raynaud's, oesophageal dysfunction, sclerodactyly, telangiectasia

Question 41

what is sclerodactyly

Answer 41

thickening of the skin of the fingers and hands which can cause loss of dexterity of hands

Question 42

name some complications of systemic sclerosis

Answer 42

pulmonary arterial hypertension pulmonary fibrosis

Question 43

non-medical management of systemic sclerosis

Answer 43

avoid smoking skin stretching, regular emollients physiotherapy for joints

Question 44

what is used to treat symptoms of raynauds

Answer 44

nifedipine

Question 45

clinical presentation of mixed connective tissue disease (6)

Answer 45

- Raynaud’s - Arthralgia/arthritis - Myositis - Sclerodactyly - Pulmonary hypertension - ILD

Question 46

3 main investigations for mixed connective tissue disease

Answer 46

bloods, antibodies, imaging

Question 47

bloods in mixed connective tissue disease

Answer 47

FBC: anaemia, leukopenia hypergammaglobulinemia raised inflammatory markers

Question 48

who usually presents with anti-phospholipid syndrome

Answer 48

women

Question 49

bloods in anti-phospholipid syndrome

Answer 49

thrombocytopenia prolonged aPTT