Connective Tissue Disease

Question 1

What structures does SLE mainly affect?

Answer 1

skin, joints, kidneys, blood cells and nervous system

Question 2

How many points of criteria does a person need to be diagnosed with SLE?

Answer 2

at least four with one being clinical and one being laboratory

Question 3

What is another way of diagnosing SLE other than the SILCC classification?

Answer 3

biopsy-proven lupus nephritis with positive ANA or anti-DNA

Question 4

What are the six things that make up the immunologic criteria for SLE?

Answer 4

ANA, anti-DNA, anti-Sm, antiphospholipid Ab, low complement, direct Coombs test

Question 5

What populations is SLE more present in?

Answer 5

Black people in the UK and the USA

Question 6

Is SLE more common in men or women?

Answer 6

women (like 90%)

Question 7

What is the 10 year survival rate in patients with SLE?

Answer 7

90%

Question 8

What are the constitutional symptoms of SLE?

Answer 8

fever, fatigue and weight loss

Question 9

What are the MSK features of SLE?

Answer 9

arthralgia, myalgia and inflammatory arthritis

Question 10

what are the Muco-cutaneous features of SLE?

Answer 10

malar rash, butterfly rash, alopecia, photosensitivity, discoid lupus, subacute cutaneous lupus, oral/nasal ulceration and Raynaud’s

Question 11

What are the renal symptoms of SLE?

Answer 11

lupus nephritis

Question 12

What are the respiratory symptoms of SLE?

Answer 12

pleurisy, pleural effusion, pneumonitis, PE, pulmonary hypertension, interstitial lung disease

Question 13

What are the haematological symptoms of SLE?

Answer 13

leukopenia, lymphopenia, anaemia and thrombocytopenia

Question 14

What are the cardiac symptoms of SLE?

Answer 14

pericarditis, pericardial effusion, pulmonary hypertension, sterile endocarditis and IHD

Question 15

What are the neuropsychiatric symptoms of SLE?

Answer 15

seizures, psychosis, headache, aseptic meningitis

Question 16

What are the GI symptoms of SLE?

Answer 16

autoimmune hepatitis, pancreatitis and mesenteric vasculitis

Question 17

What investigations would you do in SLE?

Answer 17

FBC, ANA, Anti-dsDNA, anti-Sm, anti Ro/LA/RNP, C3/4 levels, urinalysis and imaging

Question 18

How do you manage skin disease and arthralgia hydroxychloroquine caused by SLE?

Answer 18

topical steroids and NSAIDs

Question 19

How do you treat moderate SLE disease?

Answer 19

azathioprine, mycophenolate mofetil and corticosteroids

Question 20

How do you treat severe organ disease in SLE?

Answer 20

IV steroids and cyclophosphamide

Question 21

how do you treat patients with SLE where they have been unresponsive to previous treatment?

Answer 21

IV immunoglobulin and rituximab

Question 22

What is Sjogren’s syndrome

Answer 22

an autoimmune condition characterised by lymphatic infiltrates in exocrine organs

Question 23

What are the key symptoms in Sjogren’s syndrome?

Answer 23

dryness of mouth and eyes, arthralgia, fatigue, vaginal dryness and parotid gland swelling

Question 24

what can Sjogren’s syndrome be secondary to?

Answer 24

RA and SLE

Question 25

What conditions are you more at risk of if you have Sjogren’s?

Answer 25

Peripheral neuropathy, interstitial lung disease and lymphoma

Question 26

How do you diagnose Sjogren’s?

Answer 26

Schirmer’s test and +ve anti-Ro and anti La antibodies and a +ve lip gland biopsy

Question 27

How many of the American-European consensus group classification do you need to diagnose Sjogren’s?

Answer 27

four

Question 28

Is treatment for Sjogren’s curative or managing symptoms?

Answer 28

managing symptoms

Question 29

When would immunosuppression be involved in Sjogren’s?

Answer 29

when there is organ involvement like interstitial lung disease

Question 30

what symptoms present in Systemic sclerosis?

Answer 30

vasomotor disturbances, fibrosis and atrophy of the skin and subcutaneous tissue

Question 31

What causes systemic sclerosis?

Answer 31

excessive collagen deposition

Question 32

What is a finding in most patients with systemic sclerosis?

Answer 32

Raynaud’s

Question 33

What are the 3 phases of cutaneous involvement of systemic sclerosis?

Answer 33

1) oedematous, 2) indurative and 3) atrophic

Question 34

What are the major cutaneous features of systemic sclerosis?

Answer 34

central sclerosis that affects the arms, face and/or neck

Question 35

What are the minor cutaneous features of systemic sclerosis?

Answer 35

sclerodactyly, atrophy of the fingertips and bilateral lung fibrosis

Question 36

How do you diagnose, systemic sclerosis?

Answer 36

1 major and 2 minor features

Question 37

How do you classify systemic sclerosis?

Answer 37

limited or diffuse

Question 38

How do you investigate systemic sclerosis?

Answer 38

test for autoantibodies including anti-centromere and anti-Scl-70
organ screening

Question 39

How do you manage systemic sclerosis?

Answer 39

treat symptoms

Question 40

What symptoms are featured in Mixed connective tissue disease that are seen in other conditions?

Answer 40

Raynaud’s, arthralgia/ arthritis, myositis, sclerodactyly, pulmonary hypertension and interstitial lung disease

Question 41

What antibodies are associated with mixed connective tissue disease?

Answer 41

anti-RNP

Question 42

How do you manage mixed connective tissue disease?

Answer 42

maybe Ca2+ blockers, or immunosuppression

Question 43

What is anti-phospholipid syndrome?

Answer 43

recurrent venous or arterial thrombosis

Question 44

How do patients with anti-phospholipid syndrome present?

Answer 44

multiple conditions associated with clots, catastrophic APS, late spontaneous foetal loss, migraine, livedo reticularis

Question 45

How do you investigate anti-phospholipid syndrome?

Answer 45

thrombocytopenia and prolongation of APTT, lupus anticoag, anti-cardiolipin antibodies and anti-beta 2 glycoprotein

Question 46

How do you treat those with anti-phospholipid syndrome?

Answer 46

anti-coagulates, heparin if preggo