Connective Tissue Diseases: Systemic Sclerosis

Question 1

what is there a triad of

Answer 1

autoimmunity, fibrosis and vascular endothelial changes

fibrosis causes subsequent atrophy of the skin and subcutaenous tissue

Question 2

what is usually responsible for death

Answer 2

renal and lung changes

pulmonary hypertension - 12% deaths

Question 3

what is a common early finding and is unlikely to have SS without

Answer 3

Raynaud’s

Question 4

what are the 3 phases of cutaenous involvement

Answer 4

oedematous

indurative

atrophic

• skin becomes thickened and tight

Question 5

major features

Answer 5

centrally located skin sclerosis that affects face arms and neck

Question 6

minor features

Answer 6

• Sclerodactyly (skin thickening) of the fingertips and atrophy of the underlying tissues
• Internal organ fibrosis and/or vascular damage (lungs, heart, G.I. tract ± kidneys)

Question 7

what features are needed to make a diagnosis

Answer 7

1 major and 2 minors

Question 8

what happens when the skin of the face is affected

Answer 8

pinching of skin of nose - beaking

Question 9

what can be seen on the face along with beaking of the nose

Answer 9

telangectasia

Question 10

calcinosis

Answer 10

Calcinosis (skin can breakdown and discharge chalky material (calcium)).

Question 11

lung involvement

Answer 11

• Pulmonary hypertension, pulmonary fibrosis and accelerated hypertension leading to scleroderma renal crisis (10% patients).

Question 12

renal involvement

Answer 12

Scleroderma renal crisis is characterised by accelerated hypertension, rapidly progressive kidney failure and proteinuria.

Question 13

GI involvement

Answer 13

• Gut involvement leading to dysphagia, dyspepsia, malabsorption and bacterial overgrowth of small bowel
• Importantly oesophageal dysmotility
• Lower G.I.: bloating, distension, nausea, vomiting, pain, diarrhoea, constipation

Question 14

what can gut invovlement lead to

Answer 14

oesophageal dysmotility

Question 15

limited

Answer 15

• formerly CREST syndrome
• skin involvement confined to face, hands and forearm
• organ involvement tends to occur later

Question 16

CREST

Answer 16

calcinosis

raynaud’s

oesophageal and gut dysmotility

sclerodactyly

telangiectasia

Question 17

antibodies for limited

Answer 17

anti-centromere antibody and ANA

Question 18

diffuse

Answer 18

• Skin changes more rapidly and scleroderma tends to affect trunk and proximal limbs predominantly
• Can present acutely with intense skin itching
• Early significant organ involvement (hypertension, lung fibrosis and renal crisis)

Question 19

antibodies for diffuse

Answer 19

anti-topoisomerase

anti SCL-70

anti RNA III polymerase antibody

Question 20

investigations

Answer 20

auto antibodies

organ screening regularly

urinalysis

Question 21

management

Answer 21

tailored to specific issues

tight control of BP

Question 22

raynaud’s treatment and definition

Answer 22

spasm of blood vessels in fingers in cold

calcium channel blockers - vasodilate

Question 23

medication if there is renal involvement

Answer 23

ACE inhibitors

Question 24

what can reflux cause

Answer 24

fibrosis

Question 25

MCTD

Answer 25

features of SLE, polymyositis and SS

Question 26

what is a recognised complication of MCTD

Answer 26

pulmonary hypertension

Question 27

antibody for MCTD

Answer 27

anti RNP antibody

Question 28

treatment of MCTD

Answer 28

Depends on severity. Mild: NSAIDs, short course of steroids, hydroxychloroquine, symptomatic Joint inflammation: methotrexate, DMARD and short course of steroids Organ involvement: high dose steroids and IS Ca channel blockers may be helpful for Raynaud’s.