Scleroderma

Question 1

systemic sclerosis

Answer 1

multisystem autoimmune disease targeting skin, lungs, heart, GIT, kidneys, MSK
peak incidence 30-50 years
F>M 4:1

Question 2

pathology of systemic sclerosis

Answer 2

vasculopathy, endothelial cell injury and dysfunction
extensive tissue fibrosis

Question 3

raynauds phenomenon

Answer 3

episodic, reversible peripheral digital ischaemic
usually provoked by cold or emotion
vasoconstriction (pallor) > venous stasis (cyanosis) > reactive hyperaemia (erythema)

Question 4

Primary Raynaud phenomenon

Answer 4

Raynaud’s disease
F>M 9:1
familial

Question 5

secondary Raynaud’s phenomenon

Answer 5

progressive structural changes in small blood vessels with permenently impaired flow
prolonged RP episodes can result in digital ulceration or infarction

Question 6

some causes of secondary Raynaud’s phenomenon

Answer 6

connective tissue disease: systemic sclerosis, sjogrens, SLE, MP/DM, RA
occlusive arterial disease: atherosclerosis, APS, buerger’s disease

Question 7

some causes of secondary Raynaud’s phenomenon

Answer 7

connective tissue disease: systemic sclerosis, sjogrens, SLE, MP/DM, RA
occlusive arterial disease: atherosclerosis, APS, buerger’s disease
vascular injury: cold injury, vibratory trauma
drugs and toxins: beta blockers, vinyl chloride, cocaine etc.
hyper viscosity/cold reacting proteins: polycythaemia, cryglobulinaemia, cold agglutinins, paraprotienaemia

Question 8

nail fold capillary microscopy

Answer 8

examine using ophthalmoscope
enlarged/distorted capillary loops or paucity of loops suggest connective tissue disease

Question 9

skin features of systemic sclerosis

Answer 9

skin puffiness (early)
induration and tethering (late)

Question 10

muscle changes in systemic sclerosis

Answer 10

inflammatory myositis - proximal muscle weakness, increased serum CK, AST

Question 11

tendon sheaths/periarticular structures changes in systemic sclerosis

Answer 11

tendon friction rubs, flexion contractures

Question 12

joints changes in systemic sclerosis

Answer 12

synovitis, erosive arthritis
distal digital tuft resorption

Question 13

3 types systemic sclerosis

Answer 13

diffuse cutaneous
limited cutaneous
systemic sclerosis sine scleroderma

Question 14

oesophageal changes in systemic sclerosis

Answer 14

GORD, strictures, Barrett’s oesophagus

Question 15

GI changes in systemic scerosis

Answer 15

fibrosis and atrophy of lamina propriety, submucosa and muscular layer resulting in diminished peristalsis, wide mouth colonic diverticula
watermelon stomach: gastric antral vascular ectasia (GAVE)

Question 16

kidney changes in systemic sclerosis

Answer 16

scleroderma renal crisis:
abrupt onset malignant hypertension, retinopathy and renal failure

Question 17

lung changes in systemic sclerosis

Answer 17

pulmonary hypertension
interstitial lung disease

Question 18

heart changes in systemic sclerosis

Answer 18

pericardial fibrosis: constrictive pericarditis, pericardial effusions with tamponade
myocardial: vascular endothelial proliferation and myocardial fibrosis - arrhythmias, conduction defects

Question 19

things to avoid to prevent Raynaud’s phenomenon

Answer 19

avoid cold, stress, nicotine, caffeine, psuedoepphedrine, b-blockers

Question 20

pharmacotherapy for Raynaud’s phenomenon

Answer 20

calcium channel blockers, angiotensin-receptor blockers, SSRI antidepressants, PDE-5 inhibitors, prostacyclin analog infusions

Question 21

pharmacotherapy for oesophageal disease

Answer 21

PPIs, H2-antagonists, pro kinetic agents

Question 22

pharmacotherapy for lung involvement

Answer 22

prednisolone, mycophenolate mofetil, cyclophosphamide v.v. pulses, azathioprine, rituximab

Question 23

arthralgia/arthritis pharmacotherapy

Answer 23

analgesics, NSAIDs, low dose steroids, methotrexate, hydroxychloroquine, physiotherapy

Question 24

inflammatory myopathy pharmacotherapy

Answer 24

steroids in combination with methotrexate, azathioprine or other immunosuppressants