Systemic Sclerosis

Question 1

what is systemic sclerosis

Answer 1

multisystem autoimmune disorder

Question 2

what is systemic sclerosis characterised by

Answer 2

vasculopathy, autoimmunity and FIBROSIS

Question 3

what symptoms can be seen in systemic sclerosis

Answer 3

classic symptoms of Raynaud’s, skin thickening, difficulty swallowing, GORD, telangiectasia, calcinosis, +/- SOB

Question 4

what are the symptoms of Raynaud’s phenomenon, very common in systemic sclerosis patients

Answer 4

classically triphasic

blanching(white), acrocyanosis(purple/blue), reactive hyperaemia(redness)

Question 5

what are some red flags in systemic sclerosis

Answer 5

raynaud’s + SOB, raynaud’s + telangiectasia, onset raynaud’s in mid/late adulthood, raynaud’s + skin complication

Question 6

what is telangiectasia

Answer 6

small dilated blood vessels near surface of skin, also called spider veins

Question 7

what are the 2 different subtypes of systemic sclerosis

Answer 7

diffuse cutaneous and limited cutaneous

Question 8

describe diffuse systemic sclerosis

Answer 8

skin involvement on extremities ABOVE and BELOW elbows and knees, plus face and trunk

Question 9

describe limited systemic sclerosis

Answer 9

skin involvement on extremities and only BELOW the elbows and knees

Question 10

describe the pathophysiology of systemic sclerosis

Answer 10

disregulated chronic inflammation which drives fibrosis of blood vessels(vasculopathy), then causes connective tissue fibrosis which support the blood vessels and form extrinsic fibrosis around vessels

Question 11

what are the important antibodies seen in systemic sclerosis

Answer 11

anticentromere, antitopoisomerase(anti-Scl-70) and anti-RNA polymerase(less important)

Question 12

what other name did systemic sclerosis used to be called, and what does it mean

Answer 12

scleroderma, means thick skin

Question 13

what antibodies are more common in diffuse and limited systemic sclerosis

Answer 13

diffuse = antitopoisomerase
limited = anticentromere

Question 14

describe the rate of progression of diffuse cutaneous and limited cutaneous systemic sclerosis

Answer 14

diffuse faster progression, more symptoms sooner

Question 15

what face changes may be seen in systemic sclerosis

Answer 15

small “puckering” mouth, “beaked” nose, scleroderma, telangiectasia

Question 16

what are some of the GI complications of systemic sclerosis

Answer 16

dysphagia, GORD, gastric antral vascular ectasia(GAVA), malabsorption, fluctuating bowel habit/incontinence

Question 17

what cardio/pulmonary complications may be seen in systemic sclerosis

Answer 17

interstitial lung disease, pulmonary arterial hypertension, myocardial disease

Question 18

what are some of the renal complications that can be seen in systemic sclerosis

Answer 18

scleroderma renal crisis(very urgent), non specific progressive renal dysfunction

Question 19

what other complications may be seen in systemic sclerosis

Answer 19

sclerodacyly(localised thickeing of fingers/toes), digital ischaemia, myositis

Question 20

what symptoms are seen with systemic sclerosis pulmonary hypertension, due to vasculopathy, and what will it lead to

Answer 20

predominantly SOB on exertion, progressive complication that will lead to right HF
(essential to screen for PAH early)

Question 21

what symptoms/signs are seen in pulmonary fibrosis due to systemic sclerosis

Answer 21

progressive SOB, usually cough, bilateral crackles, restrictive LFT

Question 22

describe what is seen in renal disease in systemic sclerosis and what is it associated with

Answer 22

uncontrolled hypertension with proteinuria, and rapidly worsening renal function