Rheumatology 7 - Systemic Sclerosis

Question 1

define systemic sclerosis

Answer 1

autoimmune fibrotic/ scarring connective tissue disease affecting whole body

Question 2

describe the 2 types of systemic sclerosis

Answer 2

limited cutaneous

diffuse cutaneous

Question 3

5 features of limited cutaneous systemic sclerosis

Answer 3

Calcinosis (deposits of calcium at the fingertips)
Raynaud’s (mild cold - fingers completely white)
Oesophageal dysmotility - swallowing diffs, reflux
Sclerodactyly (tight, hard skin + poor ROM in fingers)
Telengiectasia

Question 4

4 features of diffuse cutaneous systemic sclerosis

Answer 4

CREST features +
cardio - HTN, CVD
lung - pulm HTN, fibrosis
kidney - GN, scleroderma renal crisis

Question 5

define the appearance of scleroderma

Answer 5

shiny tight skin in face or hands, lack normal folds

Question 6

what is scleroderma renal crisis

Answer 6

severe HTN + renal failure on background of dcSS

Question 7

key Abs in systemic sclerosis

Answer 7

ANA (non specific)
limited - anti centromere
diffuse - anti Scl70

Question 8

diagnosing systemic sclerosis

Answer 8

EULAR criteria
clinical features
antibodies
nailfold capillaroscopy

Question 9

nailfold capillaroscopy

Answer 9

examine peripheral capillaries by nailbed

abrnomal, avascular, microbleeds seen in systemic sclerosis

normal if just Raynaud’s of other cause

Question 10

treating systemic sclerosis

Answer 10

steroids / I supp + MDT

avoid smoking, use emollients, avoid cold, physio, OT

medications for complications

Question 11

medications for treatment of systemic sclerosis symptoms + complications

Answer 11

nifedipine - Raynaud's
PPIs - dysmotility
analgesia
abx for infetions
ACE inhibitors - HTN
lung support