multi system autoimmune disease

Question 1

systemic sclerosis

Answer 1

connective tissue disease characterised by fibrosis of skin and internal organs
significant alterations in microvasculature and cellular and humoral immunity

Question 2

types of scleroderma

Answer 2

systemic sclerosis
localised scleroderma
limited cutaneous systemic sclerosis (CREST)
diffuse cutaneous systemic sclerosis

Question 3

localised scleroderma

Answer 3

fibrotic involvement of skin and subcutaneous tissue
treatment guided by dermatologists
no risk of systemic involvement

Question 4

epidemiology SSc

Answer 4

30-50 years
females more than males

Question 5

SSc aetiology

Answer 5

unknown, many factors
genetic predisposition
environmental- gold and coal mines, silica, solvents, viral infections

vascular damage
leads to immune system activation
leads to fibrosis

Question 6

limited cutaneous SSc CREST

Answer 6

calcinosis
raynauds
oesophageal dysmobility
sclerodactyly
telangiectasia

anti centromere antibodies

Question 7

diffuse cutaneous SSc

Answer 7

features of CREST
plus cv problems
lung problems
kidney problems

anti Scl70 antibodies

Question 8

why dont treat scleroderma with steroids

Answer 8

can cause renal crisis

Question 9

Sjogrens sydrome

Answer 9

dry eyes, vagina and mouth for at least 3 months
can be primary or secondary to SLE or RA
anti ro, anti la
salivary gland biopsy swollen
parotid gland enlargement
systemic upset
schirmer test
female more
40-50yrs
common

Question 10

auto immune myositis

Answer 10

very rare
females
50-60yrs
increased risk of malignancy
unknown cause

Question 11

auto immune myositis signs and symptoms

Answer 11

muscle weakness
raised CK level
muscle biopsy golf standard
interstitial lung disease
hand papules
heliotrope rash on face

Question 12

mixed connective tissue disease

Answer 12

mix
raynauds
soft tissue swelling
myositis
arthralgia

can evolve into lupus, myositis, scleritis phenotype

Question 13

giant cell arteritis

Answer 13

white individuals
unknown cause

3 of
age >50
ESR>50
temporal artery tenderness/reduced pulsation- worse when brushing hair
abnormal temporal biopsy
new headache
jaw claudication

biopsy gold standard
ultrasound doppler 2nd line
then CT angiogram- for investigation of large vessel involvement

Question 14

treatment of giant cell arteritis

Answer 14

urgent high dose prednisolone
40-60mg per day
PPI
bone protection
steroid sparing medication
start during investigations

Question 15

complications of giant cell arteritis

Answer 15

aortic aneurysm
stroke
arterial stenosis
limb ischaemia
stroke

Question 16

types of ANCA associated vasculitis (Vasculitis Affecting The Small Vessels)

Answer 16

granulomatosis with polyangiitis (wegeners) GPA
microscopic polyangiitis
eosinophilic granulomatosis with polyangiitis

Question 17

GPA/ wegeners

Answer 17

stridor
upper and lower resp tract involves
necrotising granulomatous inflammation
glomerulonephritis
cANCA, anti PR3 antibodies
ENT symptoms
saddle-shaped nose due to a perforated nasal septum

Question 18

microscopic polyangiitis (MPA)

Answer 18

necrotising vasculitis
granulomatous inflammation is absent
renal failure and pulmonary involvement
pANCA, anti MPO antibodies - urine dipstick
prodrome

Question 19

tests for vasculitis

Answer 19

Inflammatory markers (CRP and ESR)

Anti neutrophil cytoplasmic antibodies (ANCA) is the blood test to remember for vasculitis

Question 20

churg strauss

Answer 20

Eosinophilia, poorly controlled asthma, CXR findings indicative of granulomatous change along with renal involvement make this the most likely diagnosis.