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E - MSK > Multisystem Autoimmune Disorders > Flashcards

Multisystem Autoimmune Disorders Flashcards

1
Q

Presentation of SLE (7)

A 
Joint pain and stiffness
Extreme tiredness
Skin rashes (butterfly)
Weight loss
Swollen glands
Sensitivity to light 
Raynaud's
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2
Q

Classification criteria for SLE (11)

A
  1. Malar rash
  2. Discoid rash
  3. Photosensitivity
  4. Oral ulcers
  5. Arthritis (>2)
  6. Serositis
  7. Proteinuria / cellular casts in urine
  8. Seizures or psychosis
  9. Haem - low WCC, platelets, lymphocytes, anaemia
  10. Immunological - anti ds-DNA, lupus anticoagulant, cardiolupin, low complement
  11. ANA test
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3
Q

what is the characteristic sign of scleroderma

A

Thickening and reddening of skin (morphea)

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4
Q

Types of scleroderma

A
  1. Localised - just affects skin (morphea or linear skin changes)
  2. Systemic - limited cutaneous systemic sclerosis and diffuse cutaneous systemic sclerosis
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5
Q

What is morphea

A

Discoloured oval patches on skin
Usually itchy
Can appear anywhere on body
Hairless and shiny

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6
Q

What is linear localised scleroderma

A

Thickened skin in lines along face, scalp, legs or arms

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7
Q

What is limited cutaneous systemic sclerosis

A

Milder form which only affects skin on the hands, lower arms, feet, lower legs and face - can eventually affect lungs and GIT
CREST Syndrome

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8
Q

What does CREST stand for

A 
Calcium deposits 
Raynaud's
Esophageal reflux
Sclerodactyly 
Telangiectasia
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9
Q

What is diffuse cutaneous systemic sclerosis

A

Scleroderma more likely to affect internal organs, skin changes can affect whole body
Involves other symptoms which come on suddenly - weight loss, fatigue, joint pain and stiffness

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10
Q

Complications of limited scleroderma

A

Pulmonary HTN (small blood vessels in lungs become fibrosed)

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11
Q

Complications of diffuse scleroderma

A

Pulmonary fibrosis
Renal crisis
Small bowel bacterial overgrowth

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12
Q

What age group does scleroderma occur in

A

30-50

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13
Q

Peak onset of Sjögrens

A

40-50

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14
Q

Symptoms of Sjögrens

A 
Dry mouth and lacrimal glands
Fatigue
Fever
Myalgia 
Arthralgia
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15
Q

Complications of Sjögrens

A 
Lymphoma 
Neuropathy 
Purpura
Interstitial lung disease
Renal tubular acidosis
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16
Q

Types of autoimmune myositis

A

Polymyositis - affects many muscles

Dermatomyositis - affects muscles and causes a rash

17
Q

Signs of dermatomyositis

A

Gottron’s Papules - discrete red areas over knuckles

Heliotrope rash - red or purplish rash occurring around eyes, limbs or trunk

18
Q

Complications of autoimmune myositis

A

Cancer

Interstitial lung disease

19
Q

Giant cell arteritis classification criteria

A 
>50 years at onset
New headache
Temporal artery tenderness / reduced pulsation 
ESR>50
Abnormal temporal biopsy
20
Q

What are the different types of ANCA associated vasculitis

A
  1. Granulomatosis with polyangitis (Wegener’s)
  2. Microscopic polyangitis
  3. Eosinophilic granulomatosis with polyangitis (Churg-Strauss)
21
Q

What is granulomatosis with polyangitis

Where does it affect in the body

A

Necrotising granulomatous inflammation

Upper and lower respiratory tract, affecting mostly small to medium vessels

22
Q

What is microscopic polyangitis

A

Necrotising vasculitis with few or no immune deposits
Mostly affects small vessels
Pulmonary capillarities common
NO Granulomatous inflammation

23
Q

What condition is common in granulomatosis with polyangitis and microscopic polyangitis

A

Necrotising glomerulonephritis

24
Q

What is eosinophilic granulomatosis with polyangitis

What conditions is it associated with

A

Eosinophil rich and necrotising granulomatous inflammation often involving small to medium vessels in the respiratory tract
Associated with asthma and eosinophilia

25
Q

What makes ANCA more frequent in eosinophilic granulomatosis with polyangitis

A

When glomerulonephritis is present

26
Q

What is ANCA

A

Anti-neutrophil cytoplasmic antibody (an abnormal antibody in the blood) that attack proteins within neutrophils

27
Q

Antibodies positive in SLE

A

dsDNA antibodies
Smith antibodies
anti-nuclear antibodies (Ro)

28
Q

Antibodies positive in scleroderma

A

Scl-70

Centromere

29
Q

Antibodies positive in polymyositis

A

anti-Jo-1

30
Q

Antibodies positive in Sjögrens disease

A

Anti-nuclear antibodies (Ro)

Anti-La

31
Q

How is lupus nephritis classed

A 
I - minimal mesangial 
II - mesangial proliferative
III - focal 
IV - diffuse
V - membranous 
VI - advanced sclerosing
32
Q

Treatment for mild organ threat in multi system autoimmune diseases

A

Hydroxychloroquine

33
Q

Treatment for moderate organ threat in multi system autoimmune diseases

A

Azathiopurine
Methotrexate
Mycophenolate

34
Q

Treatment for severe organ threat in multi system autoimmune diseases

A

Cyclophosphamide

Rituximab

E - MSK (29 decks)

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