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
What makes ANCA more frequent in eosinophilic granulomatosis with polyangitis
When glomerulonephritis is present
26
What is ANCA
Anti-neutrophil cytoplasmic antibody (an abnormal antibody in the blood) that attack proteins within neutrophils
27
Antibodies positive in SLE
dsDNA antibodies Smith antibodies anti-nuclear antibodies (Ro)
28
Antibodies positive in scleroderma
Scl-70 | Centromere
29
Antibodies positive in polymyositis
anti-Jo-1
30
Antibodies positive in Sjögrens disease
Anti-nuclear antibodies (Ro) | Anti-La
31
How is lupus nephritis classed
``` I - minimal mesangial II - mesangial proliferative III - focal IV - diffuse V - membranous VI - advanced sclerosing ```
32
Treatment for mild organ threat in multi system autoimmune diseases
Hydroxychloroquine
33
Treatment for moderate organ threat in multi system autoimmune diseases
Azathiopurine Methotrexate Mycophenolate
34
Treatment for severe organ threat in multi system autoimmune diseases
Cyclophosphamide | Rituximab