Systemic Autoimmune Diseases

Question 1

What are the major CT disease?

Answer 1

SLE
Scleroderma
Sjogren’s Syndrome
Auto-immune myositis
Mixed CT disease

Question 2

What are the major Systmic Vasculitides

Answer 2

GCA
GPA (Wegener’s)
Microscopic Polyangiitis
Churg-Strauss

Question 3

Who gets SLE?

Answer 3

Women mostly
15-50yrs

More common in afro-caribbeans > Asians > caucasian

Question 4

What criteria are needed to diagnose SLE?

Answer 4

4 or more of:

• Malar Rash
• Discoid rash
• Photosensitivity
• Oral ulcers
• Arthritis
• Serositis
• Renal (proteinuria or cellular casts)
• Neuro (seizures or psychosis)
• Haemotology (Low WCC, platelets, lymphocytes or haemolytic anaemia)
• Immunological
• ANA

Question 5

What is included in the “immunological” criteria for SLE?

Answer 5

Anti ds-DNA Abs
Anti-SM Abs
Anti Cardiolipin Abs
Lupus Anticoagulant
Low complement

Question 6

Other than the primary criteria, what other symptoms/signs can come from SLE?

Answer 6

Alopecia
Fever
Depression
Vasculitis, purpura & Urticaria
Pleural effusion & Pulm fibrosis
Raynaud’s
Aseptic necrosis of hip
Myopathy
Abdo Pain
Aortic Valve Lesions
Cranial nerve lesions, peripheral neuropathy and ataxia

Question 7

Who gets Scleroderma?

Answer 7

Mostly female
Onset 30-50yrs

Question 8

What are the types of scleroderma?

Answer 8

Localised (aka Morphea)

Generalised (limited or Diffuse)

Question 9

What does localised scleroderma look like?

Answer 9

localised patches of hardened skin that are smooth and shiny.

Usually on the trunk,
Painless

Question 10

What does limited SCleroderma look like?

Answer 10

Thickening of skin and colour change in face and extremities along with Raynaud’s

CAn progress to affect gut –> heartburn & difficulty swallowing

Question 11

What do you see in diffuse scleroderma?

Answer 11

Affects the whole body, with potential to hit the heart, lungs and kidneys

Expect fatigue, joint pain & stiffness

Question 12

What is the general presentation of Scleroderma?

Answer 12

hardening of the skin
swelling of the hands and feet
joint pain
stiffness
Raynauds’ Syndrome.

All due to excess collagen

Question 13

What are the major complications of scleroderma?

Answer 13

Limited –> Pulm Hypertension

Diffuse –> Pulm fibrosis, renal crisis and small bowel bacterial overgrowth

Question 14

Who gets Sjogren’s Syndrome?

Answer 14

Mostly women
40-50yrs

Question 15

What’s the classic presentation of Sjogren’s Syndrome?

Answer 15

Dry eyes & mouth
Parotid Enlargement
~Systemic upset incl fever, fatigue, myalgia & arthralgia

Question 16

Complications of Sjogren’s Syndrome?

Answer 16

Lymphoma
Neuropathies (Cranial nerve, peripheral, fits etc)
Purpura
Interstitial Lung Disease
Renal Tubular Acidosis

Question 17

Auto-immune myositis is very rare. how does it present?

Answer 17

Symmetrical diffuse proximal muscle weakness

Dermatomyositis can come with Gottron’s papules (hands) and heliotrope rash
(face)

Question 18

What’s found in Mixed CT Disease?

Answer 18

Soft tissue swelling
Raynaud’s
Myositis
Arthralgia

Question 19

GCA affects the large arteries, what do you need to diagnose it?

Answer 19

3+ of:
- Age >50yrs at onset
- New headache
- Temporary art tenderness
- ESR >50
- Abnormal temporal biopsy

Question 20

Which conditions are Anca associated Vasculitis?

Answer 20

Wegeners (GPA)
Micrsocopic Polyangiitis
Eosinophilic Granulomatosis with polyangiitis

Question 21

What happens in GPA?

Answer 21

Necrotising granulomatous inflammation

Occurs mainly in Upper & Lower resp tract along with necrotising glomerulonephritis

Question 22

What’s different about MPA from GPA?

Answer 22

MPA doesn’t include immune deposites or granulomatous inflammation

Instead you get necrotising vasculitis of small vessels and necrotising glomerulonephritis. Sometimes with pulm capillaritis

Question 23

What is Churg Strauss?

Answer 23

Eosinophilic Granulomatosis with Polyangiitis (EGPA)

Eosinophil rich necrotising granulomatous inflammation of small to med vessles

Mostly resp tract

Ass with asthma and eosinophilia

Question 24

AAVs can lead to what kind of complications?

Answer 24

Hearing loss
Renal failure
Lung damage
Malignancy
Nerve damage

Question 25

What ANA profiles are targeted in SLE?

Answer 25

dsDNA
Ro
Sm

Question 26

What ANA profiles are targeted in Scleroderma?

Answer 26

Scl-70
Centromere

Question 27

What ANA profiles are targeted in polymyositis?

Answer 27

Jo-1

Question 28

What ANA profiles are targeted in Sjogren’s disease?

Answer 28

Ro
La

Question 29

What other tests are relevant to these conditions?

Answer 29

FBC
U&Es + Urinalysis
CXR
ECG
ESR/CRP
aPTT

Renal biopsy for nephritis

Question 30

What are the stages of Lupus Nephritis?

Answer 30

1 - minimal mesangial
2 - mesangial proliferative
3 - focal
4 - diffuse
5 - membranous
6 - advanced sclerosing

Question 31

How do we treat these conditions?

Answer 31

Mild - Hydroxychloroquine
Mod - Azathioprine, MTX or Mycophenolate
Severe - Cyclophosphomide or Rituximab

CCS can be used an adjunct or for specific symptoms but are not general 1st line treatments

Question 32

Which antibodies are tested for in SLE patients who are pregnant and why?

Answer 32

Anti-Ro
Anti-La

They are both indicative of a high risk of neonatal Lupus

Question 33

Try using this mnemonic to remember all the criteria for SLE: Dopamine Rash

Answer 33

Discoid Rash
Oral Ulcer
Photosensitvity
Arthritis
Malar Rash
Immunological (Anti-sm, anti-dsDNA, Anti-phospholipid, anti-ro & anti-la)
Neuro symptoms (Psychosis, seizure, personality)
ESR (raised)

Renal (Proteinuria or cellular casts)
ANA
Serositis (Pleurisy & Pericarditis)
Haematology (haemolytic anaemia, thrombocytopenia, leucopenia)