Multisystem Autoimmune Disease Flashcards
name some autoimmune connective tissue diseases
systemic lupus erythematosus scleroderma Sjogren's syndrome auto-immune myositis mixed connective tissue disease
name some autoimmune systemic vasculitis diseases
giant cell arteritis
granulomatosis polyangiitis (Wegener’s)
microscopic polyangiitis
eosinophilic granulomatosis polyangiitis (Churg-Strauss)
diagnosis of autoimmune diseases
- Cardinal clinical features: history and exam
- Immunology
- Imaging
- Tissue
- Exclusion of differential diagnosis
mimics of autoimmune diseases
• Drugs – cocaine, minocycline, PTU • Infection – HIV, endocarditis, hepatitis, TB • Malignancy – lymphoma • Cardiac myxoma • Cholesterol emboli • Scurvy
SLE: UK prevalence
28/100,000
SLE: UK incidence
4/100,000
SLE: M:F
1:9
SLE: ethnicity
afro-caribbean>asain>caucasian
SLE: age of onset
15-50
SLE: classification criteria
any 4:
o Malar rash
o Discoid rash – raised, scarring, permanent marks, alopecia
o Photosensitivity
o Oral ulcers
o Arthritis (at least 2 joints)
o Serositis (pleurisy or pericarditis)
o Renal – significant proteinuria or cellular casts in urine
o Neurological – unexplained seizures or psychosis
o Haematological – low WCC, platelets, lymphocytes, haemolytic anaemia
o Immunological – anti ds-DNA, SM, cardiolipin, lupus anticoagulant, low complement
o ANA
scleroderma: UK prevalence
24/100,000
scleroderma: UK incidence
10/1,000,000
scleroderma: onset
30-50
scleroderma: M:F
1:3
scleroderma: complications
pulmonary hypertension
pulmonary fibrosis
renal crisis
smal bowel bacterial overgrowth
Sjogren’s syndrome: UK prevalence
1/100
Sjogren’s syndrome: UK incidence
4/100,000
Sjogren’s syndrome: onset
40-50
Sjogren’s syndrome: M”F
1:(
Sjogren’s syndrome: signs and symptoms
dry eyes and mouth parotid gland enlargement fatigue fever myalgia arthalgia
Sjogren’s syndrome: complications
lymphoma neuropathy purpura interstitial lung disease renal tubular acidosis
auto-immune myositis: prevalence
6/1,000,000
auto-immune myositis: symptoms
muscle weakness - symmetrical, diffuse, proximal polymyositis dermatomyositis Gottron's papules heliotrope rash
auto-immune myositis: complicatons
cancer
interstitial lung disease
auto-immune myositis: oveerlap syndromes (mixed connective tissue disease)
soft tissue swellings
Raynaud’s
myositis
arthralgia
name large vessel vasculitis
takayasu arteritis
giant cell arteritis
name medium vessel vasculitis
polyarteritis nodosa
kawasaki disease
name small vessel vasculitis: ANCA associated vasculitis
microscopic polyangiitis
granulomatosis with polyangitis
eosinophilic granulomatosis with polyangitis
name small vessel vasculitis: immune complex SVV
anti-GBM disease
cryoglobulinemic vasculitis
IgA vasculitis (Henoch-Schönlein)
Hypocomplementemic urticarial vasculitis (Anti-C1q vasculitis)
giant cell arteritis: classification criteria
3 of: age of onset >=50 new headache temporal artery tenderness/reduced pulsation EST >= 50 abnormal temporal biopsy
ANCA associated vasculitis: overall incidence
15/million
ANCA associated vasculitis: overall prevalence
150/million
Granulomatosis with Polyangiitis (Wegener’s): features
- Necrotising granulomatous inflammation
- Usually involving the upper and lower respiratory tract
- Affecting predominantly small to medium vessels
- Necrotising glomerulonephritis is common
Microscopic Polyangiitis: features
- Necrotising vasculitis, with few or no immune deposits, predominantly affecting small vessels
- Necrotising arteritis involving small and medium arteries may be present
- Necrotising glomerulonephritis is very common
- Pulmonary capillaritis often occurs
- Granulomatous inflammation is absent
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss): features
• Eosinophil rich and necrotising granulomatous inflammation often involving the respiratory
tract
• Necrotising vasculitis predominantly affecting small to medium vessels
• Associated with asthma and eosinophilia
• ANCA is more frequent when glomerulonephritis is present
ANA are found in what % of SLE?
99
ANA are found in what % of systemic sclerosis?
97
ANA are found in what % of poly/dermatomyositis?
40-80
ANA are found in what % of Sjogren’s syndrome?
48-96
ANA are found in what % of MCTD
100
ANA are found in what % of drug induced lupus
100
what conditions make ANA +ve unhelpful?
normal 30%
RA 30-40%
MS 25%
infection
disease specific ANA profile: SLE
dsDNA
Ro
Sm
disease specific ANA profile: scleroderma
Scl-70
centromere
disease specific ANA profile: polymyositis
Jo-1
disease specific ANA profile: Sjogren’s syndrome
Ro
La
lupus nephritis class I
minimal mesangial
lupus nephritis class II
mesangial proliferative
lupus nephritis class III
focal
lupus nephritis class IV
diffuse
lupus nephritis class V
membranous
lupus nephritis class VI
advanced sclerosing
treatment of mild autoimmune disease
hydroxychloroquine
treatment of moderate autoimmune disease
azathioprine
methotrexate
mycophenolate
treatment of severeautoimmune disease
cyclophosphamide
rituximab