Multisystem autoimmune **marty pls review** Flashcards
Give 5 examples of connective tissue diseases:
- Systemic Lupus Erythematosus
- Scleroderma
- Sjogren’s syndrome
- Auto-immune myositis
- Mixed connective tissue disease
Give 4 examples of systemic vasculitis:
- Giant cell arteritis
- Granulomatosis polyangiitis (Wegeners)
- Microscopic polyangiitis
- Eosinophilic granulomatosis polyangiitis (Churg-Strauss)
How is a diagnosis of systemic disease made?
- Cardinal clinical features: History & Exam
- Immunology
- Imaging
- Tissue
- Exclusion of differential diagnosis
Systemic Lupus Erythematosus [SLE]: What are it’s epidemiological characteristics?
- Disease of the young
- 9:1, Female:Male
- UK incidence: 4/100,000
- Onset: 15-50 years
What is the classification criteria for SLE?
Any 4 of the below:
- Malar rash (butterfly rash)
- Discoid rash (raised, scarring, permanent marks, alopecia)
- Photosensitivity
- Oral ulcers
- Arthritis (2 joints at least)
- Serositis (pleurisy or pericarditis)
- Renal (significant proteinuria or cellular casts in urine)
- Neurological (unexplained seizures or psychosis)
- Haematological (low WCC, platelets, lymphocytes, haemolytic anaemia)
- Immunological (anti ds-DNA, SM, cardiolipin, lupus anticoagulant, low complement)
- ANA
What are the specific skin manifestations of SLE?
- DDx butterfly: rosacea, mitral stenosis
- Discoid: Scaly centre, dark rim
What is scleroderma?
Uncommon condition that results in hard, thickened areas of skin and sometimes problems with internal organs and blood vessels
Epidemiology of scleroderma?
- UK Prevalence: 24/100,000
- UK Incidence: 10/1,000,000
- Onset: 30-50 years
- Female:Male 3:1
What are the two main subtypes of scleroderma?
- Limited cutaneus scleroderma: presents with raynauds phenomonen
- Diffuse cutaneus scleroderma: more likely to have internal organ involvement
What are complications with limited and diffuse scleroderma?
- Limited: Pulmonary hypertension
- Diffuse: Pulmonary fibrosis, Renal crisis, small bowel bacteria overgrowth
What is sjogren’s syndrome? Epidemiology?
Long-term autoimmune disease that affects the body’s moisture-producing glands.
- Prevalence: 1 in 100
- Incidence: 4 in 100,000
- Onset: 40-50yrs
- Female : Male 9:1
What are common symptoms of sjogren’s syndrome?
- Dry eyes and mouth
- Parotid gland enlargment
- 1/3 have systemic upset
- fatigue
- fever
- myalgia
- arthalgia
Serious complications of sjogrens?
- Lymphoma
- Neuropathy
- Purpura
- Interstitial lung disease
- Renal tubular acidosis
What is auto-immune myositis?
VERY rare disease
- Muscle Weakness - symmetrical, diffuse, proximal
- Polymyositis
- Dermatomyositis
- Gottron’s papules (80%)
- Heliotrope rash (30-60%)
What are mixed connective tissue diseases normally inclusive of?
- Soft tissue swelling
- Raynauds
- Myositis
- Arthalgia
What is Giant Cell arteritis?
Giant cell arteritis (GCA) is a common form of vasculitis in people aged 50 years or older. The extracranial branches of the carotid artery are usually affected.
What is GCA classification criteria?
- 3 of the following
- Age at onset ≥50 years
- New headache
- Temporal artery tenderness/reduced pulsation
- ESR≥50
- Abnormal temporal biopsy
What is a serious complication of GCS?
Irreversible blindness is the most common serious consequenc
What are ANCA associated vasculitis?
Anti-neutrophil cytoplasmic antibody (ANCA)
These are systemic vasculitis associated with the above:
- Granulomatosis with polyangiitis (Wegener’s)
- Microscopic polyangiitis
- Eosiniphilic granulomatosis with polyangiitis
They are very rare
What are the characteristcs of Wegeners?
- Necrotising granulomatous inflammation
- Usually involving the upper and lower respiratory tract
- Affecting predominantly small to medium vessels
- Necrotising glomerulonephritis is common
What are the characteristics of Microscopic polyangiitis (MPA)?
- 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
What are the features of Eosinophilic Granulomatosis with Polyangiitis (Churg Strauss) (EGPA)?
- 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
Marty please look at slides 41-51 and see if useful
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