Systemic Autoimmune Diseases Flashcards
What are the major CT disease?
SLE Scleroderma Sjogren's Syndrome Auto-immune myositis Mixed CT disease
What are the major Systmic Vasculitides
GCA
GPA (Wegener’s)
Microscopic Polyangiitis
Churg-Strauss
Who gets SLE?
Women mostly
15-50yrs
More common in afro-caribbeans > Asians > caucasian
What criteria are needed to diagnose SLE?
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
What is included in the “immunological” criteria for SLE?
Anti ds-DNA Abs Anti-SM Abs Anti Cardiolipin Abs Lupus Anticoagulant Low complement
Other than the primary criteria, what other symptoms/signs can come from SLE?
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
Who gets Scleroderma?
Mostly female
Onset 30-50yrs
What are the types of scleroderma?
Localised (aka Morphea)
Generalised (limited or Diffuse)
What does localised scleroderma look like?
localised patches of hardened skin that are smooth and shiny.
Usually on the trunk,
Painless
What does limited SCleroderma look like?
Thickening of skin and colour change in face and extremities along with Raynaud’s
CAn progress to affect gut –> heartburn & difficulty swallowing
What do you see in diffuse scleroderma?
Affects the whole body, with potential to hit the heart, lungs and kidneys
Expect fatigue, joint pain & stiffness
What is the general presentation of Scleroderma?
hardening of the skin swelling of the hands and feet joint pain stiffness Raynauds' Syndrome.
All due to excess collagen
What are the major complications of scleroderma?
Limited –> Pulm Hypertension
Diffuse –> Pulm fibrosis, renal crisis and small bowel bacterial overgrowth
Who gets Sjogren’s Syndrome?
Mostly women
40-50yrs
What’s the classic presentation of Sjogren’s Syndrome?
Dry eyes & mouth
Parotid Enlargement
~Systemic upset incl fever, fatigue, myalgia & arthralgia
Complications of Sjogren’s Syndrome?
Lymphoma Neuropathies (Cranial nerve, peripheral, fits etc) Purpura Interstitial Lung Disease Renal Tubular Acidosis
Auto-immune myositis is very rare. how does it present?
Symmetrical diffuse proximal muscle weakness
Dermatomyositis can come with Gottron’s papules (hands) and heliotrope rash
face
What’s found in Mixed CT Disease?
Soft tissue swelling
Raynaud’s
Myositis
Arthralgia
GCA affects the large arteries, what do you need to diagnose it?
3+ of:
- Age >50yrs at onset
- New headache
- Temporary art tenderness
- ESR >50
- Abnormal temporal biopsy
Which conditions are Anca associated Vasculitis?
Wegeners (GPA)
Micrsocopic Polyangiitis
Eosinophilic Granulomatosis with polyangiitis
What happens in GPA?
Necrotising granulomatous inflammation
Occurs mainly in Upper & Lower resp tract along with necrotising glomerulonephritis
What’s different about MPA from GPA?
MPA doesn’t include immune deposites or granulomatous inflammation
Instead you get necrotising vasculitis of small vessels and necrotising glomerulonephritis. Sometimes with pulm capillaritis
What is Churg Strauss?
Eosinophilic Granulomatosis with Polyangiitis (EGPA)
Eosinophil rich necrotising granulomatous inflammation of small to med vessles
Mostly resp tract
Ass with asthma and eosinophilia
AAVs can lead to what kind of complications?
Hearing loss Renal failure Lung damage Malignancy Nerve damage
