Multi-system Autoimmune Diseases Flashcards
Name some connective tissue diseases
Systemic Lupus Erythematosis Scleroderma Sjogren's syndrome Auto-immune myositis Mixed connective tissue disease
Name some systemic vasculitides
- Giant cell arteritis
- Granulomatosis polyangiitis (Wegeners)
- Microscopic polyangiitis
- Eosinophilic granulomatosis polyangiitis (Churg-Strauss)
Describe the sequence of events in autoimmunity
- Initiating event + genetic susceptibility
- Breakdown of self-tolerance
- Autoreactivity
- Humoral +/- cellular
- Autoimmune disease
How do you generally diagnose autoimmune diseases?
Cardinal clinical features through history and exam
Immunology
Imaging
Tissue
Exclusion of differential diagnosis
What can mimic autoimmune disease?
Drugs - cocaine, minocyline, PTU
Infection - HIV, endocarditis, Hepatitis, TB
Malignancy - Lymphoma
Cardiac myxoma
Cholesterol emboli
Scurvey
What is the epidemiology of systemic lupus erythematosis?
prevalence, incidence, M:F, age of onset, ethnicity
UK prevalence 28/100,000
UK incidence 4/100,000
M:F = 1:9
Onset = 15-50 years
Significant ethnic diversity
-Afro-caribbeans > asian > caucasian
What can SLE cause?
Face:
- butterfly rash
- photosensitivity
General:
- fever
- depression
Skin:
- vasculitis
- purpura
- urticaria
Chest:
- pleurisy
- pleural effusion
- fibrosis
Raynaud’s phenomenon
Alopecia
Joints:
- aseptic necrosis of hip
- arthritis small joints
Nervous system:
- fits
- hemiplegia
- ataxia
- peripheral neuropathy
- cranial nerve lesions
Heart
- pericarditis
- endocarditis
- aortic valve lesions
Abdominal pain
Myopathy
Blood
- anaemia
- leucopenia
- thrombocytopenia
CAN CAUSE ANYTHING
What is the differential for a butterfly rash?
Rosacae
Mitral stenosis
SLE
What is the key difference between the arthritis in SLE and RA?
SLE -> fingers are bent but joints look normal on x-ray
RA -> joints are abnormal on x-ray
Why is urine dipstick so important in SLE?
Kidney problems are the things that will kill you
What is the epidemiology of scleroderma?
prevalence, incidence, age of onset, M:F
UK prevalence: 24/100,000
UK incidence: 10/100,000
Onset: 30-50 years
M:F = 1:3
What is morphea?
Morphea, also known as localized scleroderma, is a disorder characterized by excessive collagen deposition leading to thickening of the dermis, subcutaneous tissues, or both.
What are the 2 types of scleroderma?
Limited
Diffuse
What are the complications of the two types of scleroderma?
Limited:
-Pulmonary hypertension
Diffuse:
- Pulmonary fibrosis
- Renal crisis
- Small bowel bacterial overgrowth -> malabsorption
What is the epidemiology of sjogren’s syndrome?
prevalence, incidence, age of onset, M:F
Prevalence: 1 in 100
Incidence: 4 in 100,000
Onset: 40-50 years
M:F = 1:9
What are sicca symptoms?
Dryness of eyes and mouth
What does Sjogren’s syndrome effect?
Gastrointestinal
- dysphagia
- abnormal oesophageal mobility
Respiratory
-intestinal lung disease
Peripheral nervous system
- sensory neuropathy
- mononeuritis multiplex
CNS
- fits
- hemiplegia
- ataxia
- cranial nerve lesions
Kidney
-renal tubular acidosis
Skin
- palpable purpura
- Rayneud’s syndrome
joints:
-arthralgia
What are the signs and symptoms of Sjogren’s syndrome?
Dry eyes and mouth
Parotif gland enlargement
1/3 have systemic upset
- fatigue
- fever
- myalgia
- arthralgia
What are the complications of Sjogren’s syndrome?
Lymphoma Neuropathy Purpura Interstitial lung disease Renal tubular acidosis
What are the signs and symptoms of auto-immune myositis?
Muscle wakness
-Symmetrical, diffuse, proximal
Polymyositis (younger)
Dermatosyositis (older)
- Gottron’s papules (80%)
- Heliotrope rash (30-60%)
What are the complications of auto-immune myositis?
Polymyositis -> interstitial lung disease
Dermatosyositis -> cancer
What are the features of Mixed Connective Tissue Disease?
Soft tissue swelling
Raynaud’s
Myositis
Arthralgia
What vasculitides effect large vessels?
Takayasu Arteritis
Giant cell arteritis
What vasculitides effect medium vessels?
Polyarteritis nodosa
Kawasaki disease
Name 3 ANCA- associated vasculitides effecting small vessels
Microscopic Polyangiitis
Granulomatosis with polyangiitis
Eosinophilic Granulomatosis with Polyangiitis
Name some immune complex small vessel vasculitides
Anti-GBM disease
Cryoglobulinemic Vasculitis
IgA Vasculitis (Henoch-Schonlein)
Hypocomplementemic Urticarial Vasculitis (Anti-C1q Vasculitis)
What is the classification criteria for giant cell arteritis?
3 of the following:
- Age at onset 50 or over
- New headache
- Temporal artery tenderness/ reduced pulsation
- ESR 50 or above
- Abnormal temporal biopsy
What is Granulomatosis with Polyangiitis (Wegener’s)?
what is it, what does it effect?
Necrotising granulomatous inflammation
Usually involving the upper and lower respiratory tract
Affecting predominantly small to medium vessels
Necrotising glomerulonephritis is common
What is microscopic polyangiitis?
what is it, what does it effect, what sets it apart?
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 is eosinophilic granulomatosis with polyangiitis (Churg Strauss)?
(what is it, what does it effect, what is it associated with)
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
What is the treatment of multi-system autoimmune diseases?
Mild:
-Hydroxychloroquine
Moderate:
- Azathioprine
- Methotrexate
- Mycophenolate
Severe:
- Cyclophosphamide
- Rituximab
(often combination- particularly in giant cell arteritis)
