Multi-System Autoimmune Disease Flashcards
What is Systemic Erythematous Lupus (SLE)?
Chronic autoimmune disease due to type 3 hypersensitivity reaction, characterised by reddening of the skin and generalised symptoms affecting multiple organs, due to widespread vasculitis
What can trigger SLE?
Drug induced
- Isoniazid
- Phenytoin
- Hydralazine
- Procainamide
UV Radiation
Smoking
Oestrogen
Infection
What sex is more likely to be affected by SLE?
F>M
9:1
Oestrogen may be a trigger?
What is the most common age of onset of SLE?
15-50 years, teens into middle age
What ethnicity is more likely to be affected by SLE?
Afro-Caribbean’s > Asians > Caucasian
What is the criteria for SLE?
DOPAMINE RASH
Any 4 of the following
Discoid rash
- Raised, scarring, permanent marks, alopecia, appearing after sun-exposure
Oral ulcers
Photosensitivity
- Other rashes appearing after sun-exposure
Arthritis
- 2 joints at least, usually small joints, symmetrical, similar to RA
Malar rash
- ‘Butterfly rash’ over the cheeks, appearing after sun-exposure
Immunological
- Anti-phospholipid antibody
Neurological
- Unexplained seizures or psychosis
Renal
- Significant proteinuria
- Glomerulonephritis
Antinuclear Antibodies (ANA)
Serositis
- Pleurisy
- Pericarditis
Haematological
- Low WCC
- Low platelets
- Haemolytic anaemia
What investigations are used in SLE diagnosis?
Antinuclear antibodies
- Most sensitive but not specific, 99% are +
- Suggests connective tissue disease but not which one
Anti-dsDNA and anti-smith antibodies
- Highly specific for lupus
Anti-Ro and Anti-La antibodies
- Not specific for lupus
Anti phospholipid antibodies
- Suggests antiphospholipid syndrome present in 25% of patients
Anti-histone antibodies
- In drug induced lupus
Low C3 and C4 complement during active disease, useful in monitoring flares
>ESR, raised CRP suggests underlying infection
What antibody test is used for screening of SLE/most sensitive?
Antinuclear Antibodies
Sensitive but not specific
If ANA is negative, there is very low chance of SLE, so is a useful rule out test
Lead onto more specific tests
What antibody test is diagnostic of SLE/most specific?
Anti-dsDNA
A specific type of ANA
How SLE managed?
Hydroxychloroquine, first line for mild
Other immunosuppresants/DMARDs
Biologics for severe disease
Give complications of SLE
Cardiovascular disease, leading cause of death
Infection
Anaemia of chronic disease
Pericarditis
Pleuritis
Pulmonary fibrosis
Lupus nephritis
VTE
Recurret miscarriage
What condition is antiphospholipid syndrome associated with?
SLE
How does antiphospholipid syndrome present?
Recurrent venous/arterial thrombosis
- CVA
- Budd-Chiari syndrome
- DVT
Recurrent spontaneous miscarriage
Livedo reticularis
Purpura
Splinter haemorrhages
Valvular heart disease
What investigations are used in antiphospholipid syndrome diagnosis?
>APTT
FBC
- Thrombocytopenia
- Haemolytic anaemia
Anticardiolipin antibodies
Lupus Anticoagulant
How is antiphospholipid syndrome managed?
Daily low dose aspirin
Lifelong warfarin after initial VTE event
What is autoimmune dermatomyositis?
Inflammatory disease of skeletal muscle of unknown aetiology causing symmetrical, proximal muscle weakness and characteristic skin lesions
What conditions is autoimmune myositis associated with?
RA
Malignancy
How does myositis present?
Muscle weakness
- Symmetrical
- Diffuse
- Proximal
Associated with malignancy, as can be a paraneoplastic syndrome
Dysphagia
Dyspnoea
Raynauds
Photosensitivity, specifically rash on eyelids
Dermatomyositis
- Gottron’s papules, erythematous scaly lesions affecting the dorsum of the hands
- Heliotrope rash
What investigations are used in myositis diagnosis?
>CK
Antibodies
- ANA
- Anti-Jo-1 Antibody
Muscle biopsy
How is myositis managed?
High dose glucocorticoids
Azathioprine or Methotrexate
Immunoglobulin
Physiotherapy
Give complications of myositis
Malignancy
Interstitial lung disease
What is Sjogren’s Syndrome?
Chronic autoimmune condition in which the immune system attacks various exocrine glands, causing fibrosis
What causes Sjogren’s Syndrome?
Primary
Secondary to other connective tissue disease
- Scleroderma
What sex is more likely to be affected by Sjogren’s?
F>M
9:1
What is the age of onset of Sjogren’s?
Middle age
40-50
How does Sjogren’s present?
Dry eyes
Dry mouth
- Unable to eat certian foods such as bread
Oral pain
Parotid gland enlargement
Vaginal dryness
Systemic upset
- Fatigue
- Fever
- Myalgia
- Arthralgia
- Arthritis
What investigations are used in Sjogren’s diagnosis?
>ESR
Antibodies
- Anti-Ro and Anti-la, most specific
- ANA
- RF
Schrimer’s Test
What is Schrimer’s Test?
Uses filter paper under the lower eyelid to measure tear production
How is Sjogren’s managed?
Artificial tears/eye drops
Mouthwash
Diet changes
Give complications of Sjogrens
Lymphoid malignancy/lymphoma
Renal tubular acidosis
What is polymyalgia rheumatica?
Chronic inflammatory condition of the elderly characterised by joint stiffness and pain in surrounding tissues along with weakness of proximal muscles
What age group does polymyalgia rheumatica affect?
Elderly
What sex is more likely to be affected by polymyalgia rheumatica?
F>M
2:1
What causes polymalgia rheumatica?
Genetic
- HLA-DR4
Associated conditions
- Giant Cell Arteritis
How does polymyalgia rheumatica present?
Sudden onset of shoulder and pelvic girdle stiffness
- Starts on one side but progresses to other side within weeks
- At least 45 minutes in morning
Pain
- Worsens with movement
- Disturbs sleep
Anaemia
Malaise
Weight loss
Fever
Depression
No true weakness of limb girdles
What investigations are used in polymyalgia rheumatica diagnosis?
Usually a clinical diagnosis, investigatons to rule out differentials
>ESR and >CRP
FBC
- Normocytic normochromic anaemia
Creatine Kinase normal, unlike conditions that affect muscle
How is polymyalgia rheumatica managed?
Corticosteroids
- Initially started on 15mg/day prednisolone
- Begin to reduce after 3-4 weeks
What is giant cell arteritis?
Giant cell granulomatous inflammation of the arteries of the face and scalp, causing narrowing which can lead to infarction of the brain and optic nerve
What is the classification criteria for giant cell arteritis?
3 of the following
Age at onset > 50 years
New headache
- Diffuse
- Persistent
- Severe
Temporal artery tenderness/reduced pulsation
ESR > 50 (>CRP)
Abnormal temporal biopsy
Jaw claudication, particularly when chewing
Fever
What investigations are used in giant cell arteritis diagnosis?
>CRP
Greatly raised ESR
FBC
- Normochromic normocytic anaemia
Superficial temporal biopsy
- Skip lesions
How is giant cell arteritis managed?
High dose 60mg prednisolone, continue even if biopsy returns as normal due to skip lesions
Bisphosphonates, for risk of osteoporosis
Urgent opthalmology review
IV methylprednisilone if blindness has occurred
Note, if co-existing infection steroid dose should be maintained or decreased
Give complications of giant cell arteritis
Blindness, due to ischaemic optic neuritis
Stroke
Aortic aneurysm
Heavily associated with polymyalgia rheumatica
What is Scleroderma/systemic sclerosis?
Chronic autoimmune condition characterised by over-production of collagen resulting in hardened sclerotic skin and other connective tissues
What sex is more likely to be affected by Scleroderma?
F>M
3:1
Describe limited cutanenous scleroderma
Raynaud’s may be first sign
Scleroderma affects face and distal limbs predominately
Associated with anti-centromere antibodies
Subtype of limited systemic sclerosis is CREST syndrome
Associated with pulmonary hypertension
Describe diffuse cutaneous scleroderma
Scleroderma affects trunk and proximal limbs predominately
Associated with scl-70 antibodies
Most common cause of death is now respiratory involvement (interstitial lung disease and pulmonary arterial hypertension)
Other complications include renal disease and hypertension
Poor prognosis
What investigations are used in scleroderma diagnosis?
Antibodies
- ANA
- Scl-70 antibodies for diffuse
- Anti-centromete antibodies for central/limited
How is scleroderma managed?
Preventative
- Handwarmers and battery powered gloves
Raynaud’s
- CCBs
- Vasodilators
Acid reflux
- Omeprazole
Arthralgia
- Hydroxychloroquine/DMARDS
What is CREST syndrome?
Calcinosis
Raynaud’s
Oesophageal dysmotility/acid reflux
Sclerodactyly
Telangiectasia/spider naevi
Associated with anti-centromere antibodies
What type of scleroderma is CREST syndrome associated with?
Limited scleroderma
What is Wegner’s Granulomatosis?
Systemic vascular disease characterised by necrotizing granulomas in the respiratory tract and focal necrotizing glomerulonephritis
How does Wegner’s present?
Cough
Pleurisy
SOB
Haemoptysis
General malaise
Haematuria
Arthralgia
Purulent/bloody nasal discharge
Purpura
Oral ulceration
Pleural effusion
Conjunctivitis
What investigations are used in Wegners diagnosis?
cANCA antibodies
FBC
- Leucocytosis
- Thrombocytosis
Blood Film
- Schistocytes
- Burr cells
Urine
- Proteinuria
- Haematuria
- Red cell casts
Imaging
Biopsy: Granulomatous changes
How is Wegners managed?
Immunosuppressants
Corticosteroids
What is Takayasu’s Arteritis?
Chronic, progressive, inflammatory, occlusive disease of the aorta and its branches
What group of people are more likely to be affected by Takayasus?
Young females
How does Takayasus present?
Systemic illness
Tenderness over palpable arteries
Bruits
Loss of pulses
Claudication
How is Takayasus managed?
Steroids and immunosuppressants
Angioplasty/surgical repair for stenosed vessels
What is Raynaud’s Syndrome?
Phenomenon characterised by spasm of arteries causing episodes of reduced blood flow to, typically, the fingers, resulting in colour change, pain and parathesia
What causes Raynaud’s syndrome?
Primary/Idiopathic
Secondary to connective tissue disease
- Scleroderma
- Mixed connective tissue disease
- Lupus
- Sjogren’s
Secondary to other
- Myeloma/paraproteinemia
- Leukaemia
- Cervical rib
Drugs
- COCP
What triggers episodes of Raynaud’s syndrome?
Cold
Stress
How does Raynaud’s present?
Typically young women with bilateral symptoms if primary Raynauds
Pain
Colour change, typically red to white to blue
Parathesia
How is Raynaud’s managed?
CCBs, such as nifedipine
IV prostacyclin (epoprostenol) infusions, effects may last several weeks/months
What is fibromyalgia?
Syndrome of unknown cause characterised by widespread pain throughout the body with tender points at specific anatomical sites
What sex is fibromyalgia most common in?
F>M
Give features of fibromyalgia
Chronic pain at multiple site, sometimes ‘pain all over’
Lethargy
Cognitive impairment, known as ‘fibro fog’
Sleep disturbance
Headaches
Dizziness
How is fibromyalgia managed?
Aerobic exercise
CBT
Medication
- Pregabalin
- Duloxetine
- Amitriptyline
What ANCA associated vasculitis is associated with pANCA?
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
What ANCA associated vasculitis is associated with cANCA?
Granulomatosis with polyangiitis
Give features of ANCA associated vasculitis
Renal impairment, haematuria
Dyspnoea, haemoptysiis
Fatigue
Weight loss
Fever
Vasculitic rash. present only in a minority of patients
ENT symptoms
Sinusitis
What is Behcet syndrome?
Autoimmune inflammation of arteries and veins characterised by the triad of oral ulcers, genital ulcer and anterior uveitis
How does Behcet syndrome present?
Triad of oral ulcers, genital ulcers and anterior uveitis
DVT and phlebitis
Arthritis
Erythema nodosum
Abdominal pain and diarrhoea
Acute pericarditis
