multi system autoimmune conditions Flashcards
Who is lupus more common in
Females 9:1
Afro-carribean>asian>caucasion
People with a family history of Lupus
What is the common age that lupus occurs
15-50 years old
What are the risk factors for Lupus
Genetic factors - family history
Hormonal factors
Environmental factors e.g UV, drugs and infections
What happens in lupus
There is an immune response against endogenous nuclear antigens - break in immunological tolerance
This results in immune complex formation which activates the compliment - T cells activate B cells which produce autoantibodies and cytokines which causes tissue injury
What are the symptoms of lupus
Alopecia - hair loss
Nervous system issues
butterfly rash
photosensitivity
purpura and urticuaria
chest pain
abdominal pain
necrosis of the pain and artitis in small joints
myopathy
Cardiac issues
Anaemia, leukopenia and thrombocytopenia
What are the classifications for lupus
Patient requires at least 4 for lupus classification
Malar rash - butterfly rash
Discoid rash - alopecia included
photosensitivity
oral ulcers
arthritis
serositis - pleurisy or pericarditis
Renal issues
neurological - unexplained seizures or psychosis
Haematological - low WCC, platelets, lymphocytes and haemolytic anaemia
Immunological - anti ds-DNA, Sm, cardiolipin, lupus anticoagulant, low compliment
ANA - antinuclear antibodies
When should a diagnosis of lupus be considered
Women of childbearing age
Constitutional symptoms - fever, weight loss, malaise and fatigue
Skin rash / stomatitis
Arthritis
pleuritic chest pain
renal disease
cytopenia
What are the autoantibodies that are screened for in lupus
ANA - anti-nuclear antibodies - high sensitivity but low specificity
What other conditions have a positive ANA - anti-nuclear antibodies
normal people
rheumatoid arthritis
MS
infection
What are the autoantibodies that should be checked in lupus
ANA - anti-nuclear antibodies
Anti- ds DNA - anti- double stranded DNA - high specificity but is raised by a number of inflammatory conditions
Anti-Sm - highly specific for lupus but not that sensitive
Anti-Ro - risk of foetal congenital heart block
Antiphospholipid antibodies - anti-cardiolipin and lupus anticoagulant - associated with thrombosis and miscarriages
What is scleroderma
Fibrosis of the skin
What is limited cutaneous systemic sclerosis
The skin fibrosis is limited to distal to the elbows and knees
Skin fibrosis at head as well
What is diffuse cutaneous systemic sclerosis
Skin fibrosis - both trunk and proximal involvement
Skin fibrosis at head as well
What is localised scleroderma associated with
A good prognosis
Who does systemic sclerosis commonly occur in
30-50 years
Females more common then males
What can cause systemic sclerosis
Environmental:
silica dust
Industrial solvents
viral infection - e.g cytomegalovirus
Genetic predisposition
What are the 3 main mechanisms which happen in systemic sclerosis
vascular damage in the microcirculation
Immune system activation/ inflammation
Fibrosis
What is the common presentation of systemic scleroderma
Reynauds’s - vasospasm that causes decrease blood flow to the fingers – fingers become white, then blue, then red
What is the antibody found in limited systemic scleroderma
Anti centromere antibodies - specific for limited
What is the antibodies found in diffuse systemic scleroderma
Anti Scl70 antibodies - exclusive for diffuse
What are the main complications in limited systemic scleroderma
Pulmonary hypertension and GI issues such as oesophageal dysmotility
What are the complications of diffuse systemic scleroderma
pulmonary fibrosis
renal crisis
small bowel bacterial overgrowth
Patients appear very ill early on
What is sjogren’s syndrome
dry mucosal surfaces due to exocrine glands being affected due to rheumatoid arthritis or other connective tissue disorders
Who does sjpgrens syndrome more commonly occur in
40-50 year olds
Female 9:1
What is the presentation of Sjorgren’s syndrome
Severely dry eyes and mouth
Parotid gland enlargement
Some have systemic symptoms - fatigue, fever, myalgia, arthralgia and dry skin
What antibodies are detected in Sjorgen’s syndrome
Anti RO and anti La (SSB_ antibodies
how is Sjogren’s syndrome diagnosed
Salivary gland biopsy
What are the complications of Sjorgen’s syndrome
lymphoma
neuropathy
cutaneous vasculitis
interstitial lung disease
renal tubular acidosis
Who is autoimmune myositis more common in
females
50-60 years old
What are the two types of autoimmune myositis
Polymyositis and dermatomyositis
What is the difference between polymyositis and dermatomyositis
Dermatomyositis has some skin involvement while polymyositis is limited to the muscle
Who is dermatomyositis more common in
Younger patients so can present in childhood
Who does polymyositis more commonly affect
The older age groups
What is the common complication of autoimmune - myositis
Increased risk of malignancy that should be screened
how does myositis present
Symmetrical, proximal muscle weakness
raised CK level - creatine kinase
Interstitial lung disease
What is the classical sign seen in dermatomyositis
Gottron’s papules - Erythematous rashes over the small hand joints
Heliotrope rash - peri-orbital oedema - rash on face but covers the nasolabial folds which separates the rash from the lupus butterfly rash
What investigations are done for myositis
Electromyogram
MRI - shows muscle oedema in myositis
Muscle biopsy - gold standard diagnostic test
What conditions are anti Jo1 antibodies found in and what does it increase the risk of
Myositis - increases risk of interstitial lung disease
What is vasculitides
inflammation in the blood vessel wall
What are the examples of large vessel vasculitides
Takayasu arteritis
Giant cell arteritis
Who does takayasu arteritis commonly affect
Young women from far east asia - less than 50 usually
What does takayasu arteritis present with
arteriole stenosis - carotid artery, subclavian, lower limbs
Who does giant cell arteritis affect
Northwest europe
Older age group over 50 years old
Women
What are thee two types of medium vessel vasculitides
polyarteritis nodosa
Kawasaki disease
What is polyarteritis nodosa
Causes stenosis and small aneurysms with the risk of hameorrhage
Affects the renal and mesenteric arteries
How does giant cell arteritis present
New sudden onset unilateral headache over the temporal area commonly
The pain is constant and only partially responds to analgesia
Jaw claudication - pain when chewing which reduces when you stop chewing
Scalp tenderness
What is one of the major complications of giant cell arteritis
Irreparable visual loss
Other complications :
aortic aneurysms
arterial stenosis and limb ischaemia
stroke
What is the treatment for giant cell arteritis
Immediate high does prednisolone : 40-60 mg per day which is then gradually tapered
PPI - gastric protection
bone protection - steroids can cause osteoporosis
steroid sparing medication
What is the giant cell arteritis classification criteria and what score is required
3 of the following required :
Age of onset over 50
new headache
temporal artery tenderness or reduced pulsation
ESR equal or greater than 50 - inflammation marker
Abnormal temporal biopsy
What is the issue of temp-oral artery biopsy in giant cell arteritis
The inflammation has skip lesions which means if you biopsy during one of the skips, you will get a false negative
What gives diagnosis of giant cell arteritis
Ultrasound doppler of the temporal artery which shows oedema in the wall of the artery if there is giant cell arteritis
What are common presentations in ANCA associated vasculitis
saddle nose
lung haemorrhage
skin vasculitis
How is granulomatosis with polyangiitis (Wegner’s) characterised
Necrotising granulomatous inflammation
What is the classical presentation of granulomatous with polyangiitis (Wegner’s)
Upper and lower respiratory tract affected
Hearing loss
sinusitis
haemoptysis
Necrotising glomerulonephritis is common
What is normally checked for in granulomatosis with polyangiitis (Wegner’s)
cANCA and anti PR3 antibodies
What is seen in microscopic polyangitis
Necrotising vasculitis affecting the small vessels
No granulomatous inflammation
Commonly renal and pulmonary involvement
Pulmonary haemorrage with renal failure is common presentation
What is looked for in microscopic polyangitis
pANCA and anti MPO antibodies
What is eosinophilic granulomatosis with polyangiitis characterised by
Eosinophil rich and necrotising granulomatous inflammation involving the respiratory tract
What is eosinophilic granulomatosis polyangiitis associated with (Churg strauss)
Late onset asthma
nasal polyps
eosinophilia
What is the common complications of eosinophilic granulomatosis polyangiitis
neurological involvement is most common
