multi system autoimmune conditions Flashcards by aryan gupta

Who is lupus more common in

Females 9:1

Afro-carribean>asian>caucasion

People with a family history of Lupus

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What is the common age that lupus occurs

15-50 years old

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What are the risk factors for Lupus

Genetic factors - family history

Hormonal factors

Environmental factors e.g UV, drugs and infections

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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

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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

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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

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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

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What are the autoantibodies that are screened for in lupus

ANA - anti-nuclear antibodies - high sensitivity but low specificity

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What other conditions have a positive ANA - anti-nuclear antibodies

normal people

rheumatoid arthritis

infection

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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

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What is scleroderma

Fibrosis of the skin

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What is limited cutaneous systemic sclerosis

The skin fibrosis is limited to distal to the elbows and knees

Skin fibrosis at head as well

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What is diffuse cutaneous systemic sclerosis

Skin fibrosis - both trunk and proximal involvement

Skin fibrosis at head as well

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What is localised scleroderma associated with

A good prognosis

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Who does systemic sclerosis commonly occur in

30-50 years

Females more common then males

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What can cause systemic sclerosis

Environmental:
silica dust
Industrial solvents
viral infection - e.g cytomegalovirus

Genetic predisposition

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What are the 3 main mechanisms which happen in systemic sclerosis

vascular damage in the microcirculation
Immune system activation/ inflammation
Fibrosis

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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

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What is the antibody found in limited systemic scleroderma

Anti centromere antibodies - specific for limited

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What is the antibodies found in diffuse systemic scleroderma

Anti Scl70 antibodies - exclusive for diffuse

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What are the main complications in limited systemic scleroderma

Pulmonary hypertension and GI issues such as oesophageal dysmotility

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What are the complications of diffuse systemic scleroderma

pulmonary fibrosis
renal crisis
small bowel bacterial overgrowth

Patients appear very ill early on

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What is sjogren’s syndrome

dry mucosal surfaces due to exocrine glands being affected due to rheumatoid arthritis or other connective tissue disorders

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Who does sjpgrens syndrome more commonly occur in

40-50 year olds

Female 9:1

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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