Crystal Athropathy Flashcards
What is vasculitis
Inflammation of the blood vessels
What causes small vessel vasculitis
Henoch-Schonlein purpura
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
Microscopic polyangiitis
Granulomatosis with polyangiitis (Wegener’s granulomatosis)
What causes medium vessel vasculitis
Polyarteritis nodosa
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
Kawasaki Disease
What causes larger vessel vasculitis
Giant cell arteritis
Takayasu’s arteritis
What generic features may lead you to suspect vasculitis
Purpura Joint and muscle pain Peripheral neuropathy Renal impairment Gastrointestinal disturbance (diarrhoea, abdominal pain and bleeding) Anterior uveitis and scleritis Hypertension
What is purpura
These are purple-coloured non-blanching spots caused by blood leaking from the vessels under the skin
What systemic features may be associated with vasculitis
Fatigue Fever Weight loss Anorexia (loss of appetite) Anaemia
What investigations should you if suspected vasculitus
- ESR/CRP
- Anti neutrophil cytoplasmic antibodies (ANCA): pANCA & cANCA
What is pANCA usually a sign of
Microscopic polyangiitis and Churg-Strauss syndrome
What is cANCA usually a sign of
Wegener’s granulomatosis
What is the management of vasculitis
- Depends on type, refer to rheumatologist
- Steroids
- Immunosupressants
What is Henoch-Schonlein purpura
- IgA vasculitis that commonly presents with a purpuric rash affecting the lower limbs or buttocks in children
- often triggered by an upper airway infection (e.g. tonsillitis) or a gastroenteritis.
- ash is caused by inflammation and leaking of blood from small blood vessels under the skin, forming purpura.
What are the four classic features of Henoch-Schonlein purpura
- purpura (100%)
- joint pain (75%)
- abdominal pain (50%)
- renal involvement (50%) IgA nephritis
What is the management of Henoch-Schonlein purpura
- supportive
- simple analgesia: abdo pain settles in a few days
- rest
- proper hydration
What is the prognosis of Henoch-Schonlein purpura
- without kidney involvement can expect to fully recover within 4-6 weeks
- 1/3 recurrence within 6 months
- 1% of patients develop end stage renal failure.
What is Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
- most associated with lung and skin problems, but can affect other organs such as kidneys.
- presents with severe asthma in late teenage years or adulthood.
- Characteristic finding: elevated eosinophil levels
What is Microscopic polyangiitis
- renal failure
- can also affect the lungs causing shortness of breath and haemoptysis.
What is Granulomatosis with polyangiitis (Wegener’s granulomatosis)
- Affect kidneys and lungs
- Lung symptoms: cough, wheeze and haemoptysis
- Kidney symptoms: Rapidly progressing glomerulonephritis.
What Upper resp tract findings can you see in Granulomatosis with polyangiitis (Wegener’s granulomatosis)
- nose bleeds (epistaxis)
- crusty nasal secretions
- hearing loss
- sinusitis.
- saddle shaped nose due to a perforated nasal septum.
What CXR findings might you see in Granulomatosis with polyangiitis (Wegener’s granulomatosis)
- consolidation- (misdiagnosed as pneumonia)
What is Polyarteritis Nodosa
- affects the medium sized vessels in locations such as the skin, gastrointestinal tract, kidneys and heart
- Can cause renal impairment, strokes and MI
- Associated with a rash called livedo reticularis
What can polyarteritis Nodosa be associated with
- Mainly Hepatitis B
- hepatitis C
- HIV
- No clear cause
What is Kawasaki Disease
- medium vessel vasculitis. It affects young children, typically under 5 years of age. There is no clear cause.
What are the key features of Kawasaki Disease
- Persistent high fever > 5 days
- Erythematous rash
- Bilateral conjunctivitis
- Erythema and desquamation (skin peeling) of palms and soles
- “Strawberry tongue” (red tongue with prominent papillae)
What is the treatment of Kawasaki’s Disease
- aspirin
- IV immunoglobulins.
What is the key complication of Kawasaki’s Disease
coronary artery aneurysms.
What is Takayasu’s arteritis
- large vessel vasculitis affecting the aorta, it’s branches and pulmonary arteries.
- large vessels and their branches can swell and form aneurysms or become narrowed and blocked.
- This leads to it’s other name of “pulseless disease”.
How does Takayasu’s arteritis present
- usually presents before the age of 40 years
- non-specific systemic symptoms: fever, malaise and muscle aches, or with more specific symptoms of arm claudication or syncope
How do you diagnose Takayasu’s arteritis
- CT or MRI angiography
- Doppler ultrasound of the carotids can be useful in detecting carotid disease.
What is a giant cell arteritis
- systemic vasculitis of the medium and large arteries
- strong link with polymyalgia rheumatica.
What is the main presentation of giant cell arteritis involving a headache
- Severe unilateral headache typically around temple and forehead
- Scalp tenderness my be noticed when brushing hair
- Jaw claudication
- Blurred or double vision
- Irreversible painless complete sight loss can occur rapidly
What systemic features may you see in giant cell arteritis
Fever Muscle aches Fatigue Weight loss Loss of appetite Peripheral oedema
How is giant cell arteritis diagnosed
Clinical presentation
Raised ESR: usually 50 mm/hour or more
Temporal artery biopsy findings
What is seen on temporal artery biopsy in giant cell arteritis
Multinucleated giant cells
What invetigations should you do if suspecting giant cell arteritis
- Temporal artery biopsy
- FBC: normocytic anaemia and thrombocytosis
- LFTs: Raised ALP
- CRP/ESR
- Duplex ultrasound of the temporal artery shows the hypoechoic halo sign
What is the immediate management of giant cell arteritis
- 40-60mg prednisolone per day Immediately without confirmed diagnosis (then taper)
- Review the response to steroids within 48 hours
- Aspirin 75mg daily decreases visual loss and strokes
- Proton pump inhibitor (e.g. omeprazole)
Who should a patient with giant cell arteritis be referred to?
- Rheumtologists
- Opthalmologists
- Vascular surgeons
What advice must you give patients on high dose steroids/addisons disease
DON’T – Don’t stop taking steroids abruptly. There is a risk of adrenal crisis.
S – Sick Day Rules.
T – Treatment Card.
O – Osteoporosis prevention with bisphosphonates and supplemental calcium and vitamin D.
P – Proton pump inhibitor for gastric protection.
What are early complications of giant cell arteritis
Vision loss Cerebrovascular accident (stroke)
What are the late complications of giant cell arteritis
Relapses of the condition are common
Steroid related side effects and complications
Cerebrovascular accident (stroke)
Aortitis leading to aortic aneurysm and aortic dissection
What is polymyalgia Rheumatica
- inflammatory condition that causes pain and stiffness in the shoulders, pelvic girdle and neck
- strong association to giant cell arteritis
Who usually suffers from polymyalgia Rheumatica
It usually affects old adults (above 50 years)
More common in women
More common in caucasians
What symptoms may indicate polymyalgia Rheumatica
- Bilateral shoulder pain that may radiate to the elbow
- Bilateral pelvic girdle pain
- Worse with movement
- Interferes with sleep
- Stiffness for at least 45 minutes in the morning
- Symptoms lasting more than 2 weeks
What are the differential diagnoses of Polymyalgia Rheumatica
Osteoarthritis Rheumatoid arhtirits Systemic lupus erythematosus Myositis (from conditions like polymyositis or medications like statins) Cervical spondylosis Adhesive capsulitis of both shoulders Hyper or hypothyroidism Osteomalacia Fibromyalgia
How is polymyalgia rheumatica diagnosed
- clinical presentation + response to steroids
- ESR/CRP may be raised but does not rule out if normal
What is the treatment for polymyalgia rheumatica
- Steriods for 1 week then assess effectiveness
- Assess 3-4 weeks after starting steroids. You would expect a 70% improvement
- Taper dose
- If symptoms recur, may need to increaser steroid
- can take 1-2 years to wean off
