Crystal Athropathy Flashcards

1
Q

What is vasculitis

A

Inflammation of the blood vessels

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2
Q

What causes small vessel vasculitis

A

Henoch-Schonlein purpura
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
Microscopic polyangiitis
Granulomatosis with polyangiitis (Wegener’s granulomatosis)

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3
Q

What causes medium vessel vasculitis

A

Polyarteritis nodosa
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
Kawasaki Disease

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4
Q

What causes larger vessel vasculitis

A

Giant cell arteritis

Takayasu’s arteritis

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5
Q

What generic features may lead you to suspect vasculitis

A
Purpura
Joint and muscle pain
Peripheral neuropathy
Renal impairment
Gastrointestinal disturbance (diarrhoea, abdominal pain and bleeding)
Anterior uveitis and scleritis
Hypertension
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6
Q

What is purpura

A

These are purple-coloured non-blanching spots caused by blood leaking from the vessels under the skin

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7
Q

What systemic features may be associated with vasculitis

A
Fatigue
Fever
Weight loss
Anorexia (loss of appetite)
Anaemia
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8
Q

What investigations should you if suspected vasculitus

A
  • ESR/CRP

- Anti neutrophil cytoplasmic antibodies (ANCA): pANCA & cANCA

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9
Q

What is pANCA usually a sign of

A

Microscopic polyangiitis and Churg-Strauss syndrome

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10
Q

What is cANCA usually a sign of

A

Wegener’s granulomatosis

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11
Q

What is the management of vasculitis

A
  • Depends on type, refer to rheumatologist
  • Steroids
  • Immunosupressants
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12
Q

What is Henoch-Schonlein purpura

A
  • 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.
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13
Q

What are the four classic features of Henoch-Schonlein purpura

A
  • purpura (100%)
  • joint pain (75%)
  • abdominal pain (50%)
  • renal involvement (50%) IgA nephritis
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14
Q

What is the management of Henoch-Schonlein purpura

A
  • supportive
  • simple analgesia: abdo pain settles in a few days
  • rest
  • proper hydration
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15
Q

What is the prognosis of Henoch-Schonlein purpura

A
  • 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.
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16
Q

What is Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)

A
  • 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
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17
Q

What is Microscopic polyangiitis

A
  • renal failure

- can also affect the lungs causing shortness of breath and haemoptysis.

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18
Q

What is Granulomatosis with polyangiitis (Wegener’s granulomatosis)

A
  • Affect kidneys and lungs
  • Lung symptoms: cough, wheeze and haemoptysis
  • Kidney symptoms: Rapidly progressing glomerulonephritis.
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19
Q

What Upper resp tract findings can you see in Granulomatosis with polyangiitis (Wegener’s granulomatosis)

A
  • nose bleeds (epistaxis)
  • crusty nasal secretions
  • hearing loss
  • sinusitis.
  • saddle shaped nose due to a perforated nasal septum.
20
Q

What CXR findings might you see in Granulomatosis with polyangiitis (Wegener’s granulomatosis)

A
  • consolidation- (misdiagnosed as pneumonia)
21
Q

What is Polyarteritis Nodosa

A
  • 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
22
Q

What can polyarteritis Nodosa be associated with

A
  • Mainly Hepatitis B
  • hepatitis C
  • HIV
  • No clear cause
23
Q

What is Kawasaki Disease

A
  • medium vessel vasculitis. It affects young children, typically under 5 years of age. There is no clear cause.
24
Q

What are the key features of Kawasaki Disease

A
  • 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)
25
Q

What is the treatment of Kawasaki’s Disease

A
  • aspirin

- IV immunoglobulins.

26
Q

What is the key complication of Kawasaki’s Disease

A

coronary artery aneurysms.

27
Q

What is Takayasu’s arteritis

A
  • 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”.
28
Q

How does Takayasu’s arteritis present

A
  • 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
29
Q

How do you diagnose Takayasu’s arteritis

A
  • CT or MRI angiography

- Doppler ultrasound of the carotids can be useful in detecting carotid disease.

30
Q

What is a giant cell arteritis

A
  • systemic vasculitis of the medium and large arteries

- strong link with polymyalgia rheumatica.

31
Q

What is the main presentation of giant cell arteritis involving a headache

A
  • 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
32
Q

What systemic features may you see in giant cell arteritis

A
Fever
Muscle aches
Fatigue
Weight loss
Loss of appetite
Peripheral oedema
33
Q

How is giant cell arteritis diagnosed

A

Clinical presentation
Raised ESR: usually 50 mm/hour or more
Temporal artery biopsy findings

34
Q

What is seen on temporal artery biopsy in giant cell arteritis

A

Multinucleated giant cells

35
Q

What invetigations should you do if suspecting giant cell arteritis

A
  • Temporal artery biopsy
  • FBC: normocytic anaemia and thrombocytosis
  • LFTs: Raised ALP
  • CRP/ESR
  • Duplex ultrasound of the temporal artery shows the hypoechoic halo sign
36
Q

What is the immediate management of giant cell arteritis

A
  • 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)
37
Q

Who should a patient with giant cell arteritis be referred to?

A
  • Rheumtologists
  • Opthalmologists
  • Vascular surgeons
38
Q

What advice must you give patients on high dose steroids/addisons disease

A

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.

39
Q

What are early complications of giant cell arteritis

A
Vision loss
Cerebrovascular accident (stroke)
40
Q

What are the late complications of giant cell arteritis

A

Relapses of the condition are common
Steroid related side effects and complications
Cerebrovascular accident (stroke)
Aortitis leading to aortic aneurysm and aortic dissection

41
Q

What is polymyalgia Rheumatica

A
  • inflammatory condition that causes pain and stiffness in the shoulders, pelvic girdle and neck
  • strong association to giant cell arteritis
42
Q

Who usually suffers from polymyalgia Rheumatica

A

It usually affects old adults (above 50 years)
More common in women
More common in caucasians

43
Q

What symptoms may indicate polymyalgia Rheumatica

A
  • 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
44
Q

What are the differential diagnoses of Polymyalgia Rheumatica

A
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
45
Q

How is polymyalgia rheumatica diagnosed

A
  • clinical presentation + response to steroids

- ESR/CRP may be raised but does not rule out if normal

46
Q

What is the treatment for polymyalgia rheumatica

A
  • 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