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Musculoskeletal > Rheumatology: Muscle Disease and Vasculitis > Flashcards

Rheumatology: Muscle Disease and Vasculitis Flashcards

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1
Q

What clinical features does muscle disease typically present as?

A

Pain, myalgia, fatigue, weakness, stiffness, abnormal bloods (sometimes)

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2
Q

What are Polymyositis and Dermatomyositis?

A

Idiopathic inflammatory myopathies

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3
Q

What is the peak incidence of age for Polymyositis and Dermatomyositis?

A

40-50years

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4
Q

What is the relationship between Polymyositis and Dermatomyositis and malignancy?

A

Are at an increased risk of developing malignancy

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5
Q

What are the main clinical features of Polymyositis and Dermatomyositis?

A

Symmetrical muscle weakness affecting proximal muscles and causing problems with every day activities such as climbing stairs

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6
Q

What are some of the clinical signs specific to Dermatomyositis

A

Shawl sign, Gorton’s sign, Heliotrope rash

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7
Q

Give a description of what the following Dermatomyositis features look like: “Shawl sign, Gottron’s sign, Heliotrope rash”

A

Shawl sign: V shaped purple rash over chest, back or shoulders, worsens with UV exposure

Gottron’s sign: discrete erythematous plaques overlying the metacarpal and inter pharyngeal joints

Helitrope rash: purple rash around upper eyelids (and swelling)

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8
Q

What is the most common other organ involvement in Polymyositis and Dermatomyositis?

A

Lungs- interstitial lung disease or weakness or respiratory muscles

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9
Q

Who is at the greatest risk of malignancy in Polymyositis and Dermatomyositis?

A

Men aged >45

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10
Q

What must be screened for in a patient with Polymyositis and Dermatomyositis?

A

Malignancy

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11
Q

Why is it important to take a drug history in particular in muscle disease?

A

Statins can cause muscle pain and inflammation

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12
Q

What is the definitive test in Polymyositis and Dermatomyositis?

A

Muscle biopsy

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13
Q

What is seen on muscle biopsy in Polymyositis and Dermatomyositis?

A

Perivascular inflammation and necrosis

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14
Q

What autoantibody is specific to Polymyositis and Dermatomyositis (raised)?

A

Anti-Jo

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15
Q

What should you look for in blood tests for diagnosis of Polymyositis and Dermatomyositis?

A

Anti-Jo postive and high CK level

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16
Q

Why is an MRI useful in Polymyositis and Dermatomyositis?

A
  • Looks at calcification

- Can help pin point the areas where muscle needs to be biopsied

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17
Q

What is the treatment for Polymyositis and Dermatomyositis?

A 
Give steriod (prednisolone) and an immunosuppressant (aziothioprine)
-Steroid in short term to control inflammation and then immunosuppressive on its own in long term
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18
Q

How does Inclusion Body Myositis tend to present and in who?

A

Older patients >50, more common in male, and as distal muscle weakness

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19
Q

What does a muscle biopsy show in Inclusion Body Myositis and what does it mean in terms of prognosis?

A

Shows inclusion bodies indicating poorer prognosis

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20
Q

Who does Polymyalgia Rheumatica tend to affect and what is the prevalence?

A

Patients >50 years, 1% of population

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21
Q

What is Polymyalgia Rheumatica associated with?

A

Temporal Arteritis/Giant cell arteritis

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22
Q

What is the clinical manifestation of Polymyalgia Rheumatica?

A

Symmetrical proximal muscle group stiffness, typically first thing in morning and improves throughout the day
-NO muscle weakness, muscle strength is normal

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23
Q

What other clinical manifestations can be reported in Polymyalgia Rheumatica when temporal arthritis is involved?

A

Unilateral, localised headaches, loss of vision, jaw claudication, tender temple and scalp

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24
Q

What would be seen on biopsy of the temporal artery?

A

Inflammation of the lining of the temporal artery

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25
How is Polymyalgia Rheumatica diagnosed?
Raised inflammatory markers, no specific tests | Temporal artery biopsy
26
Are there specific diagnostic tests for Polymyalgia Rheumatica?
No, only raised inflammatory markers
27
What is the treatment for Polymyalgia Rheumatica?
Give small dose of steroid: 15mg of prednisolone and there should be a dramatic and instant improvement
28
When should a larger dose of steroid be given in Polymyalgia Rheumatica?
If there is Giant cell arthritis present, needed to prevent visual loss
29
What are the most common clinical manifestations of fibromyalgia?
- Fatigue due to disturbed sleep, creating a cycle of broken unrefreshing sleep and depression - pain and fatigue with minimal exertion
30
What are some of the clinical manifestations of fibromyalgia?
- pain and fatigue with minimal exertion - sensation of swelling when there is none - pins and needles
31
What are the clinical finding on examination of a patient with fibromyalgia?
excessive tenderness on palpation of the soft tissue
32
How is fibromyalgia diagnosed?
- check inflammatory markers and should be normal | - no diagnostic tests, by pressure points
33
What is the difference between primary and secondary vasculitis?
Primary: results from an inflammatory response that targets the vessel walls and has no known cause Secondary: may be triggered by an infection, drug, toxin or as part of another inflammatory disease
34
What is vasculitis?
Inflammation of blood vessels often with ischaemia, necrosis and organ involvement
35
What does vasculitis affect?
Any blood vessel- arteries, veins, arterioles, venules, capillaries
36
What is the classification of vasculitis?
Small, medium and large vessel
37
What is small vessel divided into?
ANCA positive and ANCA negative
38
What are the two types of large vessel vasculitis?
Takayasu Arteritis and Giant cell Arteritis
39
Who does Takayasu Arteritis present in?
40
Who does Giant cell Arteritis present in?
>50 years old causes temporal arteritis, the aorta and other large vessels
41
What does an MRI angiogram of the aorta and its major branches in large vessel vasculitis show?
Thickened vessel wall and stenosis
42
What are the clinical findings in large vessel vasculitis?
Carotid bruit (80%), hypertension, difference in BP, claudication (both upper and lower limbs)
43
How should large vessel arthritis be managed?
Starting dose of steroids 40-60mg (with/without aziothioprine/methotrexate)
44
What are the two types of medium vessel vasculitis?
Kawasaki and Polyarteritis Nodosa
45
Who does Kawasaki present in?
Children
46
What vessels does Kawasaki usually affect?
Vasculitis of various vessels, most important are coronary arteries where aneurysms can develop
47
What is Polyarteritis Nodosa characterised by?
Necrotising inflammatory lesions that affect arteries at vessel bifurcations, resulting in micro-organism formation and aneurysms
48
What is Polyarteritis Nodosa associated with?
Chronic Hepatitis B
49
What organs does Polyarteritis Nodosa affect?
Skin, gut and kidneys
50
What can small vessel vasculitis be further classified into?
ANCA positive and ANCA negative
51
What is the treatment for most ANCA positive?
IV steriods and cyclophosphamide die to aggressive nature of disease
52
What ANA is associated with Granulomatous with polyangittis?
cANCA and PR3
53
What ANA is associated with Eosinophilic Granulomatous with polyangittis?
pANCA and MPO
54
What is the histology of Granulomatous with polyangittis?
Granulomatous inflammation of rest tract, small and medium vessels. Necrotising glomerulonephritis is common
55
What ENT symptoms are associated with Granulomatous with polyangittis?
Nose bleeds, deafness, recurrent sinusitis, nasal crusting, "saddle nose" due to cartilage ischaemia
56
What respiratory symptoms are associated with Granulomatous with polyangittis?
Diffuse alveolar haemorrhage, cavitating lesions of CXR, haemoptysis
57
What cutaneous symptoms are associated with Granulomatous with polyangittis?
Palpable purpura and cutaneous ulcers
58
What ocular symptoms are associated with Granulomatous with polyangittis?
Uvetitis, conjunctivitis, retinal artery occlusion
59
What is the criteria for diagnosis of Eosinophilic Granulomatous with polyangittis?
4 or more of: - Asthma - Eosinophilia of more than 10% in peripheral blood - Paranasal sinusitis - Pulmonary infiltrates (may be transient) - Histological proof of vasculitis with extravascular eosinophils - Mononecrotitis multiplex or polyneuropathy
60
What is the main difference between Granulomatous with polyangittis and Eosinophilic Granulomatous with polyangittis?
-Late onset of asthma and high eosinophil count
61
What is the histology of Eosinophilic Granulomatous with polyangittis?
Eosinophilic granulomatous inflammation of respiratory tract, small and medium vessels associated with asthma
62
What is an important complication of Glomerulonephritis (occurs in up to 90% of patients)?
Microscopic Polyangiitis
63
What technique is used to detect ANCA and differentiate between patterns?
Immunofluorescence
64
What is Henoch-Schonlein Purpura?
Acute immunoglobulin IgA mediated disorder
65
Where does Henoch-Schonlein Purpura affect in the body?
Vessels of the skin, GI tract, kidneys, joints, rarely lungs and CNS
66
Who does Henoch-Schonlein Purpura affect?
75% in children aged 2-11, rare in infants
67
What commonly precedes Henoch-Schonlein Purpura?
History of an upper respiratory tract infection predates HSP by 1-3 weeks, most commonly group A strep
68
What are common symptoms of Henoch-Schonlein Purpura?
Purpuric rash over buttox and lower limbs, abdominal pain and vomiting and joint pain. Renal involvement (50%)
69
How is Henoch-Schonlein Purpura treated?
Self limiting, symptoms resolve within 8 weeks, relapses may occur. Perform urinalysis to screen for renal involvement

Musculoskeletal (13 decks)

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