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Musculoskeletal > Rheumatology: Muscle Disease and Vasculitis > Flashcards

Rheumatology: Muscle Disease and Vasculitis Flashcards

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
Q

How is Polymyalgia Rheumatica diagnosed?

A

Raised inflammatory markers, no specific tests

Temporal artery biopsy

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26
Q

Are there specific diagnostic tests for Polymyalgia Rheumatica?

A

No, only raised inflammatory markers

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27
Q

What is the treatment for Polymyalgia Rheumatica?

A

Give small dose of steroid: 15mg of prednisolone and there should be a dramatic and instant improvement

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28
Q

When should a larger dose of steroid be given in Polymyalgia Rheumatica?

A

If there is Giant cell arthritis present, needed to prevent visual loss

29
Q

What are the most common clinical manifestations of fibromyalgia?

A
  • Fatigue due to disturbed sleep, creating a cycle of broken unrefreshing sleep and depression
  • pain and fatigue with minimal exertion
30
Q

What are some of the clinical manifestations of fibromyalgia?

A
  • pain and fatigue with minimal exertion
  • sensation of swelling when there is none
  • pins and needles
31
Q

What are the clinical finding on examination of a patient with fibromyalgia?

A

excessive tenderness on palpation of the soft tissue

32
Q

How is fibromyalgia diagnosed?

A
  • check inflammatory markers and should be normal

- no diagnostic tests, by pressure points

33
Q

What is the difference between primary and secondary vasculitis?

A

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
Q

What is vasculitis?

A

Inflammation of blood vessels often with ischaemia, necrosis and organ involvement

35
Q

What does vasculitis affect?

A

Any blood vessel- arteries, veins, arterioles, venules, capillaries

36
Q

What is the classification of vasculitis?

A

Small, medium and large vessel

37
Q

What is small vessel divided into?

A

ANCA positive and ANCA negative

38
Q

What are the two types of large vessel vasculitis?

A

Takayasu Arteritis and Giant cell Arteritis

39
Q

Who does Takayasu Arteritis present in?

A
40
Q

Who does Giant cell Arteritis present in?

A

> 50 years old causes temporal arteritis, the aorta and other large vessels

41
Q

What does an MRI angiogram of the aorta and its major branches in large vessel vasculitis show?

A

Thickened vessel wall and stenosis

42
Q

What are the clinical findings in large vessel vasculitis?

A

Carotid bruit (80%), hypertension, difference in BP, claudication (both upper and lower limbs)

43
Q

How should large vessel arthritis be managed?

A

Starting dose of steroids 40-60mg (with/without aziothioprine/methotrexate)

44
Q

What are the two types of medium vessel vasculitis?

A

Kawasaki and Polyarteritis Nodosa

45
Q

Who does Kawasaki present in?

A

Children

46
Q

What vessels does Kawasaki usually affect?

A

Vasculitis of various vessels, most important are coronary arteries where aneurysms can develop

47
Q

What is Polyarteritis Nodosa characterised by?

A

Necrotising inflammatory lesions that affect arteries at vessel bifurcations, resulting in micro-organism formation and aneurysms

48
Q

What is Polyarteritis Nodosa associated with?

A

Chronic Hepatitis B

49
Q

What organs does Polyarteritis Nodosa affect?

A

Skin, gut and kidneys

50
Q

What can small vessel vasculitis be further classified into?

A

ANCA positive and ANCA negative

51
Q

What is the treatment for most ANCA positive?

A

IV steriods and cyclophosphamide die to aggressive nature of disease

52
Q

What ANA is associated with Granulomatous with polyangittis?

A

cANCA and PR3

53
Q

What ANA is associated with Eosinophilic Granulomatous with polyangittis?

A

pANCA and MPO

54
Q

What is the histology of Granulomatous with polyangittis?

A

Granulomatous inflammation of rest tract, small and medium vessels. Necrotising glomerulonephritis is common

55
Q

What ENT symptoms are associated with Granulomatous with polyangittis?

A

Nose bleeds, deafness, recurrent sinusitis, nasal crusting, “saddle nose” due to cartilage ischaemia

56
Q

What respiratory symptoms are associated with Granulomatous with polyangittis?

A

Diffuse alveolar haemorrhage, cavitating lesions of CXR, haemoptysis

57
Q

What cutaneous symptoms are associated with Granulomatous with polyangittis?

A

Palpable purpura and cutaneous ulcers

58
Q

What ocular symptoms are associated with Granulomatous with polyangittis?

A

Uvetitis, conjunctivitis, retinal artery occlusion

59
Q

What is the criteria for diagnosis of Eosinophilic Granulomatous with polyangittis?

A

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
Q

What is the main difference between Granulomatous with polyangittis and Eosinophilic Granulomatous with polyangittis?

A

-Late onset of asthma and high eosinophil count

61
Q

What is the histology of Eosinophilic Granulomatous with polyangittis?

A

Eosinophilic granulomatous inflammation of respiratory tract, small and medium vessels associated with asthma

62
Q

What is an important complication of Glomerulonephritis (occurs in up to 90% of patients)?

A

Microscopic Polyangiitis

63
Q

What technique is used to detect ANCA and differentiate between patterns?

A

Immunofluorescence

64
Q

What is Henoch-Schonlein Purpura?

A

Acute immunoglobulin IgA mediated disorder

65
Q

Where does Henoch-Schonlein Purpura affect in the body?

A

Vessels of the skin, GI tract, kidneys, joints, rarely lungs and CNS

66
Q

Who does Henoch-Schonlein Purpura affect?

A

75% in children aged 2-11, rare in infants

67
Q

What commonly precedes Henoch-Schonlein Purpura?

A

History of an upper respiratory tract infection predates HSP by 1-3 weeks, most commonly group A strep

68
Q

What are common symptoms of Henoch-Schonlein Purpura?

A

Purpuric rash over buttox and lower limbs, abdominal pain and vomiting and joint pain. Renal involvement (50%)

69
Q

How is Henoch-Schonlein Purpura treated?

A

Self limiting, symptoms resolve within 8 weeks, relapses may occur. Perform urinalysis to screen for renal involvement

Musculoskeletal (13 decks)

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