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MSK > cortex 4 - Rheumatology > Flashcards

cortex 4 - Rheumatology Flashcards

1
Q

what is the common symptoms of polymylagia rheumatica ?

A

It is characterized by proximal myalgia(muscle pain) of the hip and shoulder girdles with accompanying morning stiffness that lasts for more than 1 hour. Symptoms tend to improve as the day goes on and with movement.

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2
Q

what disease is PMR possibly assoicated with ?

A

Giant cell arteritis (GCA)

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3
Q

what is polymyalgia rheumatica ? (PMR)

A

chronic inflam condition that affects elderly individuals (very rare under 50 years)

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4
Q

is there a specific diagnostic test for PMR ?

A

no but almost always CRP and PV/ESR are raised

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5
Q

what is the treatment of PMR ?

A

prednisolone 15mg daily which is gradually reduced over roughly 18 months

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6
Q

what is giant cell arteritis ?

A

most common form of systemic vasculitis in adults

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7
Q

what is the histopathology of GCA ?

A

Marked by transmural inflammation of the intima, media, and adventitia of affected arteries, as well as patchy infiltration by lymphocytes, macrophages, and multinucleated giant cells.

vessel wall thickening can occur which results in narrowing hence distal ischemia

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8
Q

what are the common signs and symptoms of GCA ?

A

visual disturbances

headache

jaw claudication

scalp tenderness

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9
Q

what should always be considered in the differential diagnosis of a new-onset headache in patients 50 years of age or older with an elevated erythrocyte sedimentation rate (ESR), CRP or plasma viscosity?

A

GCA

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10
Q

what is this clinical feature and what disease is it associated with ?

A

thickenend temporal artery which is tender to touch - associated with GCA

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11
Q

how is GCA diagnosed?

A

The most definitive test is temporal artery biopsy. Although a positive temporal artery biopsy has a 100% specificity it has a relatively low sensitivity. This is partly due to the patchy involvement of GCA on the artery.

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12
Q

what is the typical biopsy findings when a temporal biopsy is carried out for GCA?

A

Mononuclear infiltration or granulomatous inflammation, usually with multinucleated giant cells.

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13
Q

what is the treatment of GCA?

A

corticosteroids usually prednisolone 40mg (if no visual symptoms), 60mg if visual symptoms.

Treatment given as soon as diagnosis is suspected (i.e. even before biopsy) then dose decreased over 2 years.

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14
Q

what is polymyositis ?

A

An idiopathic inflammatory myopathy that causes symmetrical, proximal muscle weakness

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15
Q

what is dermatomyositis ?

A

clinically similar to polymyositis but also has typical cutaneous manifestations.

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16
Q

who are both polymyositis and dermatomyositis more common in and what age ranges do they typically affect ?

A

more common in females and usually affects people over 20 - most common in 45-60 year olds.

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17
Q

what is the pathogenisis of polymyositis?

A

A T-cell–mediated cytotoxic process directed against unidentified muscle antigens. CD8 T cells, along with macrophages, initially surround healthy nonnecrotic muscle fibers and eventually invade and destroy them.

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18
Q

what is the typical presentation of polymyositis ?

A

Usually present with symmetrical, proximal muscle weakness in the upper and lower extremities. commonly noticed e.g. when climbing the stairs.

Dysphagia can occur secondary to oropharyngeal and esophageal involvement.

Interstitial lung disease can occur (esp those positive for anti-Jo-1 antibody)

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19
Q

what are the investigations carried out to diagnose polymyositis?

A

inflam markers often raised.

Serum creatine kinase (CK) level is usually raised by x10

Autoantibodies include ANA, anti-Jo-1 and anti-SRP.

Can do MRI, Electromyographic (EMG), and muscle biopsy which is crucial to help the diagnosis

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20
Q

what are the typical features seen on muscle biopsy in a patient with polymyositis ?

A

Muscle biopsy showing chronic inflammatory infiltrate consisting of T lymphocytes, especially CD8+ T lymphocytes.

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21
Q

what is the treatment of polymyositis ?

A

Prednisolone (initially around 40mg) combined with immunosuppressive drugs such as methotrexate or azathioprine

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22
Q

what are some of the typical features of dermatomyositis ?

A

Clinically similar to polymyositis other than the addition of typical cutaneous features. These include:

  • V-shaped rash over chest
  • Gottron’s papules
  • Heliotrope rash (violaceous dis- coloration of the eyelids)
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23
Q

what is this feature and what disease is it a feature of ?

A

heliotrope rash seen in dermatomyositis

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24
Q

what is this clinical feature and what condition is it seen in ?

A

v shaped rash seen in dermatomyositis

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25
Q

what is this clinical feature and what condition is it seen in ?

A

gottons papules seen in dermatomyositis

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26
Q

what is the investigation and management of dermatomyositis ?

A

it is the same as for polymyositis

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27
Q

what does both polymyositis and dermatomyositis increase the risk for ?

A

malignancy - they should be screened for this at the time of diagnosis

common malignancies that occur are - breast, ovarian, lung, colon, oesophagus and bladder.

28
Q

what is fibromyalgia ?

A

it is an unexplained condition causing widespread muscle pain and fatigue

29
Q

who is fibromyalgia most common in ?

A

young and middle aged women but can affect anyone.

30
Q

what are 2 conditions fibromyalgia is commonly secondary to ?

A

RA and SLE

31
Q

what are the signs and symptoms of fibromyalgia ?

A

Persistent (≥ 3 months) widespread pain (pain/tenderness on both sides of the body, above and below the waist, and includes the axial spine [

Fatigue; disrupted and unrefreshing sleep

Cognitive difficulties

Multiple other unexplained symptoms, anxiety and/or depression, and functional impairment of activities of daily living (ADLs)

32
Q

what are some of the associated conditions with fibromyalgia ?

A

depression

IBS

Migraine

33
Q

what are the investigations to diagnose fibromylagia ?

A

diagnosis made on a clinical basis

34
Q

what is the treatment of fibromyalgia ?

A

mainstay of treatment is for the patient to learn self-management techniques.

Atypical analgesia including tricyclics (e.g. amitriptyline), gabapentin and pregabalin may be of benefit.

Psychological input with techniques such a cognitive behavioural therapy may help some patients.

35
Q

what is the definition of vasculitis?

A

inflam of blood vessels

36
Q

what may vasculitis result in ?

A

Vessel wall thickening, stenosis, and occlusion with subsequent ischemia.

37
Q

look at this pic for classifying the different types of vasculitis

A
38
Q

what is large vessel vasculitis in general terms ?

A

Primary vasculitis that causes chronic granulomatous inflammation predominantly of the aorta and its major branches.

39
Q

what are the 2 main types of large vessel vasculitis ?

A

GCA and Takayasu arteritis (TA)

40
Q

what is the difference in the age at which TA and GCA onset ?

A

TA rarley occurs after 50

GCA rarley occurs before 50

41
Q

what could happen if TA or GCA is untreated?

A

vascular stenosis and aneursyms can occur

42
Q

what are some of the features of large vessel vasculitis ?

A

low-grade fever, malaise, night sweats, weight loss, arthralgia and fatigue.

Following this patients can experience claudicant symptoms in both the upper and lower limbs.

43
Q

what are some of the investigations used in large vessel vasculitis ?

A

ESR, PV and CRP and elevated.

Imaging such as MR angiography can detect thickened vessel walls and stenosis

44
Q

what is the treatment of large vessel vasculitis ?

A

Corticosteroids, starting at 40-60mg prednisolone and gradually reducing. Steroid sparing agents such as methotrexate and azathioprine may be added.

45
Q

look at the flow chart for classifying small to medium vessel vasculitis

A
46
Q

what can small to medium vessel vasculitis be divided up into?

A

ANCA positive and negative

47
Q

what are some of the features of small to medium vessel vasculitis ?

A
  • Fever and weight loss
  • A raised, non blanching purpuric rash
  • Arthralgia/arthritis
  • Mononeuritis multiplex
  • Glomerulonephritis
  • Lung opacities on x-ray
48
Q

what is this clinical feature and what broad range of conditions is it seen in ?

A

small to medium vessel vasculitis

49
Q

what are the 4 types of vasculitis associated with ANCA?

A

GPA, microscopic polyangiitis, renal limited vasculitis and churg-strauss syndrome

50
Q

what is granulomatosis with polyangiitis (GPA)?

A

it is granulomatous inflam involving the resp tract, and necrotising vasculitis affecting small to medium vessels. necrotising glomerulonephritis is common

51
Q

what are the common features of GPA?

A

ENT symptoms - nose bleeds, deafness, recurrent sinusitis and nasal crusting (over time there can be collapse of the nose)

Resp - haemoptysis and cavitating lesions on x-ray

52
Q

look at this table for small to medium vessel ANCA involved - pathogenesis

A
53
Q

what is this clinical sign and what condition is it seen in ?

A

nasal cartilage collapse seen in GPA

54
Q

what is this clinical sign and what conditionis it seen in ?

A

cavitating opacities in CXR - seen in GPA

55
Q

what antibodies is GPA associated with ?

A

cANCA and PR3 (proteinase-3)

56
Q

what is EGPA characterised by ?

A

Late onset asthma, rhinitis and a raised peripheral blood eosinophil coun

57
Q

what is a very important complication to look out for in microscopic polyangiitis ?

A

glomerulonephritis occurs in 90% of patients.

58
Q

why cant ANCA be used to diagnose ANCA associated vasculitis ?

A

it is negative in a proportion of all these conditions

59
Q

what are some of the investigations used to diagnose ANCA associated vasculitis ?

A
  • ESR, PV and CRP and raised
  • Anaemia of chronic disease is common.
  • U+E looking for renal involvement
  • Anti-neutrophil cytoplasmic antibody (ANCA)
  • Urinalysis (looking for renal vasculitis)
  • CXR
  • Biopsy of an affected area e.g. skin or kidney is often helpful
60
Q

what is the treatment of ANCA associated vasculitis ?

A

IV steroids and cyclophosphamide

61
Q

what is Henoch-Schonlein purpura?

A

an acute immunoglobulin A (IgA)–mediated disorder characterized by a generalised vasculitis involving the small vessels and many different organs/systems

62
Q

who does Henoch-Schonlein purpura usually affect?

A

children

63
Q

what is Henoch-Schonlein purpura usually predated by ?

A

an upper resp tract infection

64
Q

what are the common symptoms of Henoch-Schonlein purpura?

A

purpuric rash over the buttocks and lower limbs, abdominal pain and vomiting and joint pain.

65
Q

what is the treatment of Henoch-Schonlein purpura

A

it is self limiting

66
Q

what antibodies is EPGA associated with ?

A

pANCA and MPO-ANCA

MSK (22 decks)

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