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Flashcards in Vasculitis and Fibromyalgia Deck (92):
1

What is Takayasu's arteritis? Where in the body does it usually occur, and whom does it usually affect?

-Large vessel granulomatous vasculitis with massive intimal fibrosis and vascular narrowing.
-Aorta
-Young females (15-30 yo)

2

What are some of the classic s/sx of Takayasu's arteritis?

-Decreased to absent peripheral pulses
-B symptoms during inflammatory phase

3

What are the two major phases of Takayasu's arteritis?

Inflammatory phase
Pulseless phase

4

What are some major sequelae of Takayasu's arteritis?

-Renovascular HTN
-Ocular involvement

5

What is the characteristic type of inflammation of the vessels in Takayasu's arteritis?

Segmental and patchy granulomatous inflammation of the aorta or its branches

6

What is the haplotype that is associated with Takayasu's arteritis?

HLA-B52

7

What are the major criteria for Takayasu's arteritis? (5)

-Age less than 40
-Claudication of extremities
-Decreased brachial artery pulse
-BP difference of more than 10 mmHg between arms
-Bruit over subclavian or aorta

8

What is the gold standard test for Takayasu's arteritis?

Arteriogram showing occlusion or narrowing in the aorta or its main branches

9

Takayasu's arteritis must occur before what age to meet its diagnostic criteria?

40

10

What is the BP difference between arms that is needed in the diagnostic criteria for Takayasu's arteritis?

more than 10 mmHg between arms

11

What is the gold standard for diagnosing vasculitis?

Bx

12

Who does Kawasaki's disease affect?

Asian children less than 4 yo

13

What are the s/sx of Kawasaki's disease? (5)

-Marked Fever
-LAD /peripheral edema
-conjunctival injection
-Strawberry tongue
-Desquamating rash

14

What is the treatment fro Kawasaki's disease?

ASA and IVIG

15

What are the s/sx of Churg-Strauss syndrome?

-Asthma
-Sinusitis
-Palpable purpura
-Peripheral neuropathy

16

What are the labs that are elevated with Churg-Strauss syndrome? (2)

-IgE
-MPO-ANCA (p-ANCA)

17

What are the s/sx of granulomatosis with polyangiitis (Wegener's syndrome)?

-URI
-Perforation of nasal septum
-Chronic sinusitis
-OM
-Hematuria with red cell casts

18

What is the classic triad for granulomatosis with polyangiitis (Wegener's syndrome)?

-Focal necrotizing vasculitis
-Necrotizing granulomas in the lung and upper airway
-Necrotizing glomerulonephritis

19

-Focal necrotizing vasculitis
-Necrotizing granulomas in the lung and upper airway
-Necrotizing glomerulonephritis = ?

granulomatosis with polyangiitis (Wegener's syndrome)

20

What are the abs that are elevated with granulomatosis with polyangiitis (Wegener's syndrome)?

PR3-ANCA (c-ANCA)

21

What is the treatment for granulomatosis with polyangiitis (Wegener's syndrome)?

Cyclophosphamide

22

What are the two major vasculitides that affect large arteries?

-Giant cell (temporal) arteritis
-Takayasu arteritis

23

Small or small-medium vasculitis: HSP

Small

24

Small or small-medium vasculitis: SLE

Medium

25

Small or small-medium vasculitis: cryoglobulinemia

Small

26

Small or small-medium vasculitis: polyarteritis nodosa

Medium

27

Small or small-medium vasculitis: Wegener's

Medium

28

Small or small-medium vasculitis: rheumatoid vasculitis

Medium

29

Small or small-medium vasculitis: Churg-Strauss

Medium

30

Small or small-medium vasculitis: microscopic polyangiitis

Medium

31

Small or small-medium vasculitis: Kawasaki

Medium

32

Small or small-medium vasculitis: vasculitis associated with malignancy

Small

33

Polyarteritis Nodosa is associated with what infectious disease?

Hep B

34

What age group Polyarteritis Nodosa classically affect?

Young adults

35

What are the s/sx of Polyarteritis Nodosa? (5)

-B symptoms
-HTN
-Abdominal pain / melena
-Renal damage
-Neurological disease

36

What vessels does Polyarteritis Nodosa classically involve?

renal and visceral vessels (not pulmonary arteries)

37

What type of hypersensitivity rxn is Polyarteritis Nodosa?

Type III

38

What are the characteristics bx findings of Polyarteritis Nodosa?

Transmural inflammation of the arterial wall with fibrinoid necrosis

39

What are the classic arteriogram findings of Polyarteritis Nodosa?

Innumerable microaneurysms and spasms

40

What is the treatment for Polyarteritis Nodosa? (2)

Corticosteroids
Cyclophosphamide

41

Who does Buerger's disease (thromboangiitis obliterans) classically affect?

heavy male smokers

42

What are the bx findings of Buerger's disease (thromboangiitis obliterans)?

Segmental thrombosing vasculitis

43

What is the treatment for Buerger's disease (thromboangiitis obliterans)?

Smoking cessation

44

What are the s/sx of Buerger's disease (thromboangiitis obliterans)? (2)

-Intermittent claudication leading to gangrene / amputation of digits
-Raynaud's syndrome

45

What are some of the general s/sx that may lead you to suspect a vasculitis?

-Multisystem dz
-Skin lesions
-Renal involvement
-B symptoms

46

What is mononeuritis multiplex?

Death of vasavesorum of nerves lead to nerve deficits

47

What diseases /drugs need to be r/o prior to diagnosing vasculitis?

-Thrombotic disease
-Antiphospholipid syndrome
-Cocaine / amphetamines

48

Which classically involves the lungs: PAN or MPA?

MPA

49

Which is ANCA positive: PAN or MPA?

MPA

50

What are the organs that are commonly involved in MPA?

Lungs
Kidneys
skin (palpable purpura)

51

True or false: there are no granulomas with MPA

True

52

What is the classic angiogram findings of polyarteritis nodosa?

String of pearls

53

What are the four criteria for diagnosing HSP?

-Palpable purpura
-Less than 20 years
-Bowel angina
-Granulocytes in walls of arterioles or venules

54

HSP occur below what age (according to the classification criteria)?

20

55

What is the classic triad of symptoms for HSP?

-Palpable purpura
-Arthralgias
-Abdominal pain/ melena

56

What is the ab that is deposited with HSP?

IgA

57

What happens to the severity of HSP as kids age?

Worse--need to monitor teenagers

58

HSP classically occurs after what?

A viral infection

59

What is cryoglobulinemia? What are the clinical features of it (3)?

-Immunoglobulins that undergo reversible precipitation in the cold
-Acral cyanosis, ulcerations/ necrosis, raynaud's phenomenon

60

What are the clinical features of cryoglobulinemia that are related to immune complex deposition? (4)

-Purpura
-Arthralgias
-Nephritis
-Neuropathy

61

What are the 4 diagnostic criteria for Wegener's granulomatosis (GPA)?

-Nasal or oral inflammation
-Abnormal chest radiograph (nodules / cavities)
-Urinary sediment
-Granulomatous inflammation on bx

62

What are the CXR findings of GPA?

Nodules
cavities

63

What are the UA findings of GPA?

Sediment with more than 5 RBCs / hpf or RBC casts

64

What are the nasal / oral mucosal findings of GPA?

Nasal or oral inflammation, causing a nasal deformity

65

What is polymyalgia rheumatica?

a syndrome with pain or stiffness, usually in the neck, shoulders, upper arms, and hips, but which may occur all over the body. The pain can be very sudden, or can occur gradually over a period. Most people with PMR wake up in the morning with pain in their muscles; however, cases have occurred in which the person has developed the pain during the evenings or has pain and stiffness all day long.

66

What are the symptoms of polymyalgia rheumatica?

-Pain and stiffness in the shoulders and hips, often with B symptoms
-Morning stiffness

67

Does polymyalgia rheumatica cause muscular weakness?

No

68

What is ocular findings is associated with polymyalgia rheumatica?

Temporal arteritis

69

What are the ESR, CRP, and CK findings of polymyalgia rheumatica?

Elevated ESR and CRP
Normal CK

70

Who is classically affected with polymyalgia rheumatica?

Women over 50 years, usually of northern european descent

71

What is the treatment /prognosis for polymyalgia rheumatica?

Rapid response to low doses of corticosteroids

72

What are the classic s/sx of giant cell arteritis?

-Jaw Claudication
-HA
-Temporal artery abnormality
-Visual loss

73

What are the three labs that are classically elevated with giant cell arteritis?

-CRP
-ESR
-Platelets

74

What is the treatment for giant cell arteritis?

Steroids

75

What is the severe cardiac sequelae of giant cell arteritis?

Aortic dissection

76

What are the histological findings of giant cell arteritis?

Focal granulomatous inflammation of the vessels with giant cells

77

What is the most commonly affected vessel with giant cell arteritis?

Branches of Carotids

78

What, generally, is fibromyalgia?

Chronic, widespread musculoskeletal pain associated with stiffness, paresthesias, and poor sleep

79

What is the treatment for fibromyalgia? (3)

-Exercise
-SNRIs
-anticonvulsants

80

Having a first degree relative with fibromyalgia imparts a (__)x greater risk of having it.

8.5x

81

What is the proposed pathogenesis of Fibromyalgia?

Deficits in GABAergic and glutamatergic mechanisms

82

What are the associated diseases with FM?

-IBS
-Interstitial cystitis

83

What are some of the common complaints of FM patients?

-Multiple allergies
-Palpitations
-Dysmenorrhea
-Orthostatic intolerance

84

When should SLE be screened for with fibromyalgia?

Only if history and physical suggests inflammatory systemic rheumatic disease

85

What is the ROS like with fibromyalgia?

Positive everywhere

86

What are the endocrine disorders that may have similar s/sx of FM?

-Hypothyroidism
-Hyperparathyroidism
-Cushing's
-Vit D deficiency

87

Do patients feel better immediately after stopping a statin with statin-induced myopathy?

No--2-3 months

88

What should be done with a new diagnosis of FM?

Just like somatic disorder:
-Reassure no infx or other disease
-Stress and mood management

89

What are the two treatments for restless leg syndrome? MOA?

-Ropinirole -dopamine agonist
-Pramipexole-dopamine agonist

90

What is the MOA of bupropion?

Dopamine reuptake inhibitor

91

What are the three medications that should NOT be used with fibromyalgia?

-Narcotics
-NSAIDs
-Steroids

92

What is the MOA of duloxetine?

SNRI