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MSK/Rheum Exam- Clin Med > Vasculitis syndromes > Flashcards

Vasculitis syndromes Flashcards

1
Q

Polymyalgia Rheumatica (PMR) general info

A 
Most benign
Common age >50
Avg age 75
Females>>>males
Unknown cause
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2
Q

PMR clinical presentation

A

Abrupt onset
INTENSE MORNING STIFFNESS** that can last all day
Esp seen in shoulders and hip girdle area
NO SMALL JOINT INVOLVEMENT
Normal muscle strength
Fatigue and anorexia
(15% get GCA)

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3
Q

Polymyalgia Rheumatica blood work

A

Elevated CRP and ESR
Anemia in chronic dz
Elevated platelets

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4
Q

PMR treatment

A

Low dose steroids (10-20 mg/day)
prednisone is drug of choice

This should bring CRP and ESR back to normal

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5
Q

Long term tx of Polymalgia Rheumatica

A

Usually self limiting
65% able to taper off by 1 year
>85% in 2 years

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6
Q

Giant cell arteritis General info

A

Can occur by itself or along with PMR
Rare
Age >50
involves medium/large blood vessels of the head and neck including the blood vessels that supply the optic nerve

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7
Q

GCA pathophysiology

A

Unknown trigger causes inflam response with the release of IL1 and IL6
Infiltration of inflam cells into adventitia of temporal arteries
Giant cell histologic pattern

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8
Q

GCA clinical presentation

A 
Scalp tenderness
Temporal headaches 
Jaw claudication
Sudden loss of vision 
Bounding or absent temporal artery pulses
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9
Q

GCA diagnostic studies

A

temporal arty biopsy=gold standard*
Elevated ESR and CRP (usually higher than PMR levels)
Anemia

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10
Q

GCA treatment

A

High dose prednisone*** 60 mg/day
Slow taper over 1-2years
Some pts require chronic low dose (10mg/day)

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11
Q

Possible giant cell arteritis complications

A

Blindness
Scalp necrosis
Lingual infarction
Aortic dissection/aneurysm

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12
Q

Complications from high dose steroid use

A

Osteoporosis
Cataracts
Elevated blood sugars
Weight gain

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13
Q

Granulomatosis with polyangiitis (GWP) general info

A

Potentially fatal vasculitis involving SMALL VESSELS
Pathology shoe necrotizing granulomas usually in upper airway, lungs and kidneys

Rare

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14
Q

Vasculitis

A

Inflammation of blood vessel walls leading to altered blood flow thru obstructed walls
*ischemia and tissue damage
*intense inflammatory rxn
CAN BE FATAL

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15
Q

GWP pathophysiology

A

C-ANCAs play a central role
These auto antibodies interact with primed neutrophils to cause vascular injury and necrosis

Histologic lesions show granulomas

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16
Q

GWP clinical presentation in eyes

A

Epsicleritis/scleritis

Proptosis due to retro orbital mass

17
Q

GWP presentation in CNS

A

Rare mass lesion

18
Q

GWP presentation in upper airway

A 
Otitis media 
Nasal chondritis
Sinusitis w purulent discharge and epistaxis 
Ulcerations 
Subglottic stenosis
19
Q

GWP kidney presentation

A

Neprotic syndrome
Proteinuria
Renal failure

20
Q

GWP skin presentation

A

Palpable purpura due to leukocytoclastic vasculitis
Pyoderma gangrenosum
Panniculitis

21
Q

GWP lung presentation

A

Cough
Hemoptysis
Hemorrhage
Resp failure

22
Q

GWP cardiac presentation

A

Pericarditis

Conduction abnormalities

23
Q

GWP systemic presentation

A

Fever
Night sweats
Wt loss
Fatigue

24
Q

GWP and cANCA

A

Presence of c-ANCA
(Positive 80-90% of time in generalized GWP)

*c-ANCA often negative when GWP limited to airways

25
Q

GWP lab results

A 
Elevated ESR and CRP
Anemia
Leukocytosis 
Thrombocytosis
Elevated Cr
Hematuria
Proteinuria
26
Q

GWP treatment

A

High dose steroids and Cyclophosphamide**

Methotrexate or Azathioprine sometimes used as steroid sparing agents

27
Q

Polyarteritis nodosa (PAN) general info

A

Medium vessel vasculitis
Can be caused by Hep B
Peak incidence 50s and 60s
Slightly more common in males

28
Q

Polysrteritis nodosa (PAN) pathophysiology

A

In Hep B assoc cases…immune complexes play role

Not fully understood in non Hep B cases

29
Q

PAN clinical presentation

A

Fever, fatigue, wt loss
Abdominal pain due to mesenteric angina/ischemia
Mononeuritis multiplex
Myalgias/arthalgias/mild arthritis
Skin-livedo reticularis, palpable purpura, fingertip ulcerations
subcutaneous nodules
Testicular tenderness

30
Q

Complications of PAN

A

Chronic renal failure
Bowel perforation
Stroke/cerebral hemorrhage due to HTN
Foot/wrist drop

31
Q

Labs of PAN

A 
Elevation of ESR and CRP
Absence of ANCA 
Elevated transaminases
Decreased albumin 
Proteinuria
Hematuria (no casts)
32
Q

Imaging studies of PAN

A

Mesenteric and or renal angiography*****

Biopsies seldom done

33
Q

PAN treatment

A

High dose steroids cyclophosphamide
Methotrexate or Azathoprine used as steroid sparing agents once dz is under control

(Tx hep B w anti virals)

MSK/Rheum Exam- Clin Med (7 decks)

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