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Rheumatology > Vasculitis > Flashcards

Vasculitis Flashcards

Half of this is also in nephro-glomerulonephritis includes GCA, IGA, and other vasculitis

1
Q

Classic triad of IgA vasculitis

A

purpura (rash on abdomen, legs), abdominal pain, and arthalgia

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2
Q

Definitive: Diagnosis of IgA vasculitis

A

Biopsy of skin with immunofluorescence

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3
Q

Henoch Schonlein purpura AKA

A

IgA vasculitis

happens after URI or respiratory infections

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4
Q

IgA vasculitis is a (small/medium/large) vessel dx?

A

small vessel - affects skin, bowel (leading to pain, bleeding, intussusception, less commonly affects kidneys and rarely lungs)

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5
Q

General diagnosis of IgA vasculitis

A

clinical picture of adults and kids. Can get biopsy

Don’t get IgA levels. not sensitive or specific.

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6
Q

long term prognosis of Kawasaki Dx

A

persistent coronary artery aneurysms coveys greatest risk for CAD in pts who had Kawasaki dx as a kid

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7
Q

Kawasaki Dx presentation

A

fever, rash, cervical LAD, prominent nonexudative conjunctivitis, oral mucosal and lip changes.

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8
Q

How to reduce Kawasaki coronary artery aneurysm?

A

IVIG but 10-20% don’t respond.

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9
Q

Surveillance of Kawasaki dx and coronary artery aneurysm

A

may need regular EKG, ECHO, and/or coronary artery imaginge

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10
Q

most common idiopathic glomerulonephritis in developed countries?

A

IgA nephropathy

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11
Q

What affects adverse prognosis of IgA nephropathy?

A

elevated BP and lower than normal GFR.

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12
Q

Who gets biopsies with suspected IgA nephropathy?

A

if protein excretion is >500 mg/day, elevated serum creatinine >1.5 and HTN.

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13
Q

what establishes glomerular dx as being the cause of patient’s hematuria?

A

significant proteinuria, and dysmorphic red blood cells on urinalysis.

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14
Q

anti basement membrane antibody is seen with

A

good pastures syndrome. Can see hematuria recurrently and see significant pulmonary hemorrhage

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15
Q

Giant cell arteritis presentation

A

constitutional symptoms, headache, transient vision loss ESR.

Monocular vision loss and fundoscopic examination typically normal or signs of ischemia

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16
Q

essential mixed (type 2) cryoglobulinemia

A

lymphoproliferative disorder with immune complex deposition in small to medium sized blood vessels.

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17
Q

presentation of cryoglobulinemia

A

palpable purpura, arthralgias, membranoproliferative glomerulonephritis, low serum complement, cutaneous vasculitis and neuropathy.

Purpura is seen in legs
vasculitis may show skin ulceration and necrosis

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18
Q

diagnosis of cryoglobulinemia

A

serum cryoglobulins
also check for hep C (>90% of cryoglobulinemia also are positive for hep C)

see low complement levels and falsely positive RF and ANA

usually will have history of IVDA

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19
Q

churg strauss syndrome has 3 stages

A

prodromal - asthma allergic rhinitis, or atopic dermatitis

eosinophilic phase - peripheral eosinophilia, lung eosinophilia with infiltrates, and a

vasculitic phase (polyangiitis) up to 10 years later.

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20
Q

how does hepatitis C cause false neg RF and ANA for cyroglobulinemia?

A

hep c causes non specific stimulation of lymphocytes resulting in excess production of immunoglobulin - can cause false positives for ANA and RF.

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21
Q

criteria for diagnosis of GCA

A

age >50 yrs
new onset localized headache with fever and visual disturbances
ESR>50
tenderness or decreased pulse of temporal artery
temporal artery biopsy showing necrotizing arteritis with mainly mononuclear cells.

3 out of 5 above criteria is 94% sensitive and 91% specific for GCA

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22
Q

Gold standard for diagnosis of GCA

A

temporal artery biopsy.
negative biopsy results are common because this is patchy disease

Thus we need second biopsy or can treat clinically regardless

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23
Q

management of GCA after achieving initial remission

A

keep patient on high dose steroids and slowly taperover a few months. Some pts may need 1-2 yrs of steroids.

Premature discontinuation may have high risk of relapse with GCA

Add aspirin to prevent long term complications of GCA (TIA and stroke)

Needs surveillance for aortic dissection long term with CXR for 10 years.

DEXA scan for osteoporosis

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24
Q

long term complications of GCA and how to prevent them?

A

TIA and stroke

add aspirin

25
Q

Henoch Schonlein purpura is tetrad of

A

IgA vasculitis

abdominal pain, arthritis, and glomerulonephritis and palpable purpura on lower extremities

26
Q

skin involvement (96%) of pts with HSP (IgA vasculitis)

A

ecchymoses, petechiae and palpable purpura

27
Q

MSK involvement (60% of pts) in HSP

A

arthritis, arthralgia, involving 1-4 large joints (hips knees and ankles)

28
Q

GI involvement in HSP (50%)

A

nausea, vomiting, abdominal pain and GI hemorrhage

29
Q

renal involvement (33%) in HSP

A

hematuria, proteinuria, and renal insufficiency

30
Q

Biopsy of palpable purpura of HSP will show

A

IgA deposition and HSP is immune mediated vasculitis

31
Q

Rash associated with HSP (IgA vasculitis)

A

leukocytoclastic vasculitis with lots of IgA immune complexes within affected organs.

32
Q

prodromal phase of Churg Strauss (seen in 20-30yrs)

A

atopic dx with: asthma allergic rhinitis and recurrent sinusitis

33
Q

eosinophilic phase or Churg Strauss syndrome

A

eosinophilic infiltration of multiple organs

migratory pulmonary opacities
>10% peripheral eosinophilia

eosinophilia gastroenteritis

34
Q

Vasculitis phase seen in 3 or 4th decade of life of Churg Strauss

A

life threatening systemic vasculitis- see tender subcutaneous nodules on extensor surfaces of arms (Granulomas)
heart failure and or conduction defects

peripheral neuropathy (mononeuritis multiplex)

necrotizing glomerulonephritis (usually ANCA positive)

migratory polyarthritis

35
Q

what is allergic granulomatosis and angiitis

A

Churg Strauss syndrome

36
Q

in the Allergic granulomatosis angiitis vasculitis phase what blood vessels are affected?

A

medium and small vessels (kidney)

37
Q

presentation of someone with difficult to control asthma and >10% peripheral eosinophilia and mono or polyneuropathy and transient pulmonary opacities and sinus disease

A

presentation of allergic granulomatosis angiitis or Churg Strauss syndrome

38
Q

what do you see if biopsy a skin rash of Churg Strauss dx?

A

see eosinophilic vasculitis

39
Q

diagnosis of allergic granulomatosis angiitis vasculitis

A

suspected in 4 or more of the following:
asthma and >10% peripheral eosinophilia and mono or polyneuropathy and transient pulmonary opacities and sinus disease and biopsy showing eosinophilic vasculitis

40
Q

treatment of granulomatosis angiitis vasculitis and Churg Strauss

A

IV steroids and sometimes cyclophosphamide

41
Q

what is aspirin exacerbated respiratory disease

A

seen with asthma and chronic rhinosinusitis and nasal polyposis and eosinophilia but this will not cause a pulmonary infiltrates or renal involvement.

42
Q

pt has fever, fatigue, weight loss, new unilateral headache, jaw claudication and visual symptoms and non productive cough

A

think giant cell arteritis

43
Q

aortic aneurysm and dissection seen with polymyalgia rheumatica in 50% of these pts

A

Giant cell arteritis

44
Q

Lab findings of giant cell arteritis

A

elevated ESR and CRP and normocytic anemia

will see temporal artery biopsy that confirms diagnosis. Remember biopsy can be negative due to patchy dx so repeat biopsy on other side and don’t delay treatment with steroids

45
Q

long term treatment of pts with giant cell arteritis

A

perform yearly CXR (up to 10 years) to identify pts with thoracic aortic aneurysms prior to rupture or dissection

if on steroids, needs a DEXA scan for baseline

46
Q

systemic symptoms of giant cell arteritis?

A

fever, fatigue, malaise, weight loss

47
Q

localized symptoms of giant cell arteritis?

A

headaches, located in temporal areas
jaw claudications: most specific symtpoms of GCA
PMR

Arm claudication associated with bruits in subclavian and axillary areas

aortic wall thickening and anuerysms

CNS: TIA/stroke, vertigo, hearing loss

48
Q

pt presents with arm claudication associated with bruits in subclavian and axillary areas

aortic wall thickening and anuerysms

A

need to screen for Giant cell arteritis with ESR and ask about TIAs vision changes and jaw claudication

49
Q

visual symptoms of GCA

A

amaurosis fugax, transient visual field defect progressing to monocular blindness
See anterior ischemic optic neuropathy (AION) is most common ocular manifestation

50
Q

Lab findings of GCA?

A

normocytic anemia,
elevated ESR and CRP
need to get a temporal artery biopsy.

if negative biopsy still treat empirically and repeat biopsy on other side or get ultrasound.

51
Q

Treatment of GCA with PMR only?

A

PMR only: low dose oral glucocorticoids (prednisone 10-20 mg daily for maybe a year)

52
Q

GCA treatment is

A

intermediate to high dose oral glucocorticoids (prednisone 40-60 mg daily)

53
Q

GCA treatment with vision loss

A

pulse high dose IV glucocorticoids (methylprednisolone 1000 mg daily) for 3 days followed by intermediate to high dose oral glucocorticoids.

start low dose aspirin (recommended) to reduce risk for stroke or intracranial complications.

54
Q

jaw claudication but no temporal or scalp tenderness and fatigue and loss of appetite and vertigo and TIA

A

check for GCA

most pts don’t have scalp tenderness.

55
Q

Is ESR elevated always in GCA?

A

no. up to 20% of them are negative in GCA.

56
Q

do we look at Creatine kinase to make determinations on GCA?

A

no because they can be mildly elevated for multiple of reasons, like medications.

57
Q

criteria for diagnosing Giant cell arteritis

A

age >50 yrs
new onset localized headache with fever and visual disturbances
ESR>50
tenderness or decreased pulse of temporal artery
temporal artery biospy showing necrotizing arteritis with mainly mononuclear cells

3 out of 5 criteria is 94% sensitive and 91% specific for GCA

58
Q

atypical or extracranial giant cell arteritis presentation:

A

can affect the great vessels of the chest

can cause upper extremity claudication and or aortitis

aortitis can cause aortic root dilation, aortic regurgitation and heart failure

59
Q

what test should be done for someone who has medium vessel vasculitis?

A

renal arteriography

for someone with polyarteritis nodosa.

Rheumatology (29 decks)

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